悪性化を示したRecklinghausen氏病の1例

VON RECKLINGHAUSEN'S DISEASE WITH MALIGNANT CHANGE

I．はじめに

　悪性化したRecklinghausen氏神経線維腫症の報告は少なく，本邦では未だ20数例を数えるにすぎない。

　私達は最近，全身性皮膚腫瘤のうち左側胸壁のものが悪性化を示した1例を経験したので報告する。

Reported cases of Von Recklinghausen's disease with malignant change in Japan were rare.Because the tumor extends to just under the epidermis, it should be removed as a rather large mass including surrounding tissues and skin as early as possible.

A case of this disease on the left chest in a 43-year-old man was reported. The histologic picture was pleomorphic and showed mucinomatous, schwanomatous, or fibrosarcomatous appear-ance. It had increased nuclear atypicality, mitotic figure and cellular elements. There were no wavy structure of fibrils, glomerular arrangement of tumor cells, bundle structure of nerve fiber and skin appendages etc. as in benign one. The bundle of fibrils was thin with pleomorphic orientation and structure. It was reddish yellow or yellow by Van Gieson's stain, weakly posi-tive by PAS stain, slightly metachromatic by toluidine-blue. There were many mast cells in the part with abundant fibrous elements, but very few in the remaining area. The orientation of increased argyrophilic fibers by Gomori's stain was irregular and their wavy figure became more even. Bundles of nerve fibers as in the benign tumor were not proved by Bodian's stain.

Histologic specimen from the small skin tumor, which appeared benign clinically, also included parts rich in cellular elements and showed latent tendency to malignancy.