안구 내 흑색종 치료 종합정보

주요 내용

▲ 안구 내 흑색 종은 안구 조직에 악성 (암) 세포가 형성되는 질병입니다.

▲ 나이가 많고 피부가 고르면 안구 내 흑색 종의 위험이 높아질 수 있습니다.

▲ 안구 내 흑색종의 징후로는 시야가 흐려지거나 홍채의 어두운 점이 있습니다.

▲ 눈을 검사하는 검사는 안구 내 흑색 종을 진단하는 데 사용됩니다.

▲ 안구 내 흑색 종을 진단하기 위해 종양 생검이 거의 필요하지 않습니다.

▲ 특정 요인은 예후 (회복 가능성) 및 치료 옵션에 영향을 미칩니다.

▲ 안구 내 흑색종은 안구 조직에 악성(암) 세포가 형성되는 질병입니다.

안내 흑색종은 눈 벽의 3개 층 중간에서 시작된다. 바깥층은 흰색 공막( 눈의 흰색)과 눈 앞쪽의 투명한 각막을 포함한다. 내부 층은 빛을 감지하고 시신경을 따라 뇌로 화상을 보내는 망막이라는 신경조직의 내벽을 갖고 있다.

안구 내 흑색종이 발병하는 중간층은 포도막 혹은 포도막계라 부르고 3개의 주요 부위로 이루어진다. 안내 내 흑색 종이 형성되는 중간층은 포도막 또는 포도막 관이라고하며 세 가지 주요 부분이 있다.

홍채

홍채는 눈의 전방(이하 눈동자 색)의 착색 영역이다. 홍채는 맑은 각막을 통해 볼 수 있다. 동공은 홍채의 중심에 있고 동공을 변화시켜 빛이 눈 속으로 들어오게 한다. 홍채의 안구 내 흑색 종은 일반적으로 천천히 성장하고 신체의 다른 부위로 거의 퍼지지 않는 작은 종양이다.

섬모체(혹은 모양체)

섬모체는 근육 섬유질로 이루어진 조직의 고리로 동공의 크기와 수정체의 모양을 변화시킨다. 수정체의 변화는 눈에 초점을 맞추는 데 도움이 된다. 섬모체는 또한 각막과 홍채 사이의 공간을 채우는 투명한 액체를 만든다. 섬모체의 안구 내 흑색종은 종종 홍채의 안구 내 흑색종보다 더 크고 신체의 다른 부위로 퍼질 가능성이 더 높다.

맥락막

맥락막은 눈에 산소와 영양소를 운반하는 혈관의 한 층이다. 대부분의 안구 흑색종은 맥락막에서 시작된다. 맥락막의 안구 흑색 종은 종종 홍채의 안구 흑색종보다 더 크고 신체의 다른 부위로 퍼질 가능성이 더 높다.

나이가 많고 피부가 희면 안구 내 흑색 종의 위험이 높아질 수 있다. 질병에 걸릴 위험을 증가시키는 모든 것을 위험 인자라고 한다. 위험 인자가 있다고 해서 암에 걸리게 되는 것은 아니다. 위험 요인이 없다고 해서 암에 걸리지 않는다는 의미는 아니다. 위험하다고 생각되면 의사와 상담하라. 

안구 흑색종의 위험 인자는 다음과 같다

▲ 다음을 포함하는 흰 피부색을 갖고 있다.

- 주근깨가 생기고 쉽게 타는 흰 피부로, 햇볕에 타지 않거나 곱게 타지 않는다.

- 파란색 또는 녹색 또는 기타 밝은 색의 눈을 갖고 있다..

▲ 노년기이다.

▲ 백인이다.

안구 내 흑색 종의 징후로는 시야가 흐려지거나 홍채에 어두운 점이 생긴다. 안구 내 흑색종은 초기 징후나 증상을 유발하지 않을 수 있다. 의사가 동공을 확장하고 눈을 들여다보는 정기 눈 검사 중에 때때로 발견된다. 징후와 증상은 안구 흑색종이나 다른 질환으로 인해 발병할 수 있다. 다음 중 하나라도 있으면 의사에게 문의하라. 

▲ 시야가 흐려지거나 혹은 기타 시력의 변화

▲ 플로터 (시야에서 표류하는 점들) 또는 섬광

▲ 홍채의 어두운 점

▲ 동공의 크기 또는 모양의 변화

▲ 안와(눈구멍)에서 안구의 크기와 위치의 변화

눈을 조사하는 검사는 안구 흑색종을 진단하는 데 사용된다. 다음 테스트 및 시술을 이용할 수 있다.

▲ 신체검사 및 건강 이력 : 멍울이나 비정상적으로 보이는 기타 질병의 징후 확인을 포함하여 건강의 일반적인 징후를 확인하기 위한 신체검사이다. 환자의 건강 습관과 과거의 질병 및 치료에 대한 기록도 수집된다.

▲ 동공을 확장시킨 눈 검사 : 의사가 수정체와 동공을 통해 망막을 볼 수 있도록 약용 점안액으로 동공을 확대하는 눈 검사다. 망막과 시신경을 포함한 눈 안쪽을 검사한다. 종양 크기의 변화를 추적하기 위해 시간이 지남에 따라 사진을 찍을 수 있다. 눈 검사에는 여러 유형이 있다.

- 검안경 검사 : 작은 확대 렌즈와 빛을 이용하여 눈의 뒤쪽을 검사하여 망막과 시신경을 체크한다.

- 세극등 생체 현미경 검사 : 강한 광선과 현미경을 사용하여 망막, 시신경 및 눈의 다른 부분을 확인하기 위해 눈 안쪽을 검사한다.

- 전방각경검사 : 각막과 홍채 사이의 눈 앞부분을 검사한다. 눈에서 액체가 빠져 나가는 부분이 막혀 있는지 확인하기 위해 특수 기구가 사용된다.

▲ 눈의 초음파 검사 : 고 에너지 음파 (초음파)가 눈의 내부 조직에서 반사되어 반향(메아리)을 만드는 시술이다. 점안액을 사용하여 눈을 마비시키고 음파를 송수신하는 작은 탐침을 눈 표면에 부드럽게 배치한다. 반향은 눈 안쪽의 그림을 만들고 각막에서 망막까지의 거리를 측정한다. 초음파 모니터 화면에 초음파라는 화상이 표시된다.

▲ 고해상도 초음파 생체 현미경 : 고 에너지 음파 (초음파)가 눈의 내부 조직에서 반사되어 반향을 만드는 시술이다. 점안액을 사용하여 눈을 마비시키고 음파를 송수신하는 작은 탐침을 눈 표면에 부드럽게 배치한다. 반향(에코)은 일반 초음파보다 눈 안쪽을 더 자세히 보여준다. 종양의 크기, 모양 및 두께와 종양이 주변 조직으로 퍼졌다는 징후가 있는지 확인한다.

▲ 구체 및 홍채의 투시 : 위 또는 아래 눈꺼풀에 조명을 배치하여 홍채, 각막, 수정체 및 섬 모체를 검사한다.

▲ 형광안저조영술: 혈관과 눈 내 의 혈액 흐름을 보는 시술이다. 주황색 형광 염료(플루오레세인)가 팔의 혈관에 주입 되어 혈류로 들어간다. 염료가 눈의 혈관을 통과할 때 특수 카메라가 망막과 맥락막의 사진을 찍어 막히거나 새는 부분을 찾는다.

▲ 인도시아닌 안저 촬영 : 눈의 맥락막 층에 있는 혈관을 관찰하는 시술이다. 녹색 염료(인도시아닌 그린)가 팔의 혈관에 주입되어 혈류로 들어간다. 염료가 눈의 혈관을 통과 할 때 특수 카메라가 망막과 맥락막의 사진을 찍어 막히거나 새는 부분을 찾는다.

▲ 시각 단층 촬영 혈관 조영술 : 광파를 사용하여 망막, 때로는 맥락막의 단면 사진을 찍어 망막 아래에 부종이나 체액이 있는지 확인 하는 영상 검사다.

