A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1
<p>Abstract</p> <p>Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunctio...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2012-07-01
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| Series: | World Journal of Surgical Oncology |
| Subjects: | |
| Online Access: | http://www.wjso.com/content/10/1/153 |
| _version_ | 1818393729106444288 |
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| author | Nishi Takeshi Kawabata Yasunari Hari Youko Imaoka Hiroshi Ishikawa Noriyoshi Yano Seiji Maruyama Riruke Tajima Yoshitsugu |
| author_facet | Nishi Takeshi Kawabata Yasunari Hari Youko Imaoka Hiroshi Ishikawa Noriyoshi Yano Seiji Maruyama Riruke Tajima Yoshitsugu |
| author_sort | Nishi Takeshi |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunction and was admitted to our hospital for further examination. An abdominal contrast-enhanced computed tomography scan demonstrated a 30-mm tumor in the head of the pancreas. The scan showed an invasion of the tumor into the duodenum, and biopsy under an endoscopic ultrasonography indicated that the tumor was a NET. A subtotal stomach-preserving pancreaticoduodenectomy was performed. Macroscopically, the pancreatic tumor was white and elastic hard. Microscopically, tumor cells were composed of ribbons, cords, and solid nests with an acinus-like structure. The tumor was diagnosed as NET G2 according to the WHO classification (2010). The product of the<it>NF-1</it> gene, i.e., neurofibromin, was weakly positive in the tumor cells, suggesting that the tumor was induced by a mutation in the <it>NF-1</it> gene. This is the seventh case of PNET arising in NF-1 patients worldwide.</p> |
| first_indexed | 2024-12-14T05:49:56Z |
| format | Article |
| id | doaj.art-149a982c71a74682971ccdaa44805447 |
| institution | Directory Open Access Journal |
| issn | 1477-7819 |
| language | English |
| last_indexed | 2024-12-14T05:49:56Z |
| publishDate | 2012-07-01 |
| publisher | BMC |
| record_format | Article |
| series | World Journal of Surgical Oncology |
| spelling | doaj.art-149a982c71a74682971ccdaa448054472022-12-21T23:14:45ZengBMCWorld Journal of Surgical Oncology1477-78192012-07-0110115310.1186/1477-7819-10-153A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1Nishi TakeshiKawabata YasunariHari YoukoImaoka HiroshiIshikawa NoriyoshiYano SeijiMaruyama RirukeTajima Yoshitsugu<p>Abstract</p> <p>Patients with neurofibromatosis-1 (NF-1) sometime develop neuroendocrine tumors (NET). Although these NETs usually occur in the duodenum or peri-ampullary region, they occasionally grow in the pancreas (PNET). A 62-year-old man with NF-1 had mild liver dysfunction and was admitted to our hospital for further examination. An abdominal contrast-enhanced computed tomography scan demonstrated a 30-mm tumor in the head of the pancreas. The scan showed an invasion of the tumor into the duodenum, and biopsy under an endoscopic ultrasonography indicated that the tumor was a NET. A subtotal stomach-preserving pancreaticoduodenectomy was performed. Macroscopically, the pancreatic tumor was white and elastic hard. Microscopically, tumor cells were composed of ribbons, cords, and solid nests with an acinus-like structure. The tumor was diagnosed as NET G2 according to the WHO classification (2010). The product of the<it>NF-1</it> gene, i.e., neurofibromin, was weakly positive in the tumor cells, suggesting that the tumor was induced by a mutation in the <it>NF-1</it> gene. This is the seventh case of PNET arising in NF-1 patients worldwide.</p>http://www.wjso.com/content/10/1/153Neuroendocrine tumorNeurofibromatosis-1NeurofibrominPancreatic acinar-endocrine carcinomaPancreatic neuroendocrine tumorvon Recklinghausen’s disease |
| spellingShingle | Nishi Takeshi Kawabata Yasunari Hari Youko Imaoka Hiroshi Ishikawa Noriyoshi Yano Seiji Maruyama Riruke Tajima Yoshitsugu A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 World Journal of Surgical Oncology Neuroendocrine tumor Neurofibromatosis-1 Neurofibromin Pancreatic acinar-endocrine carcinoma Pancreatic neuroendocrine tumor von Recklinghausen’s disease |
| title | A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 |
| title_full | A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 |
| title_fullStr | A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 |
| title_full_unstemmed | A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 |
| title_short | A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1 |
| title_sort | case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis 1 |
| topic | Neuroendocrine tumor Neurofibromatosis-1 Neurofibromin Pancreatic acinar-endocrine carcinoma Pancreatic neuroendocrine tumor von Recklinghausen’s disease |
| url | http://www.wjso.com/content/10/1/153 |
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