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Case Report
The coexistence of neuroendocrine papillary tumor and gastrointestinal stromal tumor in neurofibromatosis Type 1: A case report
1 Department of Gastroenterology, Sahloul Hospital, Tunisia
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H. Jaziri
Department of Gastroenterology, Sahloul Hospital,
Tunisia
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Article ID: 100056Z10HJ2018
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Jaziri H, Ameur WB, Ksiaa M, Hammami A, Elleuch N, Brahem A, Ajmi S, Slama AB, Jmaa A. The coexistence of neuroendocrine papillary tumor and gastrointestinal stromal tumor in neurofibromatosis Type 1: A case report. J Case Rep Images Oncology 2018;4:100056Z10HJ2018.ABSTRACT
Introduction: Neurofibromatosis Type 1 (NF-1, Recklinghausen disease) is the most common hereditary multitumor syndrome with an incidence at birth of approximately 1/3000. In type 1 neurofibromatosis, gastrointestinal tumors are relatively infrequent and generally correspond with a hyperplasia of neural cells in the myenteric and submucosal plexus, gastrointestinal stromal tumors (GIST) and periampullary neuro endocrine tumors. However, the association of GIST, the neuro endocrine tumor of ampulla of Vater and neurofibromatosis is rare.
Case Report: The case reports a 66-year-old woman without a familial history of neurofibromatosis diagnosed with neuro endocrine tumor of ampulla and GIST of the duodenum. She underwent cephalic duodeno pancreatectomy. A working diagnosis of NF-1 was made and the patient was referred to genetic counseling.
Conclusion: The case clearly points out the important value of gastrointestinal findings in identifying undiagnosed NF-1.
Keywords: Gastrointestinal stromal tumor, Neurofibromatosis Type 1, Neuroendocrine tumor
SUPPORTING INFORMATION
Author Contributions
H. Jaziri - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
W. Ben Ameur - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
M. Ksiaa - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
A. Hammami - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
N. Elleuch - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
A. Brahem - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
S. Ajmi - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
A. Ben Slama - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
A. Jmaa - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this case report.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2018 H. Jaziri et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