안구 내 흑색종을 진단하기 위해 종양의 생검이 거의 필요하지 않는다. 조직 검사는 세포나 조직을 절제하여 암의 징후를 확인하기 위해 현미경으로 볼 수 있다. 드물게 안구 내 흑색종을 진단하기 위해 종양 생검이 필요하다. 종양 제거를 위한 생검 또는 수술 중에 제거 된 조직을 검사하여 예후 및 가장 좋은 치료 옵션 에 대한 자세한 정보를 얻을 수 있다.

조직의 검체에 대해 다음 검사를 수행 할 수 있습니다

▲ 세포 유전학적 분석 : 조직 샘플에 있는 세포의 염색체를 계산하고 파손, 누락, 재배열 또는 추가 염색체와 같은 변화가 있는지 확인하는 실험실 검사다. 특정 염색체의 변화는 암의 징후일 수 있다. 세포 유전학적 분석은 암을 진단하고 치료를 계획하거나 치료가 얼마나 효과가 있는지 알아보는 데 사용된다.

▲ 유전자 발현 프로파일링 : 메신저 RNA를 생성 (발현)하는 세포 또는 조직의모든 유전자를 확인하는 실험실 테스트. 메신저 RNA의 분자는 캐리 유전하기 위해 필요한 정보를 단백질로부터 DNA 세포에서 핵 세포에서 단백질을 만드는 기계에 세포질.

생검으로 인해 망막 박리가 발생할 수 있다.(망막이 눈의 다른 조직과 분리됨) 이것은 수술로 치료할 수 있다. 특정 요인은 예후(회복 가능성) 및 치료 옵션에 영향을 미친다.

예후 및 치료 옵션은 다음에 따라 다른다. 

▲ 흑색종 세포가 현미경에 어떻게 나타나는지

▲ 종양의 크기와 두께

▲ 종양이 있는 눈의 일부 (홍채, 섬모체 또는 맥락막)

▲ 종양이 눈의 내부 또는 신체의 다른 부위로 퍼 졌는지 여부

▲ 안구 흑색종과 관련된 유전자에 특정 변화가 있는지 여부

▲ 환자의 나이와 일반적인 건강 상태

▲ 치료 후 종양이 재발 했는지 여부

2. 안구 내(포도막) 흑색종의 병기

▲ 안구 흑색종이 진단 된 후 암세포가 신체의 다른 부위로 퍼 졌는지 확인하기 위해 검사를 실시한다.

▲ 다음 크기는 안내 흑색 종을 설명하고 치료를 계획하는 데 사용된다.

작다, 중간 정도이다, 크다.

▲ 암이 몸에서 퍼지는 세 가지 방법이 있다.

▲ 암이 시작된 곳에서 신체의 다른 부위로 퍼질 수 있다.

▲ 섬모체 및 맥락막 안구 흑색종에는 다음 병기가 사용된다.

1기, II기, III기, IV기

▲ 홍채의 안구 내 흑색종에 대한 병기 결정 시스템은 없다.

▲ 안구 내 흑색종은 치료 후 재발 할 수 있다.

안구 내 흑색종이 진단 된 후 암세포가 신체의 다른 부위로 퍼졌는지 확인하기 위해 검사를 실시한다. 암이 신체의 다른 부위로 퍼졌는지 확인하는 데 사용되는 과정을 병기 결정 이라고 한다. 병기 결정 과정에서 수집된 정보는 질병의 병기를 결정한다. 치료를 계획하기 위해서는 병기를 아는 것이 중요하다.

병기결정 과정에서 다음 테스트 및 시술이 사용할 수 있다

▲ 혈액 화학 연구 : 신체의 장기 와 조직 에서혈액으로 방출되는 특정 물질의 양을 측정하기 위해 혈액 샘플을 확인하는 절차다. 비정상적인 (정상보다 높거나 낮은) 양의 물질은 질병의 징후 일 수 있다.

▲ 간 기능 검사 : 간에서 혈액으로 분비되는 특정 물질의 양을 측정하시위해 혈액 샘플을 검사하는 시술. 정상적인 양보다 많은 양의 물질은 암이 간에 퍼졌다는 신호일 수 있다.

▲ 초음파 검사 : 고에너지 음파(초음파)를 간 등 내부 조직이나 기관에서 튕겨 나와 메아리를 만드는 시술. 메아리는 초음파라고 불리는 신체 조직의 그림을 형성한다.

▲ 흉부 X-레이 : 가슴 안쪽의 장기 및 뼈의 엑스레이. 엑스레이는 몸속을 통과해 필름에 들어갈 수 있는 에너지 빔의 일종으로, 몸 안의 부위를 촬영한다.

▲ MRI(자기 공명 영상) : 자석과 전파, 컴퓨터를 이용해 간 등 몸속의 부위를 일련의 세밀한 사진을 만드는 시술. 이 시술을 핵자기공명영상(NMRI)이라고도 한다.

▲ CT 스캔 : 가슴과 복부, 골반 등 신체 내부 부위를 서로 다른 각도에서 촬영한 일련의 세밀한 사진을 만드는 시술. 이 사진들은 엑스레이 기계와 연결된 컴퓨터에 의해 만들어진다. 염료는 정맥에 주사하거나 삼켜서 장기나 조직이 더 선명하게 나타나도록 도울 수 있다. 이 시술을 컴퓨터단층촬영이라 부른다.

▲ PET 스캔(양전자 방출 단층 촬영 스캔) : 몸에서 악성 종양세포를 찾는 시술. 매우 적은 양의 방사성 포도당을 정맥에 주입한다. PET 스캐너는 몸 주위를 회전하며 몸 안에서 포도당이 사용되고 있는 곳을 그림으로 만든다. 악성 종양 세포는 정상 세포보다 활동성이 좋고 포도당을 많이 섭취하기 때문에 사진에서 더 밝게 나타난다. 때때로 PET 스캔과 CT 스캔이 동시에 이루어지기도 한다. 암이 생기면 발견될 확률이 높아진다.

안구 내 흑색종을 파악하고 치료계획을 세우기 위해 다음과 같은 크기를 사용한다.

크기가 작다.

종양의 지름은 5~16mm이고 두께는 1~3mm이다.

크기가 중간이다.

종양은 직경이 16mm 이하, 두께 3.1~8mm이다.

크기가 크다.

종양은 다음과 같다

▲ 8mm 이상의 두께와 직경은 무관하다.

▲ 2mm 이상의 두께와 16mm 이상의 직경이다.

대부분의 안구 내 흑색종양들이 자라면서 부풀어 오르지만, 일부는 평평하다. 이러한 확산성 종양은 포도막(안구) 전역에서 광범위하게 자란다. 체내에 암이 퍼지는 길은 세 가지다. 암은 조직과 림프계 및 혈액을 통해 퍼질 수 있다.

▲ 조직. 암은 자라면서 시작된 곳에서 인근 부위로 퍼진다.

▲ 림프계. 암은 림프계에 들어가면서 시작된 곳에서 퍼진다.

암은 림프관을 통해 신체의 다른 부분으로 이동한다.

▲ 혈액. 암은 혈액 속으로 들어가면서 시작된 곳에서 퍼진다.

암은 혈관을 통해 신체의 다른 부분으로 이동한다.

안구 내 흑색종이 안와(눈구멍)의 시신경이나 인근 조직으로 퍼지면 이를 안구 외(바깥) 확장이라고 한다. 암은 시작된 곳에서 신체의 다른 부분으로 전이될 수도 있다. 암이 몸의 다른 부분으로 퍼지면 전이라고 한다. 암세포는 시작된 곳(1차종양 혹은 원발소)에서 벗어나 림프계나 혈액을 통해 이동한다.

▲ 림프계. 암은 림프계에 들어가 림프관을 통해 이동하며 신체의 다른 부위에 종양(금속종양)을 형성한다.

▲ 혈액. 암은 혈액 속으로 들어가 혈관을 통해 이동하며 몸의 다른 부위에 종양(전이 암)을 형성한다.

전이성 종양은 1차 종양과 같은 종류의 암이다. 예를 들어 안구 내 흑색종이 간으로 퍼지면 간 내 암세포는 사실상 안구 내 흑색종 세포다. 그 병은 간암이 아니라 전이성 안구 내 흑색종이다. 

