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

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.