Hamartoma compress medial and radial nerve in neurofibromatosis type 1.

UNLABELLED

Hamartoma of soft tissue in the extremity is uncommon. We report a 36-year-old female patient with neurofibromatosis type 1 (NF-1). She had had a gradually increased mass in her right forearm and wrist for ten years. The mass increased suddenly after the trauma. As a result, she felt pain and progressive numbness of forearm and hand. Her radial and median nerves were compressed by the mass. We resected the tumor and released the compression of nerves. After the surgery, the patient gained neural functional recovery. Two years after surgery, she had no tumor recurrence. We discuss its clinical diagnosis, radiological features, MRI findings, pathophysiology, and treatment.

BACKGROUND

Hamartoma consists of multiple tissue elements. It can develop a bony disorder, rupture the blood vessels and create altered hemodynamics. NF-1 is caused by the mutation of a gene on chromosome 17 that is responsible for the control of cell division. It is also known as von Recklinghausen's disease. No case of the radial and median nerves that are compressed by the forearm hamartoma in NF-1 has been described in the literature before.