섬모체와 맥락막의 안구 내 흑색종에는 다음과 같은 병기가 사용된다. 섬모체와 맥락막의 안구 내 흑색종은 네 가지 크기의 카테고리(범주)를 가지고 있다. 종양이 얼마나 넓고 두꺼운지에 따라 카테고리(범주)가 달라진다. 카테고리1 종양이 가장 작고 카테고리4 종양이 가장 크다.

카테고리 1

▲ 종양은 폭 12mm 이하, 두께 3mm 이하인 경우

▲ 종양은 폭 9mm 이하, 두께 3.1~6mm 이하

카테고리 2

▲ 종양의 넓이는 12.1~18mm이고 두께는 3mm 이하인 경우

▲ 종양의 넓이는 9.1~15mm이고 두께는 3.1~6mm이다.

▲ 종양의 넓이는 12mm 이하, 두께는 6.1~9mm 이하.

카테고리 3

▲ 종양의 넓이는 15.1~18mm이고 두께는 3.1~6mm이다.

▲ 종양의 넓이는 12.1~18mm이고 두께는 6.1~9mm이다.

▲ 종양의 넓이는 18mm 이하, 두께는 9.1~12mm 이하, 또는

▲ 종양은 넓이가 15mm 이하, 두께가 12.1~15mm 이하.

카테고리 4

▲ 종양의 넓이는 18mm 이상이고 두께는 어느 정도일 수 있다.

▲ 종양의 넓이는 15.1~18mm이고 두께는 12mm 이상이다.

▲ 종양의 넓이는 15mm 이하, 두께는 15mm 이상이다.

1기 : 1기에는 종양은 크기가 카테고리1이며, 맥락막에만 있다.

2기 : 2기는 IIA와 IIB기로 나뉜다.

▲ IIA기에서 종양은

① 크기가 카테고리1이며 섬모체로 확산됨. 또는 크기가 카테고리1이며 공막을 통해 안구 바깥쪽으로 퍼져 있다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 섬모체까지 전이되었을 수 있다. 

② 크기가 카테고리2이며, 맥락막에만 있다.

▲ IIB기에서 종양은 

① 크기가 카테고리2이며, 섬모체까지 확산됨. 또는 크기가 카테고리3이며, 맥락막에만 있다.

② 3기는 IIIA, IIIB, IIIC기로 나뉜다.

▲ IIIA에서 종양은 

① 크기가 카테고리2이며 공막을 통해 안구 바깥쪽으로 퍼져 있다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 섬모체까지 전이되었을 수 있다.

② 크기가 카테고리3이며, 섬모체까지 확산됨. 

③ 크기가 카테고리3이며 공막을 통해 안구 바깥쪽으로 퍼졌다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 섬모체까지 전이되지 않았거나

④ 크기가 카테고리4이며, 맥락막에만 있다.

▲ IIIB기에서 종양은 

① 크기가 카테고리3이며 공막을 통해 안구 바깥쪽으로 퍼졌다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 섬모체까지 퍼졌거나

② 크기 카테고리 4이며, 담도체까지 확산됨.  

③ 사이즈 범주 4이며 눈알을 통해 안구 바깥쪽으로 퍼져 있다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 담도체까지 전이되지 않았다.

▲ IIIB에서 종양은 

① 사이즈 범주 4이며 눈알을 통해 안구 바깥쪽으로 퍼져 있다. 안구 바깥쪽 종양의 부위는 5mm 이하의 두께로 되어 있다. 종양이 담도체까지 퍼졌거나

② 크기와는 무관하며 공막을 통해 안구 바깥쪽으로 퍼졌다. 안구 바깥쪽 종양의 부위는 5밀리미터가 넘는다.

4기

① 4기에서 종양은 크기와 무관할 수도 있고 아래의 부위로 퍼졌다.

② 하나 이상의 인근 림프절 또는 1차 종양과는 별개의 안와로 퍼졌거나

③ 간, 폐, 뼈, 뇌 또는 피부 아래의 조직과 같은 신체의 다른 부위로 퍼졌다.

④ 홍채의 안구 내 흑색종을 위한 병기 측정 시스템이 없다.

⑤ 안구 내 흑색종은 치료 후 재발할 수 있다

⑥ 흑색종은 눈이나 몸의 다른 부위에 재발할 수 있다.

3. 치료 옵션 개요

5가지 표준 치료법이 사용된다. 

▲ 수술

▲ 예의 주시

▲ 방사선요법

▲ 광응고(광선응고법 : 레이저 광선 등에 의한 안질 치료법)

▲ 온열요법

안구 내 흑색종 환자들을 위한 치료법에는 여러 종류가 있다. 안구 내 흑색종 환자에게는 다양한 종류의 치료가 가능하다. 어떤 치료법은 표준 치료법(현재 사용되고 있는 치료법)이며, 어떤 치료법은 임상시험에서 시험되고 있다. 치료 임상시험은 현재의 치료법을 개선하거나 암환자의 새로운 치료법에 대한 정보를 얻는데 도움을 주기 위한 연구다. 새로운 치료법이 표준 치료법보다 낫다는 임상시험 결과가 나오면 새로운 치료법이 표준 치료법이 될 수도 있다. 환자들은 임상 실험에 참여하는 것에 대해 생각하고 싶을 것이다. 일부 임상시험은 치료를 시작하지 않은 환자에게만 개방된다.

다음과 같은 5가지 유형의 표준 치료가 사용된다.

① 수술

수술은 안구 내 흑색종의 가장 흔한 치료법이다. 다음과 같은 유형의 수술을 사용할 수 있다.

▲ 절제 : 종양과 그 주변의 건강한 조직을 소량 제거하는 수술

▲ 적출(안구 제거) : 눈과 시신경의 일부를 제거하는 수술. 시력을 구할 수 없고 종양이 크거나 시신경으로 전이되거나 눈 안쪽에 고압의 원인이 되는 경우 이 작업을 수행한다. 수술 후에는 보통 다른 눈의 크기와 색깔에 맞게 인공 눈을 맞춘다.

▲ 안와 내용물 제거 : 눈과 눈꺼풀과 근육, 신경 및 지방을 제거하는 수술. 수술 후, 다른 눈의 크기와 색깔에 맞게 인공눈을 설치하거나 안면 보형물을 착용할 수 있다.

② 예의 주시

예의 주시란 징후나 증상이 나타나거나 바뀔 때까지 아무런 치료도 하지 않고 환자의 상태를 면밀히 관찰하는 것이다. 종양의 크기와 성장속도를 추적하기 위해 시간을 두고 사진을 찍어서 관찰한다. 징후나 증상이 없고 종양이 자라지 않는 환자에게는 예의 주시를 한다. 또한 종양이 유용한 시력을 가진 외눈에 있을 때에도 사용된다.

③ 방사선요법

방사선 치료는 암세포를 죽이거나 성장하지 못하도록 고에너지 X선이나 다른 종류의 방사선을 이용하는 암 치료법이다. 방사선 치료에는 두 가지 유형이 있다.

외부 방사선 치료는 암을 향해 방사선을 조사하기 위해 체외 기구를 사용한다. 방사선 치료를 하는 특정한 방법은 방사능이 근처의 건강한 조직을 손상시키지 않도록 하는데 도움을 줄 수 있다. 이러한 유형의 외부 방사선 치료에는 다음이 포함된다. 

▲ 충전-입자 외부 빔 방사선 치료는 외부 빔 방사선 치료의 일종이다. 특별한 방사선 치료기는 양성자나 헬륨 이온이라고 불리는 작고 눈에 보이지 않는 입자들로 암세포를 겨 하여 주변의 정상조직에 거의 손상을 주지 않고 그들을 죽인다. 충전-입자 방사선 치료는 X-선 치료와는 다른 종류의 방사선을 사용한다.

▲ 감마 나이프 요법은 일부 흑색종에 사용되는 정위 방사선 수술의 일종이다. 이 치료는 한 번의 치료로 할 수 있다. 그것은 종양에 직접 집중된 감마선을 겨냥하여 건강한 조직에 거의 손상을 주지 않는다. 감마 나이프 요법은 종양을 제거하기 위해 칼을 사용하지 않으며 수술이 아니다.

