Lipofibromatous Hamartoma of the Median Nerve の1例  [in Japanese] A Case of Lipofibromatous Hamartoma of the Median Nerve  [in Japanese]

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Abstract

Lipofibromatous hamartoma of nerve(神経内線維脂肪性過誤腫: 以下LFHと略す)は末梢神経の線維脂肪性腫大を特徴とした稀な疾患で特に正中神経に好発し,臨床的に四肢末梢皮下のびまん性腫脹を特徴としている。今回16歳男性の正中神経より指神経に発生し,手掌より示指にかけて腫脹を主訴とした本症の典型例を経験したので報告した。LFHは四肢末梢の神経の間質成分である線維組織と脂肪組織が非腫瘍性に増殖した過誤腫といわれ,1953年Masonにより初めて報告されて以来国内外で約100例の報告がある。診断はCTやMRI等の画像診断が有効であるといわれているが,実際は生検によることが多い。治療法は1. 顕微鏡下脂肪腫摘出,2. 神経·腫瘍摘出と神経吻合あるいは神経移植,3. 神経·腫瘍摘出,4.手根管症候群の場合は手根管開放と神経被膜切開による除圧などがある。今回は神経·腫瘍の部分切除を行ったが,術後に懸念されていた神経脱落症状は認められなかった。

Lipofibromatous hamartoma of the nerve (LFH) is a rare benign tumor-like nodule characterized by an overgrowth of fibrous and adipose tissue of the peripheral nerve. It frequently affects the median nerve, and is clinically characterized by peripheral subcutaneous diffuse swelling of the extremities. I describe a 16-year-old male adolescent who presented with the main complaint of swelling from the palm to the index finger and was diagnosed to have LFH which affected the median and index digital nerve. LFH is considered to be due to a non-neoplastic proliferation of fibrous and adipose tissues, which is also composed of the peripheral nerves in the extremities. Since Mason reported the first case in 1953, about 100 cases have been reported in various countries, including Japan. Diagnostic imaging methods, such as CT or MRI, have been suggested to be useful for the diagnosis of LFH, although its actual diagnosis is often made based on the intra-operative and histopathological findings. Various therapies have been proposed, as follows: a microscopic resection of the nodule; a resection of the nerve and nodule followed by neuroanastomosis or nerve grafting; a resection of the nerve and nodule; and in the case of carpal tunnel syndrome, decompression by releasing the carpal tunnel. A partial excision of the nerve and nodule was performed in this case, and no serious neurological deficiency symptom was observed after surgery.

Journal

  • The Nishinihon Jourrnal of Dermatology  

    The Nishinihon Jourrnal of Dermatology 64(2), 187-189, 2002-04-01 

    Western Division of Japanese Dermatological Association

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