Radiosurgical treatment of ulnar plexiform neurofibroma in a neurofibromatosis type 1 (NF1) patient.

摘要

Plexiform neurofibromas (PNFs) are among the most disabling complications of neurofibromatosis type 1 (NF1).Surgery is considered the standard therapy for PNFs, but a gross total removal cannot always be achieved and the long-term local control appears to be less than optimal [11]. There are no other currently accepted medical treatments for PNFs.We report the case of a 50-year-old woman affected by NF1, and treated with radiosurgery for an ulnar nerve PNF.When she was first admitted to our hospital, she suffered from increasing numbness, with paraesthesia and neuropathic pain along the ulnar nerve territory. A left ulnar nerve PNF was demonstrated by a contrast-enhanced MRI. The neurological examination also revealed a moderate hypoesthesia along the ulnar territory, a mild hypotrophy of the first left posterior interosseous muscle, and a left-hand motor impairment.