Epithelioid malignant peripheral nerve sheath tumour of the ulnar nerve around the elbow: a diagnostic and therapeutic challenge

BMJ Case Rep. 2023 Nov 24;16(11):e256900. doi: 10.1136/bcr-2023-256900.


Epithelioid malignant peripheral nerve sheath tumour (EMPNST) is a rare histological subtype of malignant peripheral nerve sheath tumour (MPNST), accounting for 5% to 17% of MPNSTs. The clinical and MRI findings of EMPNST mimic those of nerve abscesses, similar to the presentation in Hansen's disease. We present one such case with this kind of diagnostic dilemma. Intraoperative findings suggest a tumour changed the course of management subsequently. The development of neurological deficits postoperatively after tumour resection was a reconstructive challenge. To provide motor power and sensation through a procedure that provides a complete functional outcome for a young patient, distal nerve transfers were chosen. This provided an improvement in the quality of life and hastened the neurological recovery of the involved limb. Level of evidence: V.

Keywords: pain (neurology); pathology; peripheral nerve disease.

Publication types

  • Case Reports

MeSH terms

  • Elbow / pathology
  • Humans
  • Nerve Sheath Neoplasms* / diagnosis
  • Nerve Sheath Neoplasms* / pathology
  • Nerve Sheath Neoplasms* / surgery
  • Neurofibrosarcoma*
  • Quality of Life
  • Skin Neoplasms*
  • Ulnar Nerve / pathology
  • Ulnar Nerve / surgery