내부 방사선 치료는 바늘과 씨앗 모양, 전선 또는 카테터에 밀봉된 방사성 물질을 사용하여 암에 직접 또는 암 근처에 배치한다. 방사선 치료를 하는 특정한 방법은 방사선이 건강한 조직을 손상시키지 않도록 하는데 도움을 줄 수 있다. 이러한 유형의 내부 방사선 치료에는 다음이 포함될 수 있다.  

▲ 국소 플라크 방사선 치료는 눈의 종양에 사용될 수 있는 내부 방사선 치료의 일종이다. 방사성 씨앗 모양의 방사능 물질은 원반 한쪽에 붙어 있으며, 플라크라고 불리며 종양 근처의 눈 외벽에 바로 놓여 있다. 씨앗이 묻힌 판(플라크)의 옆면은 안구를 향하며 종양을 향해 방사선을 겨누고 있다. 이 판은 방사능으로부터 근처의 다른 조직을 보호하는 데 도움을 준다.

방사선치료를 받는 방법은 치료 중인 암의 종류와 병기에 따라 달라진다. 안구 내 흑색종 치료에 외부 및 내부 방사선치료가 사용된다.

④ 광응고술

광응고술은 레이저광선을 이용해 종양에 영양분을 공급하는 혈관을 파괴해 종양세포가 죽게 하는 시술이다. 광응고술은 작은 종양을 치료하는 데 사용될 수 있다. 이것을 빛 응고(술)라고도 한다.

⑤ 온열요법

온열요법은 레이저의 열을 이용해 암세포를 파괴하고 종양을 수축시키는 것이다. 새로운 종류의 치료법이 임상시험에서 시험되고 있다. 임상시험에 대한 정보는 국립암연구소(NC)I 웹사이트에서 확인할 수 있다. 안구 내 흑색종 치료는 부작용을 일으킬 수 있다. 환자들은 임상 실험에 참여하는 것에 대해 생각하고 싶을 것이다.

일부 환자에게는 임상 실험에 참여하는 것이 최선의 치료 선택일 수도 있다. 임상시험은 암 연구과정의 일부분이다. 새로운 암 치료법이 표준 치료법보다 안전하고 효과적이거나 좋은지 알아보기 위해 임상시험을 한다.

오늘날 암에 대한 표준 치료법들 중 많은 것들이 이전의 임상실험에 기초하고 있다. 임상시험에 참여한 환자는 표준 치료를 받거나 새로운 치료를 처음 받는 환자 중 한 명이 될 수 있다.

임상시험에 참여하는 환자도 앞으로 암 치료 방식을 개선하는 데 도움이 된다. 임상시험이 효과적인 새로운 치료법으로 이어지지 않을 때도 중요한 질문에 답해 연구를 진전시키는 데 도움을 주는 경우가 많다.

환자들은 암 치료를 시작하기 전과 중 또는 후에 임상 시험에 들어갈 수 있다. 일부 임상시험에는 아직 치료를 받지 못한 환자만 포함된다. 다른 시험에서는 암이 낫지 않은 환자들을 위해 치료법을 시험한다. 암이 재발하지 않도록 새로운 방법을 시험하거나 암 치료의 부작용을 줄이는 임상 실험도 있다.

전국 각지에서 임상시험이 진행되고 있다. NCI가 지원하는 임상시험에 대한 정보는 NCI의 임상시험 검색 홈페이지에서 확인할 수 있다. 다른 기관이 지원하는 임상시험은 ClinicalTrials.gov 웹사이트에서 확인할 수 있다.

추적 검사가 필요할 수 있다

암을 진단하거나 암의 병기를 알아내기 위해 행해진 몇몇 검사들은 반복될 수 있다. 치료가 얼마나 잘 되고 있는지 알아보기 위해 몇 가지 검사를 반복할 것이다. 치료를 계속할지, 변경할지 또는 중단할지에 대한 결정은 이러한 시험의 결과에 기초할 수 있다.

일부 검사는 치료가 끝난 후에도 수시로 계속된다. 이러한 검사 결과는 당신의 상태가 변했는지 또는 암이 재발했는지를 보여줄 수 있다. 이러한 검사를 후속 검사 또는 검사라고 부르기도 한다.

4. 안구 내(포도막) 흑색종의 치료

치료 대상 암의 종류

▲ 홍채 흑색종

▲ 섬모체 흑색종

▲ 맥락막 흑색종

▲ 안구 외 흑색종 및 전이 안구 내(포도막) 흑색종

▲ 재발 안구 내(포도막) 흑색종

홍채 흑색종 치료에는 다음이 포함될 수 있다.

▲ 예의 주시

▲ 수술(절제 또는 안구 적출)

▲ 수술로 제거할 수 없는 종양에 대한 판 방사선 치료

당사의 임상 시험 검색을 사용하여 환자를 수용하는 NCI 지원 암 임상 시험을 찾으십시오. 암의 종류, 환자의 나이, 그리고 실험이 행해지고 있는 곳을 기준으로 하여 실험을 검색할 수 있다. 임상시험에 대한 일반 정보도 이용할 수 있다.

섬모체 흑색종 치료에는 다음이 포함될 수 있다.

▲ 판(플라크) 방사선 치료.

▲ 충전-입자 외부 빔 방사선 치료

▲ 수술(절제 혹은 안구 적출)

당사의 임상 시험 검색을 사용하여 환자를 수용하는 NCI 지원 암 임상 시험을 찾으십시오. 암의 종류, 환자의 나이, 그리고 실험이 행해지고 있는 곳을 기준으로 하여 실험을 검색할 수 있다. 임상시험에 대한 일반 정보도 이용할 수 있다.

크기가 작은 맥락막 흑색종 치료에는 다음이 포함될 수 있다.

▲ 판 방사선 치료.

▲ 충전-입자 외부 빔 방사선 치료

▲ 감마 나이프 치료.

▲ 온열요법.

▲ 수술(절제 혹은 안구 적출)

중간 크기의 맥락막 흑색종 치료에는 다음이 포함될 수 있다.

▲ 광응고 또는 열 요법을 사용하거나 사용하지 않는 판 방사선 치료

▲ 충전-입자 외부 빔 방사선 치료

▲ 수술(절제 혹은 안구 적출)

크기가 큰 맥락막 흑색종 치료에는 다음이 포함될 수 있다.

▲ 눈을 살리는 치료를 하기에는 종양이 너무 클 때 안구 적출

당사의 임상 시험 검색을 사용하여 환자를 수용하는 NCI 지원 암 임상 시험을 찾으십시오. 암의 종류, 환자의 나이, 그리고 실험이 행해지고 있는 곳을 기준으로 하여 실험을 검색할 수 있다. 임상 시험에 대한 일반 정보도 이용할 수 있다.

안구 외 확장 흑색종과 전이성 안구 내(포도막) 흑색종

눈 주위 뼈까지 전이된 안구 외 확산 흑색종의 치료에는 다음이 포함될 수 있다. 

▲ 수술(안구 적출)

▲ 임상시험

전이성 안구 내 흑색종에 대한 효과적인 치료법은 발견되지 않았다. 임상시험은 치료 선택사항이 될 수 있다. 치료 방법에 대해 의사와 상의하십시오.

당사의 임상 시험 검색을 사용하여 환자를 수용하는 NCI 지원 암 임상 시험을 찾으십시오. 암의 종류, 환자의 나이, 그리고 실험이 행해지고 있는 곳을 기준으로 하여 실험을 검색할 수 있다. 임상시험에 대한 일반 정보도 이용할 수 있다.

재발 안구 내(포도막) 흑색종

재발한 안구 내 흑색종에 대한 효과적인 치료법은 발견되지 않았다. 임상시험은 치료 선택사항일 수 있다. 치료 방법에 대해 의사와 상의하십시오.

