von Recklinghausen病に合併した3重複癌の1例

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  • A Case of Triple Cancer Associated with von Recklinghausen's Disease.

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von Recklinghausen's disease (R disease) is an autosomal dominant inheritable disease, characterized by café-aulait skin macules and multiple neurofibromas. It has a variety of symptoms such as bone lesions, ophthalmologic diseases, and benign and/or malignant tumors. This paper presents a very rare case of R disease with triple cancer, breast cancer, lung cancer, and malignant gastrointestinal stromal tumor (GIST) of the small intestine.<br> A 74-year-old woman was found having an intraabdominal tumor on palpation during observation after surgery for breast and lung cancer. Abdominal CT scan visualized a solid tumor, 4cm in diameter, in the small intestine. Excision of the small intestine was performed with a diagnosis of primary small bowel tumor. Histopathological diagnosis was malignant GIST of the small intestine.<br> R disease is reportedly associated with various malignant tumors, but it is very rare that R disease is associated with triple cancer like this case. Strict follow-up observation would mandatory for patients with R disease by keeping a possibility of associated malignancies in mind.

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