Nevoid basal cell carcinoma syndrome の1例

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We report a typical case of nevoid basal cell carcinoma syndrome (NBCCS) . The patient, a 71-year-old woman, visited us because of deformation of the lower eyelid due to ulcerated black lesions on the cheek. She had undergone surgery on thelower jawbone at the age of 38, and four black nodules on the face had been removed surgically at the age of 48. Her family history was non-contributory. At presentation, she had a large tumor measuring 60×56×25mm on the scalp and more than 80 black tumors on the head, trunk, buttocks, genitals and thigh. We resected 34 of the tumors, which were shown microscopically to be basal cell carcinoma. As well as the multiple basal cell carcinomas, the patient showed calcification of the falx, palmar pits, mandibular cysts, hypertelorism, frontal and temporal bossing and mild mandibular prognathism. The NBCCS tumors showed no aggressive malignancy. We intend to treat the remaining small tumors with liquid nitrogen and the large tumors by surgery. [Skin Cancer (Japan) 2001; 16: 104-109]
日本皮膚悪性腫瘍学会の論文
2001-04-20