A Case of von Recklinghausen Disease Associated with Adenocarcinoma, Multiple Carcinoid, Neurofibromatosis, and Ganglioneuromatosis of Rectum.
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- Kamiji I.
- Surgery, Hosogi Hospital
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- Kitamura M.
- Surgery, Hosogi Hospital
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- Misawa A.
- Surgery, Hosogi Hospital
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- Nakayama H.
- First Department of Pathology, Kochi University School of Medicine
Bibliographic Information
- Other Title
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- 直腸にAdenocarcinoma,Multiple Carcinoid,Neurofibromatosis,Ganglioneuromatosisを合併したvon Recklinghausen病の1例
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Abstract
A 39-year-old male with multiple small soft tumors and cafe au lait spots over the entire body was admitted to our hospital because of bloody stool. Endoscopic examination revealed multiple polypoid lesions of the rectum. The histologic diagnosis of the specimen by polypectomy was adenocarcinoma (signet ring cell type). An ultrasound and a CTscan showed no metastatic tumor. Abdominoperineal resection of the rectum with regional lymph node resection was performed. The resected specimen revealed various states of tissue, adenocarcinoma, multiple carcinoids, neurofibromatosis, and ganglioneurofibromatosis of the rectum. von Recklinghausen disease (VRD) is an autosomal dominant inheritant disease which is characterized by cafe au lait spots and multiple neurofibromas, of the skin and peripheral nerves. Sometimes the association of nonepithelial tumors of nerve origin in the gastrointestinal organs has been reported, but that of gastrointestinal epithelial tumor has been rarely seen. To our knowledge, VRD associated with adenocarcinoma (signet ring cell type), multiple carcinoid, neurofibromatosis, and ganglioneuromatosis of the rectum has not been previously reported.
Journal
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- Nippon Daicho Komonbyo Gakkai Zasshi
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Nippon Daicho Komonbyo Gakkai Zasshi 51 (7), 485-489, 1998
The Japan Society of Coloproctology
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Keywords
Details 詳細情報について
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- CRID
- 1390001204855108480
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- NII Article ID
- 10007908436
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- NII Book ID
- AN00195100
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- ISSN
- 18829619
- 00471801
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- Text Lang
- ja
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- Data Source
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- JaLC
- Crossref
- CiNii Articles
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- Abstract License Flag
- Disallowed