업데이트 : 2020 년 10 월 2 일

Intraocular (Uveal) Melanoma Treatment (PDQ®)–Patient Version

Updated: October 2, 2020

On This Page

•General Information About Intraocular (Uveal) Melanoma

•Stages of Intraocular (Uveal) Melanoma

•Treatment Option Overview

•Treatment of Intraocular (Uveal) Melanoma

•To Learn More About Intraocular (Uveal) Melanoma

•About This PDQ Summary

1. General Information About Intraocular (Uveal) Melanoma

Key Points

•Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye.

•Being older and having fair skin may increase the risk of intraocular melanoma.

•Signs of intraocular melanoma include blurred vision or a dark spot on the iris.

•Tests that examine the eye are used to help diagnose intraocular melanoma.

•A biopsy of the tumor is rarely needed to diagnose intraocular melanoma.

•Certain factors affect prognosis (chance of recovery) and treatment options.

Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye.

Intraocular melanoma begins in the middle of three layers of the wall of the eye. The outer layer includes the white sclera (the "white of the eye") and the clear cornea at the front of the eye. The inner layer has a lining of nerve tissue, called the retina, which senses light and sends images along the optic nerve to the brain.

The middle layer, where intraocular melanoma forms, is called the uvea or uveal tract, and has three main parts:

Iris

The iris is the colored area at the front of the eye (the "eye color"). It can be seen through the clear cornea. The pupil is in the center of the iris and it changes size to let more or less light into the eye. Intraocular melanoma of the iris is usually a small tumor that grows slowly and rarely spreads to other parts of the body.

Ciliary body

The ciliary body is a ring of tissue with muscle fibers that change the size of the pupil and the shape of the lens. It is found behind the iris. Changes in the shape of the lens help the eye focus. The ciliary body also makes the clear fluid that fills the space between the cornea and the iris. Intraocular melanoma of the ciliary body is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris.

Choroid

The choroid is a layer of blood vessels that bring oxygen and nutrients to the eye. Most intraocular melanomas begin in the choroid. Intraocular melanoma of the choroid is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris.

Intraocular melanoma is a rare cancer that forms from cells that make melanin in the iris, ciliary body, and choroid. It is the most common eye cancer in adults.

Being older and having fair skin may increase the risk of intraocular melanoma.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk.

Risk factors for intraocular melanoma include the following:

•Having a fair complexion, which includes the following:

-Fair skin that freckles and burns easily, does not tan, or tans poorly.

-Blue or green or other light-colored eyes.

•Older age.

•Being white.

Signs of intraocular melanoma include blurred vision or a dark

spot on the iris.

Intraocular melanoma may not cause early signs or symptoms. It is sometimes found during a regular eye exam when the doctor dilates the pupil and looks into the eye. Signs and symptoms may be caused by intraocular melanoma or by other conditions. Check with your doctor if you have any of the following:

•Blurred vision or other change in vision.

•Floaters (spots that drift in your field of vision) or flashes of light.

•A dark spot on the iris.

•A change in the size or shape of the pupil.

•A change in the position of the eyeball in the eye socket.

Tests that examine the eye are used to help diagnose intraocular melanoma.

The following tests and procedures may be used:

•Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

•Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (enlarged) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is checked. Pictures may be taken over time to keep track of changes in the size of the tumor. There are several types of eye exams:

-Ophthalmoscopy: An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light.

-Slit-lamp biomicroscopy: An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope.

-Gonioscopy: An exam of the front part of the eye between the cornea and iris. A special instrument is used to see if the area where fluid drains out of the eye is blocked.

•Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor.

•High-resolution ultrasound biomicroscopy: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a more detailed picture of the inside of the eye than a regular ultrasound. The tumor is checked for its size, shape, and thickness, and for signs that the tumor has spread to nearby tissue.

•Transillumination of the globe and iris: An exam of the iris, cornea, lens, and ciliary body with a light placed on either the upper or lower lid.

•Fluorescein angiography: A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye (fluorescein) is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any areas that are blocked or leaking.

•Indocyanine green angiography: A procedure to look at blood vessels in the choroid layer of the eye. A green dye (indocyanine green) is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any areas that are blocked or leaking.

•Ocular coherence tomography: An imaging test that uses light waves to take cross-section pictures of the retina, and sometimes the choroid, to see if there is swelling or fluid beneath the retina.

A biopsy of the tumor is rarely needed to diagnose intraocular melanoma.

A biopsy is the removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Rarely, a biopsy of the tumor is needed to diagnose intraocular melanoma. Tissue that is removed during a biopsy or surgery to remove the tumor may be tested to get more information about prognosis and which treatment options are best.

The following tests may be done on the sample of tissue:

•Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.

•Gene expression profiling: A laboratory test that identifies all of the genes in a cell or tissue that are making (expressing) messenger RNA. Messenger RNA molecules carry the genetic information that is needed to make proteins from the DNA in the cell nucleus to the protein-making machinery in the cell cytoplasm.

A biopsy may result in retinal detachment (the retina separates from other tissues in the eye). This can be repaired by surgery.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

•How the melanoma cells look under a microscope.

•The size and thickness of the tumor.

•The part of the eye the tumor is in (the iris, ciliary body, or choroid).

•Whether the tumor has spread within the eye or to other places in the body.

•Whether there are certain changes in the genes linked to intraocular melanoma.

•The patient's age and general health.

•Whether the tumor has recurred (come back) after treatment.

2. Stages of Intraocular (Uveal) Melanoma

Key Points

•After intraocular melanoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

•The following sizes are used to describe intraocular melanoma and plan treatment:

-Small

-Medium

-Large

•There are three ways that cancer spreads in the body.

•Cancer may spread from where it began to other parts of the body.

•The following stages are used for intraocular melanoma of the ciliary body and choroid:

-Stage I

-Stage II

-Stage III

-Stage IV

•There is no staging system for intraocular melanoma of the iris.

•Intraocular melanoma can recur (come back) after it has been treated.

After intraocular melanoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The process used to find out if cancer has spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

The following tests and procedures may be used in the staging process:

•Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.

•Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign the cancer has spread to the liver.

•Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the liver, and make echoes. The echoes form a picture of body tissues called a sonogram.

•Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

•MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the liver. This procedure is also called nuclear magnetic resonance imaging (NMRI).

•CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, or pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

•PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A very small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.

The following sizes are used to describe intraocular melanoma and plan treatment:

Small

The tumor is 5 to 16 millimeters in diameter and from 1 to 3 millimeters thick.

Medium

The tumor is 16 millimeters or smaller in diameter and from 3.1 to 8 millimeters thick.

Large

The tumor is:

•more than 8 millimeters thick and any diameter; or

•at least 2 millimeters thick and more than 16 millimeters in diameter.

Though most intraocular melanoma tumors are raised, some are flat. These diffuse tumors grow widely across the uvea.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

•Tissue. The cancer spreads from where it began by growing into nearby areas.

•Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.

•Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

If intraocular melanoma spreads to the optic nerve or nearby tissue of the eye socket, it is called extraocular extension.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

•Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.

•Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if intraocular melanoma spreads to the liver, the cancer cells in the liver are actually intraocular melanoma cells. The disease is metastatic intraocular melanoma, not liver cancer.

The following stages are used for intraocular melanoma of the ciliary body and choroid:

Intraocular melanoma of the ciliary body and choroid has four size categories. The category depends on how wide and thick the tumor is. Category 1 tumors are the smallest and category 4 tumors are the largest.

Category 1:

•The tumor is not more than 12 millimeters wide and not more than 3 millimeters thick; or

•the tumor is not more than 9 millimeters wide and 3.1 to 6 millimeters thick.

Category 2:

•The tumor is 12.1 to 18 millimeters wide and not more than 3 millimeters thick; or

•the tumor is 9.1 to 15 millimeters wide and 3.1 to 6 millimeters thick; or

•the tumor is not more than 12 millimeters wide and 6.1 to 9 millimeters thick.

Category 3:

•The tumor is 15.1 to 18 millimeters wide and 3.1 to 6 millimeters thick; or

•the tumor is 12.1 to 18 millimeters wide and 6.1 to 9 millimeters thick; or

•the tumor is not more than 18 millimeters wide and 9.1 to 12 millimeters thick; or

•the tumor is not more than 15 millimeters wide and 12.1 to 15 millimeters thick.

