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

Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).

Staging

The International Classification for Intraocular Retinoblastoma, based upon the Philadelphia and Murphree systems,[1] [2][3] is currently the classification being used by the Children's Oncology Group in multicenter treatment protocols [4][3]. The goal of International Classification of Retinoblastoma (ICRB) is to reflect the likelihood of ocular survival based on modern treatment techniques such as chemotherapy and focal therapy. The International Classification of Retinoblastoma (ICRB) was developed to better predict those with intraocular retinoblastoma who are likely to be cured without the need for external-beam radiation treatment or enucleation.

The International Retinoblastoma Staging System (IRSS)

Stage Features
Stage 0
  • Patients treated conservatively
Stage I
  • eye enucleated, completely resected histologically
Stage II
  • eye enucleated, microscopic residual tumor
Stage III
Stage III A
Stage III B
  • regional extension
  • overt orbital disease
  • preauricular or cervical lymph node extension
Stage IV
Stage IV A




Stage IV B


  • Metastasis
  • haematogeneous metastasis without CNS involvement
  • single lesion
  • multiple lesions
  • preauricular or cervical lymph node extension
  • CNS metastatic involvement
  • prechiasmatic lesion
  • CNS mass
  • leptomeningeal disease

References

  1. Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D (1997). "Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes". Oncologist. 2 (1): 1–5. PMID 10388023.
  2. Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA (2004). "Practical approach to management of retinoblastoma". Arch Ophthalmol. 122 (5): 729–35. doi:10.1001/archopht.122.5.729. PMID 15136321.
  3. 3.0 3.1 Linn Murphree A (2005). "Intraocular retinoblastoma: the case for a new group classification". Ophthalmol Clin North Am. 18 (1): 41–53, viii. doi:10.1016/j.ohc.2004.11.003. PMID 15763190.
  4. Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT; et al. (2006). "The International Classification of Retinoblastoma predicts chemoreduction success". Ophthalmology. 113 (12): 2276–80. doi:10.1016/j.ophtha.2006.06.018. PMID 16996605.

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See also


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