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Pineoblastoma

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Pinealoblastoma
SpecialtyOncology Edit this on Wikidata

Pinealoblastoma or pineoblastoma is a malignant tumor of the pineal gland. A pinealoblastoma is a supratentorial midline primitive neuroectodermal tumor.

Pinealoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma. When retinoblastoma patients present with pinealoblastoma 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

  1. ^ Provenzale JM, Weber AL, Klintworth GK, McLendon RE (January 1995). "Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma)". AJNR. American Journal of Neuroradiology. 16 (1): 157–65. PMID 7900586.
  2. ^ de Jong MC, Kors WA, de Graaf P, Castelijns JA, Moll AC, Kivelä T (December 2015). "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. 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. doi:10.1200/JCO.1999.17.6.1829. 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.


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