Primitive Neuroectodermal Tumour in Young Adults - A Report of two Rare Cases and Review of Literature
Journal Title: International Journal of Medical Research & Health Sciences (IJMRHS) - Year 2017, Vol 6, Issue 10
Abstract
Primitive Neuro Ectodermal Tumours’ (PNET) are highly aggressive embryonal tumours of presumed neural crest origin. They are derived from neoplastic transformation of common progenitor cells in the sub ependymal matrix layer. They are more common in children. They are small round cell tumours affecting the central nervous system (CNS), others being Ewing’s sarcoma, medullobalstoma, lymphomas etc. They are classifed based on their immune histochemical characteristics- neuronal, astrocytic, ependymal, retinal photo receptor, undifferentiated. Undifferentiated variety carries better prognosis. GFAP expression is an important prognostic factor. Presence of p53 germ line mutation indicates an increased risk for developing PNET. Spinal PNET are secondary to CSF metastasis from cranium commonly. Primary spinal PNET tumours are rare and extradural location is extremely rare. PNET needs multimodality approach but carries poor prognosis when compared to other CNS tumours.
Authors and Affiliations
Vidhya Lakshmi S| Associate Professor, Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, India, Corresponding e-mail: drvidhyalakshmi@yahoo.co.in, Thirumurthy KS| Professor, Department of Neurosurgery, PSG Institute of Medical Sciences and Research, Coimbatore, India
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