Polyneuropathy associated with nerve angiomatosis and multiple soft tissue tumors: A newly recognized syndrome

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3 Scopus citations

Abstract

We report the case of a 33-year-old woman affected by a severe sensorimotor polyneuropathy and multiple soft tissue tumors since childhood. In the biopsied sural nerve, there was a striking vascular proliferation of small vessels. Three subcutaneous nodules were biopsied and disclosed pathologic findings resembling those found in tumors in infantile myofibromatosis. One of the three biopsied subcutaneous nodules was intimately contiguous with a peripheral nerve. The ultrastructural features and immunohistochemical reactivity of the soft tissue tumors and the vessels proliferating in the nerve suggested a common cellular differentiation. The finding of a diffuse polyneuropathy associated with widespread angiomatosis in nerve and multiple soft tissue tumors suggests that these are not coincidental findings, but a manifestation of a syndrome perhaps not previously recognized.

Original languageEnglish (US)
Pages (from-to)1325-1332
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume19
Issue number11
DOIs
StatePublished - Jan 1 1995

Keywords

  • Nerve angiomatosis
  • Peripheral nerve
  • Polyneuropathy
  • Soft tissue tumors

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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