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