von Recklinghausen 病に合併した縦隔dumbbell型神経線維腫の1例

概要

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A 10-year-old boy suffering from von Recklinghausen's disease complained of chest pain with common cold symptoms. Detailed examinations revealed dumbbell-type tumor located in the left upper mediatinum. There were no neurologic symptoms. Transthoracic excision of the tumor was successfully achieved. The tumor possibly origi-nated from sympathic trunk intruding through the 3rd and 4th thoracic intervertebral foramina. Histological examination disclosed neurofibroma. Post-operative course was uneventful. Mediastinal dumbbell-type neurofibroma is very rare affection of von Recklinghausen's disease and only 5 cases were found in the literature. In this paper it is emphasized that early diagnosis and treatment are invariably necessary in order to avoid irreversible neurologic disorders secondary to dumbbell-type tumor, although its affection is rare in von Reckling-hausen's disease.
日本小児外科学会の論文
1987-08-20