Polyneuropathy associated with nerve angiomatosis and multiple soft tissue tumors

A newly recognized syndrome

Research output: Contribution to journalArticle

3 Citations (Scopus)

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
StatePublished - 1995

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Angiomatosis
Polyneuropathies
Neoplasms
Sural Nerve
Peripheral Nerves
Blood Vessels

Keywords

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

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

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title = "Polyneuropathy associated with nerve angiomatosis and multiple soft tissue tumors: A newly recognized syndrome",
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.",
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T1 - Polyneuropathy associated with nerve angiomatosis and multiple soft tissue tumors

T2 - A newly recognized syndrome

AU - Giannini, Caterina

AU - Wright, A.

AU - Dyck, Peter J

PY - 1995

Y1 - 1995

N2 - 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.

AB - 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.

KW - Nerve angiomatosis

KW - Peripheral nerve

KW - Polyneuropathy

KW - Soft tissue tumors

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