Pinealoblastoma

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Pinealoblastoma
Other namesPineoblastoma
Specialty Oncology   OOjs UI icon edit-ltr-progressive.svg

Pinealoblastoma is a malignant tumor of the pineal gland. A pinealoblastoma is a supratentorial midline primitive neuroectodermal tumor.[ citation needed ]

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]

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Trilateral retinoblastoma 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. In about a fourth of the cases the tumor develops in another intracranial region, most commonly supra- or parasellar, 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. Recent advances in (high-dose) chemotherapy treatment regimens and early detection have improved survival of patients with trilateral retinoblastoma.

Santosh Gajanan Honavar is a renowned Indian ophthalmologist and is currently the editor of the Indian Journal of Ophthalmology and Indian Journal of Ophthalmology - Case Reports, the official journals of the All India Ophthalmological Society; Director, Medical Services ; Director, Department of Ocular Oncology and Oculoplasty at Centre for Sight, Hyderabad; and Director, National Retinoblastoma Foundation. A former head of the Department of Ophthalmic Plastic Surgery and Ocular Oncology and associate director at L. V. Prasad Eye Institute, he is known for his research on retinoblastoma. The Council of Scientific and Industrial Research, the apex agency of the Government of India for scientific research, awarded him the Shanti Swarup Bhatnagar Prize for Science and Technology, one of the highest Indian science awards, for his contributions to Medical Sciences in 2009.. He is counted among the top 2% of world researchers and among the top 10 Indian ophthalmologists in research. Dr Honavar is the only Indian Ophthalmologist to receive the Lifetime Achievement Award by the American Academy of Ophthalmology and the Honorary Fellowship of the Royal College of Ophthalmologists, London, UK.

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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. hdl: 10138/223832 . 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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