Trilateral retinoblastoma

Trilateral retinoblastoma
Classification and external resources

Trilateral retinoblastoma (TRb) is a malignant midline primitive neuroectodermal tumor occurring in patients with inherited uni- or bilateral retinoblastoma. In most cases trilateral retinoblastoma presents itself as pineoblastoma (pineal TRb). In about a fourth of the cases the tumor develops in another intracranial region, most commonly supra- or parasellar (non-pineal TRb), but there are reported cases with non-pineal TRb in the 3rd ventricle. In most cases pineal TRb is diagnosed before the age of 5, but after the diagnosis of retinoblastoma. Non-pineal TRb, however, is often diagnosed simultaneous with retinoblastoma. Prognosis of patients with trilateral retinoblastoma is dismal, only a few patients have survived more than 5 years after diagnosis; all survivors were diagnosed with small tumors in a subclinical stage.[1] Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma.[2]

References

  1. ↑ Kivelä T (June 1999). "Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma". Journal of Clinical Oncology 17 (6): 1829–37. PMID 10561222.
  2. ↑ De Jong MC, Kors WA, De Graaf P, Castelijns JA, Kivelä T, Moll AC (September 2014). "Trilateral retinoblastoma: a systematic review and meta-analysis". The Lancet Oncology 15 (10): 1157–67. doi:10.1016/s1470-2045(14)70336-5. PMID 25126964.

See also


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