Retinoblastoma staging
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Retinoblastoma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Retinoblastoma staging On the Web |
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American Roentgen Ray Society Images of Retinoblastoma staging |
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Risk calculators and risk factors for Retinoblastoma staging |
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 | ||||