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Von-Recklinghausen's Disease Associated with Neurofibroma of the Stomach, a Case Report Y. Maruyama 1 1Faculty of Medicine, Shinshu University pp.889-893
Published Date 1975/7/25
DOI https://doi.org/10.11477/mf.1403112397
  • Abstract
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 A 69 years old farmer manifested typical clinical history of von Recklinghausen's disease with many café-au-lait spots, dermal neurofibromatous tumors, retarded inteligence, paresis of left trigeminus nerve, etc.

 The patient was admitted to our Hospital in April 1973 because of gastric tumor associated with continued melena and occasional hematemesis. Roentogenologically a large irregularly shaped movable polypoid lesion was detected on the relatively upper portion of the stomach. During the examination and treatment in our Hospital, the patient suddenly appeared extrahepatic jaundice and then pressing operation was done to resect the large gastric tumor. A large myxoid and polypoid lesion had developed on the cardiac mucosa and extended to the second portion of the duodenum through the pyloric ring pressing upon the Vater's pailla by edematously swollen and hemorrhagic head of the lesion. The lesion was completely resected together with basic gastric wall but the patient died after 3 weeks by marked liver metastases and decreased general condition. Microscopic examination revealed that many neurofibromas and dysplastic conditions of peripheral nerve developing on the gastrointestinal canal and some malignant lesions developed on them in the stomach as described above and the jejunum. Anaplastic spindle-shaped or satellite-type sarcoma cells loosely developing with rich myxoid stroma and basement membrane associatedly developed with these malignant cells on electrono-microscopic appearance suggest these cells as nervous origin. Associatedly insuloma was also observed ectopically at the duodenal wall and clinically hypoglycemia was suspected.


Copyright © 1975, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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