Retinoblastoma staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

According to the International classification of retinoblastoma, there are five stages of retinoblastoma based on the tumor size, location, local spread, neovascular glaucoma, intravascular hemorrhage, and orbital cellulitis.

Staging

• There is two known staging system for retinoblastoma:
  • American Joint Committee on Cancer (AJCC) staging system
  • International Retinoblastoma Staging System
    • This staging system is used in the clinical setting greater than the AJCC staging system.

American Joint Committee on Cancer (AJCC) staging system

Definition of primary tumour (cT)
Stage	Sub-stage	Finding
cTX		Unknown evidence of intraocular tumour
T1a	Ia	Growth limited to one ovary; no ascites present containing malignant cells. No tumor on the external surface; capsule intact
T1b	Ib	Growth limited to one or both ovaries; no ascites present containing malignant cells. No tumor on the external surfaces; capsules intact
T1c	Ic	Tumor either stage Ia or Ib, but with the tumor on surface of one or both ovaries, or with capsule ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings
T2	II	Growth involving one or both ovaries with pelvic extension
T2a	IIa	Extension and/or metastases to the uterus and/or tubes
T2b	IIb	Extension to other pelvic tissues
T2c	IIc	Tumor either stage IIa or IIb, but with the tumor on surface of one or both ovaries, or with capsule(s) ruptured, or with ascites present containing malignant cells, or with positive peritoneal washings
T3	III	Tumor involving one or both ovaries with histologically confirmed peritoneal implants outside the pelvis and/or positive regional lymph nodes. Superficial liver metastases equal stage III. The tumor is limited to the true pelvis, but with histologically proven malignant extension to small bowel or omentum
T3a	IIIa2	Tumor grossly limited to the true pelvis with histologically confirmed microscopic seeding of abdominal peritoneal surfaces, or histologic proven extension to small bowel or mesentery with or without positive retroperitoneal lymph nodes
T3b	IIIb	Tumor of one or both ovaries with histologically confirmed implants, peritoneal metastasis of abdominal peritoneal surfaces, none exceeding 2 cm in diameter with or without extension metastasis to the retroperitoneal lymph nodes
T3c	IIIc	Peritoneal metastasis beyond the pelvis >2 cm in diameter with or without metastasis to the retroperitoneal lymph nodes (includes the extension of tumor to the capsule of liver and spleen without parenchymal involvement of either organ)
AnyT	IV	Growth involving one or both ovaries with distant metastases. If pleural effusion is present, there must be positive cytology to allow a case to stage IV. Parenchymal liver metastasis equals stage IV.
Regional lymph node involvement
Node involvement	FIGO stage	Finding
N0		No lymph node involvement
N0(i+)		Tumor cells are present in regional lymph node(s), no greater than 0.2 mm
N1	IIIA1	Histologically confirmed retroperitoneal lymph nodes
N1a	IIIA1i	Metastasis <and= 10 mm in greatest dimension
N1b	IIIA1ii	Metastasis more than 10 mm in greatest dimension
Distant metastasis
Presence of metastasis	FIGO stage	Finding
M0		No distant metastasis
M1	IV	Distant metastasis, including positive cytology of pleural effusion; liver or splenic parenchymal metastasis; metastasis to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside the abdominal cavity); and transmural involvement of intestine
M1a	IVa	Pleural effusion with positive cytology
M1b	IVb	Liver or splenic parenchymal metastases; metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside the abdominal cavity); transmural involvement of intestine

International Retinoblastoma Staging System

Stage	Description
Stage 0	Eye enucleated and no dissemination of the disease
Stage I	Eye enucleated, completely resected histologically
Stage II	Eye enucleated, microscopic residual tumor
Stage III	Regional extension			a.Overt orbital disease b.Preauricular or cervical lymph node extension
Stage IV	Metastatic disease			a.Hematogenous metastasis (without CNS involvement) —Single lesion —Multiple lesions b.CNS extension (with or without any other site of regional or metastatic disease) —Prechiasmatic lesion —CNS mass —Leptomeningeal and CSF disease