Category 4:

•The tumor is more than 18 millimeters wide and may be any thickness; or

•the tumor is 15.1 to 18 millimeters wide and more than 12 millimeters thick; or

•the tumor is not more than 15 millimeters wide and more than 15 millimeters thick.

Stage I

In stage I, the tumor is size category 1 and is in the choroid only.

Stage II

Stage II is divided into stages IIA and IIB.

•In stage IIA, the tumor:

-is size category 1 and has spread to the ciliary body; or

-is size category 1 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; or

-is size category 2 and is in the choroid only.

•In stage IIB, the tumor:

-is size category 2 and has spread to the ciliary body; or

-is size category 3 and is in the choroid only.

Stage III

Stage III is divided into stages IIIA, IIIB, and IIIC.

•In stage IIIA, the tumor:

-is size category 2 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; or

-is size category 3 and has spread to the ciliary body; or

•is size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body; or

-is size category 4 and is in the choroid only.

•In stage IIIB, the tumor:

-is size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; or

-is size category 4 and has spread to the ciliary body; or

-is size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body.

•In stage IIIC, the tumor:

•is size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; or

-may be any size and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is more than 5 millimeters thick.

Stage IV

In stage IV, the tumor may be any size and has spread:

•to one or more nearby lymph nodes or to the eye socket separate from the primary tumor; or

•to other parts of the body, such as the liver, lung, bone, brain, or tissue under the skin.

There is no staging system for intraocular melanoma of the iris.

Intraocular melanoma can recur (come back) after it has been treated.

The melanoma may come back in the eye or in other parts of the body.

3. Treatment Option Overview

Key Points

•There are different types of treatments for patients with

intraocular melanoma.

•Five types of standard treatment are used:

-Surgery

-Watchful Waiting

-Radiation therapy

- Photocoagulation

- Thermotherapy

•New types of treatment are being tested in clinical trials.

•Treatment for intraocular (uveal) melanoma may cause side effects.

•Patients may want to think about taking part in a clinical trial.

•Patients can enter clinical trials before, during, or after starting their cancer treatment.

•Follow-up tests may be needed.

There are different types of treatments for patients with intraocular melanoma.

Different types of treatments are available for patients with intraocular melanoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Five types of standard treatment are used:

Surgery

Surgery is the most common treatment for intraocular melanoma. The following types of surgery may be used:

•Resection: Surgery to remove the tumor and a small amount of healthy tissue around it.

•Enucleation: Surgery to remove the eye and part of the optic nerve. This is done if vision cannot be saved and the tumor is large, has spread to the optic nerve, or causes high pressure inside the eye. After surgery, the patient is usually fitted for an artificial eye to match the size and color of the other eye.

•Exenteration: Surgery to remove the eye and eyelid, and muscles, nerves, and fat in the eye socket. After surgery, the patient may be fitted for an artificial eye to match the size and color of the other eye or a facial prosthesis.

Watchful Waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Pictures are taken over time to keep track of changes in the size of the tumor and how fast it is growing.

Watchful waiting is used for patients who do not have signs or symptoms and the tumor is not growing. It is also used when the tumor is in the only eye with useful vision.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

•External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following:

-Charged-particle external beam radiation therapy is a type of external-beam radiation therapy. A special radiation therapy machine aims tiny, invisible particles, called protons or helium ions, at the cancer cells to kill them with little damage to nearby normal tissues. Charged-particle radiation therapy uses a different type of radiation than the x-ray type of radiation therapy.

-Gamma Knife therapy is a type of stereotactic radiosurgery used for some melanomas. This treatment can be given in one treatment. It aims tightly focused gamma rays directly at the tumor so there is little damage to healthy tissue. Gamma Knife therapy does not use a knife to remove the tumor and is not an operation.

•Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue. This type of internal radiation therapy may include the following:

-Localized plaque radiation therapy is a type of internal radiation therapy that may be used for tumors of the eye. Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.

The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat intraocular melanoma.

Photocoagulation

Photocoagulation is a procedure that uses laser light to destroy blood vessels that bring nutrients to the tumor, causing the tumor cells to die. Photocoagulation may be used to treat small tumors. This is also called light coagulation.

Thermotherapy

Thermotherapy is the use of heat from a laser to destroy cancer cells and shrink the tumor.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for intraocular (uveal) melanoma may cause side effects.

For information about side effects caused by treatment for cancer, see our Side Effects page.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

4. Treatment of Intraocular (Uveal) Melanoma

In This Section

•Iris Melanoma

•Ciliary Body Melanoma

•Choroid Melanoma

•Extraocular Extension Melanoma and Metastatic Intraocular (Uveal) Melanoma

•Recurrent Intraocular (Uveal) Melanoma

For information about the treatments listed below, see the Treatment Option Overview section.

Iris Melanoma

Treatment of iris melanoma may include the following:

•Watchful waiting.

•Surgery (resection or enucleation).

•Plaque radiation therapy, for tumors that cannot be removed by surgery.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Ciliary Body Melanoma

Treatment of ciliary body melanoma may include the following:

•Plaque radiation therapy.

•Charged-particle external-beam radiation therapy.

•Surgery (resection or enucleation).

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Choroid Melanoma

Treatment of small choroid melanoma may include the following:

•Watchful waiting.

•Plaque radiation therapy.

•Charged-particle external-beam radiation therapy.

•Gamma Knife therapy.

•Thermotherapy.

•Surgery (resection or enucleation).

Treatment of medium choroid melanoma may include the following:

•Plaque radiation therapy with or without photocoagulation or thermotherapy.

•Charged-particle external-beam radiation therapy.

•Surgery (resection or enucleation).

Treatment of large choroid melanoma may include the following:

•Enucleation when the tumor is too large for treatments that save the eye.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Extraocular Extension Melanoma and Metastatic Intraocular (Uveal) Melanoma

Treatment of extraocular extension melanoma that has spread to the bone around the eye may include the following:

•Surgery (exenteration).

•A clinical trial.

An effective treatment for metastatic intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Recurrent Intraocular (Uveal) Melanoma

An effective treatment for recurrent intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Retinoblastoma Treatment (PDQ®)–Patient Version

Updated: September 11, 2020

On This Page

•General Information About Retinoblastoma

•Stages of Retinoblastoma

•Treatment Option Overview

•Treatment of Unilateral, Bilateral, and Cavitary Retinoblastoma

•Treatment of Extraocular Retinoblastoma

•Treatment of Progressive or Recurrent Retinoblastoma

•To Learn More About Childhood Cancer

•About This PDQ Summary

1. General Information About Retinoblastoma

Key Points

•Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.

•Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma.

•Retinoblastoma occurs in heritable and nonheritable forms.

•A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers.

• Genetic testing can determine whether a child has the heritable or nonheritable form of retinoblastoma.

•Signs and symptoms of retinoblastoma include "white pupil" and eyes that appear to be looking in different directions (crossed eyes).

•Tests that examine the retina are used to diagnose retinoblastoma.

•Certain factors affect prognosis (chance of recovery) and treatment options.

Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.

The retina is made of nerve tissue that lines the inside wall of the back of the eye. It receives light and converts the light into signals that travel down the optic nerve to the brain. The brain decodes the signals so that you can see the image.

Retinoblastoma may be in one eye (unilateral) or in both eyes (bilateral). Cavitary retinoblastoma is a rare type of retinoblastoma in which cavities (hollow spaces) form within the tumor.

Although retinoblastoma may occur at any age, it occurs most often in children younger than 2 years.

Children with a family history of retinoblastoma should have eye exams to check for retinoblastoma.

A child with a family history of retinoblastoma should have regular eye exams beginning early in life to check for retinoblastoma, unless it is known that the child does not have the RB1 gene change. Early diagnosis of retinoblastoma may mean the child will need less intense treatment.

Talk with your child’s doctor about the type of eye exam, how often eye exams are done, and at what age eye exams to check for retinoblastoma can stop.

Retinoblastoma occurs in heritable and nonheritable forms.

A child is thought to have the heritable (inherited) form of retinoblastoma when there is a certain mutation (change) in the RB1 gene. The mutation in the RB1 gene may be passed from the parent to the child, or it may occur in the egg or sperm before conception or soon after conception.

