Lipofibromatous Hamartoma and Related Peripheral Nerve Lesions

Darian S. Kameh, MD, Juan Luis Perez-Berenguer, MD, Gary S. Pearl, MD, PhD, Department of Pathology, Orlando Regional Healthcare System, Orlando, Fla.

South Med J. 2000;93(8) 

In This Article

Abstract and Introduction

Three unusual cases of a rare, tumor-like condition, lipofibromatous hamartoma, are reported. This lesion is composed of fibrous and fatty tissue that infiltrates peripheral nerves, typically on the volar aspect of the upper extremities of children and young adults. All three patients had a painless soft tissue mass of the wrist and/or hand, which followed nerve distribution, and only one patient had neurologic symptoms due to compression. Diagnosis was made by open biopsy and histologic examination.

Lipofibromatous hamartoma, an unusual tumor-like lesion, has also been previously reported as neurolipomatosis, neural fibrolipoma, and macrodystrophia lipomatosa (lipofibromatous hamartoma with localized gigantism).[1,2,3,4,5] In these three cases, the patients had typical presentations, and all had surgery for definitive diagnosis by biopsy and/or symptomatic relief.

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