Primitive neuroectodermal tumor of the median nerve Case report with cytogenetic analysis

Abstract

The authors describe a malignant peripheral primitive neuroectodermal tumor (PNET) that originated in the median nerve in an elderly adult. After the diagnosis was made by biopsy, the patient underwent radical local resection with interpositional vein grafting of the brachial artery. The tumor had the typical appearance of a primitive neural tumor with small, round cells forming rosettes. It stained positively for both the Ewing's sarcoma/peripheral PNET antigen (HBA-71) and neuron-specific enolase, confirming its neural origin. Ultrastructural examination revealed dense core granules and suggested neural differentiation of the neoplasm. Cytogenetic analysis suggested a chromosome (11;22) translocation typical of peripheral PNET. Early reports consisted of tumors arising solely in peripheral nerves, but recent series have focused mainly on tumors arising in the soft tissues other than nerves. There are no other cases of true PNET of peripheral nerve in the modern literature that have been fully characterized by immunohistochemical, ultrastructural, and cytogenetic criteria. Although peripheral PNETs occur more commonly in children, this unusual neoplasm should be considered in the differential diagnosis of peripheral nerve neoplasms in adults. Early diagnosis is desirable because of its aggressive nature and poor outcome.

Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina

“…Recent studies of the tumours, however, have focused mainly on the lesions in soft tissues or bones without close relation to peripheral nerves. Recently, Akeyson et al reviewed 36 cases of the tumours involving the peripheral nerves in the literature [2]. PNET arising in the cauda equina is rare, and only 6 cases have been reported previously [12,14,15,25].…”

Extra-osseous Ewing′s sarcoma of sciatic nerve masquerading as an infected hemangioma: A rare case report

“…[ 11 ] Aggressive surgical approach had been advocated by most of the earlier cases reported in adults. [ 7 8 ] Similarly, in our case, complete excision was done by sacrificing the involved nerve segment. The gap was bridged by grafting a segment of peroneal nerve.…”

“…Extensive search of the English literature revealed only isolated case reports in the adult age group. [ 6 7 8 9 ] The handful of reports also give credence to the fact that intra-neural EES outside the cranio-spinal vault is very rarely reported.…”

Ewing Sarcoma in the Sciatic Nerve: Case Report

“…11 Reports of other cases of ESFT originating in the nerves are scarce. Nesbitt and Vidone 12 reported a case of PNET of the sciatic nerve in a 6-year-old patient; Martins et al 13 reported a case of PNET arising from a branch of the sciatic nerve in a 17-year-old female patient; and Akeyson et al 14 described a case of PNET originating in the median nerve of an elderly patient. They are typically fast-growing tumors, usually painful.…”