Other factors that suggest the child may have the heritable form of retinoblastoma include the following:

•There is a family history of retinoblastoma.

•There is more than one tumor in the eye.

•There is a tumor in both eyes.

After heritable retinoblastoma has been diagnosed and treated, new tumors may continue to form for a few years. Regular eye exams to check for new tumors are usually done every 2 to 4 months for at least 28 months.

Most cases of retinoblastoma are the nonheritable form. Nonheritable retinoblastoma is not passed down from parents. This type of retinoblastoma is caused by mutations in the RB1 gene that occur by chance after a child is born. Nonheritable retinoblastoma usually occurs in one eye.

A child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers.

A child with heritable retinoblastoma has an increased risk of a pineal tumor in the brain. When retinoblastoma and a brain tumor occur at the same time, it is called trilateral retinoblastoma. The brain tumor is usually diagnosed between 20 and 36 months of age. Regular screening using MRI (magnetic resonance imaging) may be done for a child thought to have heritable retinoblastoma or for a child with retinoblastoma in one eye and a family history of the disease. CT (computerized tomography) scans are usually not used for routine screening in order to avoid exposing the child to ionizing radiation.

Heritable retinoblastoma also increases the child's risk of other types of cancer such as lung cancer, bladder cancer, or melanoma in later years. Regular follow-up exams are important.

Genetic testing can determine whether a child has the heritable or nonheritable form of retinoblastoma.

It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following:

•Their family medical history.

•Their options for RB1 gene testing.

•The risk of retinoblastoma for the child and the child's brothers or sisters.

•The risks and benefits of learning genetic information.

Genetic counselors can also help people cope with their genetic testing results, including how to discuss the results with family members.

Once it is known that the child has heritable retinoblastoma, other family members can be screened for the RB1 mutation. For one specific mutation, the risk of retinoblastoma in a sibling may depend partly on whether the mutation is inherited from the mother or from the father.

Signs and symptoms of retinoblastoma include "white pupil" and eyes that appear to be looking in different directions (crossed eyes).

These and other signs and symptoms may be caused by retinoblastoma or by other conditions. Check with a doctor if your child has any of the following:

•Pupil of the eye that appears white instead of red when light shines into it. This may be seen in flash photographs of the child.

•Eyes that appear to be looking in different directions (crossed eyes).

•Pain or redness in the eye.

•Infection around the eye.

•Eyeball that is larger than normal.

•Colored part of the eye and pupil look cloudy.

Tests that examine the retina are used to diagnose retinoblastoma.

The following tests and procedures may be used:

•Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken. The doctor will ask if there is a family history of retinoblastoma.

•Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (opened wider) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is examined with a light. In young children, this exam may be done under anesthesia.

There are several types of eye exams that are done with the pupil dilated:

-Ophthalmoscopy: An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light.

-Fluorescein angiography: A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye called fluorescein is injected into a blood vessel in the arm and goes into the bloodstream. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any blood vessels that are blocked or leaking.

•RB1 gene test: A laboratory test in which a sample of blood or tissue is tested for a change in the RB1 gene.

•Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor. The picture can be printed to be looked at later.

•MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the eye. This procedure is also called nuclear magnetic resonance imaging (NMRI).

Retinoblastoma can usually be diagnosed without a biopsy.

When retinoblastoma is in one eye, it sometimes forms in the other eye. Exams of the unaffected eye are done until it is known that the retinoblastoma is the nonheritable form.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

•Whether the cancer is in one or both eyes.

•The size and number of tumors.

•Whether the tumor has spread to the area around the eye, to the brain, or to other parts of the body.

•The age of the child.

•How likely it is that vision can be saved in one or both eyes.

•Whether a second type of cancer has formed.

•Whether the cancer is newly diagnosed or has recurred (come back).

2. Stages of Retinoblastoma

Key Points

•After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body.

•The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma.

-Stage 0

-Stage I

-Stage II

-Stage III

-Stage IV

•There are three ways that cancer spreads in the body.

•Cancer may spread from where it began to other parts of the body.

•Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye).

-Intraocular retinoblastoma

-Extraocular retinoblastoma (metastatic)

•Treatment for retinoblastoma also depends on whether the cancer has progressed or recurred.

After retinoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body.

The process used to find out if cancer has spread within the eye or to other parts of the body is called staging. The information gathered from the staging process determines whether retinoblastoma is only in the eye (intraocular) or has spread outside the eye (extraocular). It is important to know the stage in order to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease.

The following tests and procedures may be used in the staging process:

•MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain. This procedure is also called nuclear magnetic resonance imaging (NMRI).

•Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner that also takes a picture of the body. Areas of bone with cancer show up brighter in the picture because they take up more radioactive material than normal bone cells do.

EnlargeBone scan; drawing shows a child lying on a table that slides under the scanner, a technician operating the scanner, and a computer monitor that will show images made during the scan.

Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.

•Bone marrow aspiration and biopsy: The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow under a microscope to look for signs of cancer. A bone marrow aspiration and biopsy is done if the doctor thinks the cancer has spread outside of the eye.

•Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs that the cancer has spread to the brain and spinal cord and sometimes it is also checked for a tumor marker called ganglioside GD2. This procedure is also called an LP or spinal tap.

The International Retinoblastoma Staging System (IRSS) may be used for staging retinoblastoma.

There are several staging systems for retinoblastoma. The IRSS stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.

Stage 0

The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.

Stage I

The tumor is in the eye only. The eye has been removed and no cancer cells remain.

Stage II

The tumor is in the eye only. The eye has been removed and cancer cells remain that can be seen only with a microscope.

Stage III

Stage III is divided into stage IIIa and stage IIIb:

•In stage IIIa, cancer has spread from the eye to tissues around the eye socket.

•In stage IIIb, cancer has spread from the eye to lymph nodes near the ear or in the neck.

Stage IV

Stage IV is divided into stage IVa and stage IVb:

•In stage IVa, cancer has spread to one or more parts of the body, such as the bone or liver.

•In stage IVb, cancer has spread to the brain or spinal cord. It also may have spread to other parts of the body.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

•Tissue. The cancer spreads from where it began by growing into nearby areas.

•Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.

•Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

•Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.

•Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if retinoblastoma spreads to the bone, the cancer cells in the bone are actually retinoblastoma cells. The disease is metastatic retinoblastoma, not bone cancer.

Treatment for retinoblastoma depends on whether it is intraocular (within the eye) or extraocular (outside the eye).

Intraocular retinoblastoma

In intraocular retinoblastoma, cancer is found in one or both eyes and may be in the retina only or may also be in other parts of the eye such as the choroid, ciliary body, or part of the optic nerve. Cancer has not spread to tissues around the outside of the eye or to other parts of the body.

Extraocular retinoblastoma (metastatic)

In extraocular retinoblastoma, cancer has spread beyond the eye. It may be found in tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system (brain and spinal cord) or to other parts of the body such as the liver, bone, bone marrow, or lymph nodes.

Treatment for retinoblastoma also depends on whether the cancer has progressed or recurred.

Progressive retinoblastoma is cancer that continues to grow, spread or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.

Recurrent retinoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may recur in the eye, in tissues around the eye, or in other places in the body, such as the liver, bone, bone marrow, or lymph nodes.

3. Treatment Option Overview

Key Points

•There are different types of treatment for patients with retinoblastoma.

•Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

•Six types of standard treatment are used:

-Cryotherapy

-Thermotherapy

-Chemotherapy

-Radiation therapy

-High-dose chemotherapy with stem cell rescue

-Surgery (enucleation)

•New types of treatment are being tested in clinical trials.

-Targeted therapy

•Treatment for retinoblastoma may cause side effects.

•Patients may want to think about taking part in a clinical trial.

•Patients can enter clinical trials before, during, or after starting their cancer treatment.

•Follow-up tests may be needed.

There are different types of treatment for patients with retinoblastoma.

Different types of treatment are available for patients with retinoblastoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with retinoblastoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

The goals of treatment are to save the child's life, to save vision and the eye, and to prevent serious side effects. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with eye cancer and who specialize in certain areas of medicine. These may include a pediatric ophthalmologist (children's eye doctor) who has a lot of experience in treating retinoblastoma and the following specialists:

•Pediatric surgeon.

