Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report
<p>Abstract</p> <p>Background</p> <p>Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder with characteristic features of skin and central nervous system involvement. Gastrointestinal involvement is rare, but the risk of malignancy development is co...
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| Format: | Article |
| Language: | English |
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BMC
2005-07-01
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| Series: | BMC Cancer |
| Online Access: | http://www.biomedcentral.com/1471-2407/5/85 |
| _version_ | 1818556215922262016 |
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| author | Choi Sung-Kyu Cho Chol-Kyoon Kim Hyun-Soo Joo Young-Eun Park Chang-Hwan Kim Jung-Chul Koh Yang-Seok Lee Wan-Sik Rew Jong-Sun Kim Sei-Jong |
| author_facet | Choi Sung-Kyu Cho Chol-Kyoon Kim Hyun-Soo Joo Young-Eun Park Chang-Hwan Kim Jung-Chul Koh Yang-Seok Lee Wan-Sik Rew Jong-Sun Kim Sei-Jong |
| author_sort | Choi Sung-Kyu |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder with characteristic features of skin and central nervous system involvement. Gastrointestinal involvement is rare, but the risk of malignancy development is considerable. Zollinger-Ellison syndrome is caused by gastrin-secreting tumors called gastrinomas. Correct diagnosis is often difficult, and curative treatment can only be achieved surgically.</p> <p>Case presentation</p> <p>A 41-year-old female affected by neurofibromatosis type 1 presented with a history of recurrent epigastric soreness, diarrhea, and relapsing chronic duodenal ulcer. Her serum fasting gastrin level was over 1000 pg/mL. An abdominal CT scan revealed a 3 × 2-cm, well-enhanced mass adjacent to the duodenal loop. She was not associated with multiple endocrine neoplasia type 1. Operative resection was performed and gastrinoma was diagnosed by immunohistochemical staining. The serum gastrin level decreased to 99.1 pg/mL after surgery, and symptoms and endoscopic findings completely resolved without recurrences.</p> <p>Conclusion</p> <p>Gastrinoma is difficult to detect even in the general population, and hence symptoms such as recurrent idiopathic peptic ulcer and diarrhea in neurofibromatosis type 1 patients should be accounted for as possibly contributing to Zollinger-Ellison syndrome.</p> |
| first_indexed | 2024-12-13T23:44:31Z |
| format | Article |
| id | doaj.art-b708a213d2174f138ed5f7e24c0764a7 |
| institution | Directory Open Access Journal |
| issn | 1471-2407 |
| language | English |
| last_indexed | 2024-12-13T23:44:31Z |
| publishDate | 2005-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj.art-b708a213d2174f138ed5f7e24c0764a72022-12-21T23:27:03ZengBMCBMC Cancer1471-24072005-07-01518510.1186/1471-2407-5-85Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case reportChoi Sung-KyuCho Chol-KyoonKim Hyun-SooJoo Young-EunPark Chang-HwanKim Jung-ChulKoh Yang-SeokLee Wan-SikRew Jong-SunKim Sei-Jong<p>Abstract</p> <p>Background</p> <p>Neurofibromatosis type 1 is an autosomal dominant neurocutaneous disorder with characteristic features of skin and central nervous system involvement. Gastrointestinal involvement is rare, but the risk of malignancy development is considerable. Zollinger-Ellison syndrome is caused by gastrin-secreting tumors called gastrinomas. Correct diagnosis is often difficult, and curative treatment can only be achieved surgically.</p> <p>Case presentation</p> <p>A 41-year-old female affected by neurofibromatosis type 1 presented with a history of recurrent epigastric soreness, diarrhea, and relapsing chronic duodenal ulcer. Her serum fasting gastrin level was over 1000 pg/mL. An abdominal CT scan revealed a 3 × 2-cm, well-enhanced mass adjacent to the duodenal loop. She was not associated with multiple endocrine neoplasia type 1. Operative resection was performed and gastrinoma was diagnosed by immunohistochemical staining. The serum gastrin level decreased to 99.1 pg/mL after surgery, and symptoms and endoscopic findings completely resolved without recurrences.</p> <p>Conclusion</p> <p>Gastrinoma is difficult to detect even in the general population, and hence symptoms such as recurrent idiopathic peptic ulcer and diarrhea in neurofibromatosis type 1 patients should be accounted for as possibly contributing to Zollinger-Ellison syndrome.</p>http://www.biomedcentral.com/1471-2407/5/85 |
| spellingShingle | Choi Sung-Kyu Cho Chol-Kyoon Kim Hyun-Soo Joo Young-Eun Park Chang-Hwan Kim Jung-Chul Koh Yang-Seok Lee Wan-Sik Rew Jong-Sun Kim Sei-Jong Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report BMC Cancer |
| title | Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report |
| title_full | Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report |
| title_fullStr | Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report |
| title_full_unstemmed | Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report |
| title_short | Zollinger-Ellison syndrome associated with neurofibromatosis type 1: a case report |
| title_sort | zollinger ellison syndrome associated with neurofibromatosis type 1 a case report |
| url | http://www.biomedcentral.com/1471-2407/5/85 |
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