Academic journal article Genetic Counseling

Lipofibromatous Hamartoma of the Median Nerve and Macrodactyly

Academic journal article Genetic Counseling

Lipofibromatous Hamartoma of the Median Nerve and Macrodactyly

Article excerpt

Summary: Lipofibromatous hamartoma of the median nerve and macrodactyly: A case of lipofibromatous hamartoma of the median nerve with macrodactyly of 2 digits is described. Nerve stripping resulted in a growth control.

Key-words: Macrodactyly - Lipofibroma - Overgrowth - Nerve

INTRODUCTION

Lipofibromatous hamartoma is a rare tumor of the nerve. The median nerve is the most involved nerve. The nerve is diffusely enlarged and normal nerve bundles can not be separated from this tumor. It is frequently associated with enlargement of finger(s) in the median nerve territory. This always opens the discussion in which way macrodactyly is nerve driven. The tumor can also be the cause of (childhood) carpal tunnel syndrome. We report a case with additional arguments for the neural influence on growth.

CASE REPORT

A 5-years-old girl in general good health consulted for macrodactyly of the third and fourth finger of the right hand (Fig. 1). The hand (and fingers) was of normal size at birth but progressively overgrowth occurred. The girl also complained of pain and "needles and pins" feelings in the hand. A large soft tissue mass was observed at the palmar side of the carpal tunnel region.

An operative procedure was planned. The carpal tunnel was opened (Fig. 2). A large soft tissue mass was found. This tumor invaded the median nerve. It was not possible to separate the nerve bundles from the tumor. Its aspect was a typical lipofibromatous hamartoma. The third finger was reduced by removing a strip of skin and subcutaneous tissue on the ulnar border. The digital nerves of the third and fourth finger were stripped. Pain and paresthesiae disappeared immediately postoperatively. The wounds healed within normal delays.

At 6-years follow-up the size of the enlarged fingers remained under control (Fig. 3). The range of motion of the fingers and the sensibility were normal.

DISCUSSION

The relationship between fibrolipomatous hamartoma and macrodactyly has been reported by several authors (1, 4, 7, 8).

Amadio et al. (2) hypothized that both macrodactyly and fibrolimatous hamartoma are both caused by a genetic abnormality in end organs. An unknown trophic factor should be responsible for both conditions.

Although, it has been postulated in 1942 (6) and in 1950 (5) that the nerve tumors and nerve dysfunction influence directly the growth process, there is no hard proof for this hypothesis. In the series of Al-Qattan (1), 6 of the cases with lipofibromatous hamartoma had genuine macrodactyly, 2 had fatty tumors and 2 had bony tumors. …

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