•Radiation oncologist.

•Pediatrician.

•Pediatric nurse specialist.

•Rehabilitation specialist.

•Social worker.

•Geneticist or genetic counselor.

Six types of standard treatment are used:

Cryotherapy

Cryotherapy is the use of extreme cold to destroy abnormal tissue. A small metal probe is cooled below freezing and placed on the surface of the eye near the tumor, which freezes and kills cancer cells. Cryotherapy is usually used to treat small retinoblastoma tumors that are located toward the front of the eye. This type of treatment is also called cryosurgery.

Thermotherapy

Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using a laser beam aimed through the dilated pupil or onto the outside of the eyeball. Thermotherapy may be used alone for small tumors or combined with chemotherapy for larger tumors. This treatment is a type of laser therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. The way the chemotherapy is given depends on the stage of the cancer and where the cancer is in the body.

There are different types of chemotherapy:

•Systemic chemotherapy: When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body. Systemic chemotherapy is given to shrink the tumor (chemoreduction) and avoid surgery to remove the eye. After chemoreduction, other treatments may include radiation therapy, cryotherapy, laser therapy, or regional chemotherapy. However, extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy.

Systemic chemotherapy may also be given to kill any cancer cells that are left after the initial treatment or to kill cancer cells that are outside the eye. Treatment given after the initial treatment, to lower the risk that the cancer will come back, is called adjuvant therapy.

•Regional chemotherapy: When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ (such as the eye), or a body cavity, the drugs mainly affect cancer cells in those areas. Several types of regional chemotherapy are used to treat retinoblastoma.

-Ophthalmic artery infusion chemotherapy: Ophthalmic artery infusion chemotherapy carries anticancer drugs directly to the eye. A catheter is put into an artery that leads to the eye and the anticancer drug is given through the catheter. After the drug is given, a small balloon may be inserted into the artery to block it and keep most of the anticancer drug trapped near the tumor. This type of chemotherapy may be given as the initial treatment when the tumor is in the eye only or when the tumor has not responded to other types of treatment. Ophthalmic artery infusion chemotherapy is given at special retinoblastoma treatment centers.

-Intravitreal chemotherapy: Intravitreal chemotherapy is the injection of anticancer drugs directly into the vitreous humor (jelly-like substance) inside of the eye. It is used to treat cancer that has spread to the vitreous humor and has not responded to treatment or has come back after treatment.

-Intrathecal chemotherapy: Intrathecal chemotherapy is the injection of anticancer drugs directly into the cerebrospinal fluid (CSF). It is used to treat cancer that has spread to the brain.

See Drugs Approved for Retinoblastoma for more information.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

•External-beam radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following:

-Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) external radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles.

-Proton-beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them.

•Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of internal radiation therapy may include the following:

-Plaque radiotherapy: Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.

Whether internal or external radiation therapy is given depends on the stage of the cancer being treated, where it is found in the body, and how the cancer responded to other treatments.

High-dose chemotherapy with stem cell rescue

High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Extraorbital disease requires intensive chemotherapy and may include consolidation with high-dose chemotherapy and autologous stem cell transplantation with or without radiation therapy.

See Drugs Approved for Retinoblastoma for more information.

Surgery (enucleation)

Enucleation is surgery to remove the eye and part of the optic nerve. A sample of the eye tissue that is removed will be checked under a microscope to see if there are any signs that the cancer is likely to spread to other parts of the body. This should be done by an experienced pathologist, who is familiar with retinoblastoma and other diseases of the eye. Enucleation is done if there is little or no chance that vision can be saved and when the tumor is large, did not respond to treatment, or comes back after treatment. The patient will be fitted for an artificial eye.

Close follow-up is needed for 2 years or more to check for signs of recurrence in the area around the affected eye and to check the other eye.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

•Oncolytic virus therapy: This treatment uses a virus that infects and breaks down cancer cells but not normal cells. An oncolytic adenovirus targeting the RB1 gene is injected into the tumor and may have anticancer effects in retinoblastoma. Targeted therapy is being studied for the treatment of retinoblastoma that has progressed or recurred.

Treatment for retinoblastoma may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of treatment for retinoblastoma may include the following:

•Physical problems such as seeing or hearing problems.

•If the eye is removed, there may be changes in the shape and size of the bone around the eye until an artificial eye is fitted. This is most likely to occur in children aged younger than 3 years.

•Changes in mood, feelings, thinking, learning, or memory.

•Second cancers (new types of cancer), such as lung and bladder cancers, osteosarcoma, soft tissue sarcoma, or melanoma.

The following risk factors may increase the risk of having a second cancer:

•Having the heritable form of retinoblastoma.

•Past treatment with radiation therapy, especially before age 1 year.

•Having already had a previous second cancer.

It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Regular follow-up by health professionals who are experts in diagnosing and treating late effects is important. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

In retinoblastoma, new tumors may form for a few years after treatment. Regular eye exams are done to check for tumors in both eyes. Children with the heritable form of retinoblastoma will also have imaging tests to check whether cancer has spread to the brain.

4. Treatment of Unilateral, Bilateral, and Cavitary Retinoblastoma

For information about the treatments listed below, see the Treatment Option Overview section.

If it is likely that the eye can be saved, treatment may include the following:

•Systemic chemotherapy or ophthalmic artery infusion chemotherapy, with or without intravitreal chemotherapy, to shrink the tumor. This may be followed by one or more of the following:

-Cryotherapy.

-Thermotherapy.

-Plaque radiotherapy.

•External-beam radiation therapy for bilateral intraocular retinoblastoma that does not respond to other treatments.

If the tumor is large and it is not likely that the eye can be saved, treatment may include the following:

•Surgery (enucleation). After surgery, systemic chemotherapy may be given to lower the risk that the cancer will spread to other parts of the body.

When retinoblastoma is in both eyes, the treatment for each eye may be different, depending on the size of the tumor and whether it is likely that the eye can be saved. The dose of systemic chemotherapy is usually based on the eye that has more cancer.

Treatment of cavitary retinoblastoma, a type of intraocular retinoblastoma, may include the following:

•Systemic chemotherapy or ophthalmic artery infusion chemotherapy.

5. Treatment of Extraocular Retinoblastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of extraocular retinoblastoma (cancer has spread to the area around the eye) may include the following:

•Systemic chemotherapy and external-beam radiation therapy.

•Enucleation.

•Systemic chemotherapy followed by surgery (enucleation). External-beam radiation therapy and more chemotherapy may be given after surgery.

Treatment for extraocular retinoblastoma (cancer has spread to the brain) may include the following:

•Systemic or intrathecal chemotherapy and external-beam radiation therapy to the brain and spinal cord.

•Chemotherapy followed by high-dose chemotherapy with stem cell rescue with or without radiation therapy.

It is not clear whether treatment with chemotherapy, radiation therapy, or high-dose chemotherapy with stem cell rescue helps patients with extraocular retinoblastoma live longer.

For trilateral retinoblastoma (retinoblastoma and a brain tumor that occur at the same time), treatment may include the following:

•Systemic chemotherapy followed by surgery and high-dose chemotherapy with stem cell rescue.

•Systemic chemotherapy followed by surgery and external-beam radiation therapy.

For retinoblastoma that has spread to other parts of the body, but not the brain, treatment may include the following:

•Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

6. Treatment of Progressive or Recurrent Retinoblastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of progressive or recurrent intraocular retinoblastoma may include the following:

•External-beam radiation therapy or plaque radiotherapy.

•Cryotherapy.

•Thermotherapy.

•Systemic chemotherapy or ophthalmic artery infusion chemotherapy.

•Intravitreal chemotherapy.

•Surgery (enucleation).

•A clinical trial of targeted therapy with an oncolytic virus that targets the RB1 gene and is injected into the tumor.

•A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

7. Treatment of progressive or recurrent extraocular retinoblastoma may include the following:

•Systemic chemotherapy and external-beam radiation therapy for retinoblastoma that comes back after surgery to remove the eye.

•Systemic chemotherapy followed by high-dose chemotherapy with stem cell rescue and external-beam radiation therapy.

•A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.