title: Cervicomedullary intramedullary peripheral primitive neuroectodermal tumor with intratumoral bleed: Report of one case and review of literature.
abstract: Primitive neuroectodermal tumors (PNET) are highly malignant, yet relatively uncommon neoplasms of the central nervous system. Although a host of different parts of the nervous system can be affected, intramedullary location of PNET is extremely rare. Most reports on intramedullary PNET have reported central PNET (cPNET); peripheral PNET (pPNET) affecting intramedullary spinal location is extremely rare. Till now, seven such cases of intramedullary pPNET have been described in medical literature in English. Here, we report an 11-year-old boy with cervicomedullary junction intramedullary pPNET who presented with intratumoral bleed, wherein the clinical presentation and radiological features gave us no clue preoperatively about the underlying diagnosis. In this report, we additionally review certain salient aspects of this dreaded disease in light of the existing evidence.
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Pradeep
authors.initials: P
authors.lastname: Sharma
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Kuntal K
authors.initials: KK
authors.lastname: Das
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Anant
authors.initials: A
authors.lastname: Mehrotra
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Arun K
authors.initials: AK
authors.lastname: Srivastava
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Rabi N
authors.initials: RN
authors.lastname: Sahu
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Awadhesh
authors.initials: A
authors.lastname: Jaiswal
authors.affiliation: Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Rakesh
authors.initials: R
authors.lastname: Pandey
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Behari
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Kamlesh S
authors.initials: KS
authors.lastname: Bhaisora
authors.affiliation: Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
authors.forename: Jayesh
authors.initials: J
authors.lastname: Sardhara
keywords: Intramedullary; intratumoral bleed; peripheral primitive neuroectodermal tumors (pPNET); surgery