Primary primitive neuroectodermal tumor of the lumbar extradural space

Abstract

<jats:p>Primary spinal primitive neuroectodermal tumors (PNETs) of the extradural space are very rare; only 10 cases have been reported in the English language literature. The histopathological diagnosis of primary spinal PNETs has been discussed for many years. These tumors have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach for these patients. The authors present a case of primary PNET located in the lumbar extradural space in a 13-year-old girl and report the clinical, radiological, histopathological, and surgical findings. They compare their findings with those from the other 10 cases reported in the literature and review relevant literature.</jats:p>

Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors

“…On close examination, the pial enhancement extended inferiorly to the cervical-thoracic junction and posteriorly and superiorly to the ventral medulla and pons (Fig. 1), which suggests that a pPNET 49 18 y/M C-T Intramedullary Biopsy, RT, CT Alive at 6 PNET 2007 50 27 y/M L4-5 Extradural S(TR), CT, RT Alive at 72 pPNET 2007 50 16 y/F L2-Sa1 CE S(PR), CT, RT Alive at 5 pPNET 2007 51 19 y/F T11-L2 Extramedullary S(TR), RT, CT, ASCR Alive at 24 pPNET 2008 52 4 y/F T11-L3/4 Extradural S(subTR), RT, CT Alive at 22 ES 2008 53 27 y/M Sa1-2 CE S(TR), RT, CT Alive at 24 PNET 2008 54 13 y/F L Extradural N.A. N.A.…”

Peripheral primitive neuroectodermal tumor causing cauda equina syndrome with destruction of L5 vertebra

“…Ozdemir12 reported that out of 10 cases of extradural tumor reported before, two died after a mean period of 18 months and seven were alive at 25 months. Survival of one patient was unknown.…”

Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature

“…Primitive neuroectodermal tumors are a group of malignant neoplasms, arising from pluripotent neural crest cells [2]. Although PNET is the second malignant neoplasm in childhood, the spinal cord, as primary site for pPNET, is relatively rare in all age groups, and information concerning these tumors in the medical literature is limited to single case reports.…”

“…Immunoreactivity for synaptophysin, NSE, S100 and neurofilament, indicating neuroectodermal differentiation [4]. Cytogenetic analysis detect the presence of a reciprocal translocation t (11;22) (q24;q12), it is important to confirm the diagnosis in certain cases [2]. The differential diagnosis includes central PNET, eosinophilic granuloma, malignant meningioma, rhabdomyosarcoma, neuroblastoma, and lymphoma [4, 6].…”

“…Primary pPNETs of the spine are extremely rare. Extradural locations are even rarer [2]. Due to their low incidence, the available epidemiology is likely unreliable, and there are currently no standard clinical guidelines outlining their management [1].…”