Pinealoblastoma

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Pinealoblastoma
Classification and external resources
Specialty oncology
ICD-10 C75.3
ICD-O 9362/3
MeSH D010871

Pineoblastoma (also pinealoblastoma) is a malignant tumor of the pineal gland. A pineoblastoma is a supratentorial midline primitive neuroectodermal tumor.

Pineoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma. When retinoblastoma patients present with pineoblastoma this is characterized as "trilateral retinoblastoma".[1] Up to 5% of patients with hereditary retinoblastoma are at risk of developing trilateral retinoblastoma.[2] 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.[3] Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma to up to 50%.[4]

References[edit]

  1. ^ Provenzale JM, Weber AL, Klintworth GK, McLendon RE (January 1995). "Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma)". AJNR Am J Neuroradiol. 16 (1): 157–65. PMID 7900586. 
  2. ^ de Jong, Marcus C.; Kors, Wijnanda A.; de Graaf, Pim; Castelijns, Jonas A.; Moll, Annette C.; Kivelä, Tero (2015-12-01). "The Incidence of Trilateral Retinoblastoma: A Systematic Review and Meta-Analysis". American Journal of Ophthalmology. 160 (6): 1116–1126.e5. doi:10.1016/j.ajo.2015.09.009. ISSN 1879-1891. PMID 26374932. 
  3. ^ 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. 
  4. ^ 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.