A small intraneural epithelioid malignant peripheral nerve sheath tumour of the median nerve simulating a benign lesion. Description of a case and review of the literature

Domenico Corradi, Sara Alquati, Franco Bertoni, Veronica Bartoli, Angelo Paolo Dei Tos, Doris Wenger, Caterina Giannini

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

The epithelioid variant of malignant peripheral nerve sheath tumours (MPNSTs) is a very rare malignancy. We describe the case of a 30-year-old man complaining of acute pain in his right elbow, mild distal paraesthesias, and some motor deficiencies. He was discovered as having a small fusiform swelling of the median nerve. In view of its very small size, shape, and nonspecific MRI signal, it had initially suggested a benign lesion. The diagnosis of epithelioid MPNST was made only at the histopathological examination. This malignant neoplasm recurred locally fourteen months after surgery. In addition to describe the above very rare case, we have reviewed the literature on epithelioid MPNSTs clearly involving deep major nerve trunks. This case serves as a warning that, even in major nerve trunks, tiny lesions may in reality be early intraneural MPNSTs which, due to their deep location, must be treated adequately with wide margin surgery since the resection margin status represents one of the major parameters influencing the local control of disease and its clinical outcome.

Original languageEnglish (US)
Pages (from-to)101-106
Number of pages6
JournalPathology and Oncology Research
Volume18
Issue number1
DOIs
StatePublished - Jan 2012

Keywords

  • Epithelioid variant
  • Immunohistochemistry
  • Malignant peripheral nerve sheath tumour
  • Outcome
  • Surgical margins

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Oncology
  • Cancer Research

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