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Year : 2019  |  Volume : 14  |  Issue : 4  |  Page : 228-231

Do Glioblastomas with Syndromic Association Have Better Prognosis? A Case of Supratentorial Glioblastoma with Embryonal Tumor Differentiation in a Child with Multiple Enchondromatosis

1 Department of Neurosurgery, Shatabdi Hospital Phase 2, King George Medical University, Lucknow, Uttar Pradesh, India
2 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Pathology, King George Medical University, Lucknow, Uttar Pradesh, India

Correspondence Address:
Dr. Siddharth Vankipuram
Department of Neurosurgery, Shatabdi Hospital Phase 2, King George Medical University, Lucknow 226003, Uttar Pradesh.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_82_19

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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.


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