Polyneuropathy Associated with Nerve Angiomatosis and Multiple Soft Tissue Tumors A Newly Recognized Syndrome

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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 dis-closed pathologic findings resembling those found in tumors in infantile myolibromatosis. 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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