An ossified fibrolipomatous epineural hamartoma limited to the right ulnar nerve and some of its branches and extending from the elbow distally is described in a 5-year-old boy who complained of pain in the right hand. Such extensive involvement of the ulnar nerve is unusual; moreover, metaplastic bone is rarely present in the more common fibrolipomatous hamartoma of the median nerve. Increased awareness of this entity is necessary to avoid sacrifice of large nerves that may be included in the lesion.

Case report 667. Fibrolipomatous hamartoma of the median nerve.Skeletal Radiol. 1991;20(3):237-9.

Fibrolipomatous hamartoma of a nerve represents an uncommon soft-tissue tumor which typically involves the median nerve distribution near the wrist. Macrodactyly may or may not be present. The application of MRI using routine T1- and T2-weighted spin echo imaging sequences reveals serpiginous structures with low signal intensity. These structures probably represent the neural elements and associated perineural fibrosis. Further experience may allow for an accurate preoperative diagnosis of this tumor.

Fibrolipomatous hamartoma of the nerve--a rare etiology of macrodactyly. A case report.Handchir Mikrochir Plast Chir. 1999 Jan;31(1):53-6.

Fibrolipomatous hamartoma of nerve is a rare, tumor-like fibro-fatty growth arising from the epi- and perineurium. It mainly affects peripheral nerves and the median nerve in particular. An association with Klippel-Trenaunay syndrome and macrodactyly has been described. We present a 30-year-old patient, in whom the diagnosis of Klippel-Trenaunay syndrome had been made at the age of two, based on macrodactyly of the right hand. Because of increasing functional limitation and pain, the patient was referred to our department, after angiographic exclusion of Klippel-Trenaunay syndrome, for operative reduction of the tumor. Histologic examination revealed a fibrolipomatous hamartoma of the nerve. Further treatment consisted of surgical tumor reduction, which resulted in satisfactory recovery of function in the right hand. After eight months follow-up, the patient was able to perform all normal daily activities, and there was no evidence of recurrence of the lesion. Diagnostic evaluation of macrodactyly is necessary, since there may be an underlying cause such as fibrolipomatous hamartoma of a nerve, which if corrected early by surgical excision may prevent long-term disability.