悪性黒色腫にて死亡したWerner症候群の剖検例

概要

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We reported a patient with Werner's syndrome who developed malignant melanoma and meningioma in the head. A 56-year-old male visited our hospital because of left nasal bleeding. A biopsy specimen from the nasal tumor confirmed malignant melanoma. CT scans and MRI revealed another tumor of meningioma in the pterygopalatine fossa. He was born to consanguineous parents. He was 143cm tall,weigted 46.5kg and had keratosis in the whole body, canities, a high-pitched hoarse voice and cataract. Surgical therapy was not selected because the tumor was not completely resectable. Although he was treated with immunotherapy and chemotherapy, he died 26 months after his first visit. Autopsy revealed malignant melanomas in the nasal cavity, paranasal sinus and base of the brain, and meningiomas in the left temporal lobe and temporal mandibular space. Metastatic lesions from the malignant melanoma were observed in the cerebrum, lung and liver. Systemic arteriosclerosis in the coronary arteries and left ventricular fibrosis were present, which are characteristic findings in Werner's syndrome.
信州医学会の論文
2004-02-10