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- Title
Do Glioblastomas with Syndromic Association Have Better Prognosis? A Case of Supratentorial Glioblastoma with Embryonal Tumor Differentiation in a Child with Multiple Enchondromatosis.
- Authors
Vankipuram, Siddharth; Sahoo, Sushant; Bhalla, Shalini; Srivastava, Chittij
- Abstract
Glioblastoma (GBM) is an aggressive cancerous neoplasm of the brain that has numerous morphological subtypes. Primitive neuroectodermal differentiation (hereafter, referred to as embryonal tumor [ET] differentiation) in GBM is one of them and is known to occur in adults. Their presentation in pediatric population is rare and can be a source of diagnostic confusion. The dual pathology leads to doubts where one could ask whether it is ET differentiation in GBM specimen or glial differentiation in ET specimen. This histological discrimination has a bearing on the treatment regimens and prognosis. We report a case of a 10-year-old boy presenting with a supratentorial GBM, isocitrate dehydrogenase wild type with ET differentiation, and multiple benign bony lesions of both extremities. He underwent surgical excision for the brain neoplasm followed by radiotherapy and has shown prolonged survival with no recurrence. In this article, we discuss prognostic factors associated with long-term survival of these tumors.
- Subjects
GLIOMA treatment; ARM; ADJUVANT treatment of cancer; CRANIOTOMY; GLIOMAS; HISTOLOGICAL techniques; LEG; SUPRATENTORIAL brain tumors; SURVIVAL; TREATMENT effectiveness; ENCHONDROMATOSIS; CHEMORADIOTHERAPY; CHILDREN
- Publication
Journal of Pediatric Neurosciences, 2019, Vol 14, Issue 4, p228
- ISSN
1817-1745
- Publication type
Article
- DOI
10.4103/jpn.JPN_82_19