ReviewJournal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract2010
Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases.
Review in Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 1 of them a synthesis that pooled it.
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Who cites it
43 citing papers in PubMed, 1 synthesis or guideline pooled it, 118 citations in OpenAlex.
- Gastrointestinal stromal tumors associated with neurofibromatosis 1: a single centre experience and systematic review of the literature including 252 cases.International journal of surgical oncology · 2013Pooled it
- Composite gangliocytoma/neuroma and neuroendocrine tumor: a clinicopathologic, immunohistochemical, and molecular genetic study of 11 cases.Virchows Archiv : an international journal of pathology · 2026Article
- Multifocal Small Bowel Gastrointestinal Stromal Tumors and Concurrent Neuroendocrine Tumor in Neurofibromatosis Type 1.ACG case reports journal · 2026Article
- Ampullary Mixed Adenoma and Well-Differentiated Neuroendocrine Tumor (MANET) with Aberrant Beta-Catenin Expression in a Patient with Neurofibromatosis Type 1.Endocrine pathology · 2026Article
- Periampullary duodenal neuroendocrine tumor in a patient with neurofibromatosis-1: A case report.World journal of clinical oncology · 2024Article
- Duodenal neuroendocrine tumor after bilateral breast cancer with type 1 neurofibromatosis: a case report.Surgical case reports · 2024Article
- Neurofibromatosis Type 1: Optimizing Management with a Multidisciplinary Approach.Journal of multidisciplinary healthcare · 2024Review
- Duodenal ampulla neuroendocrine tumor and gastrointestinal stromal tumors in a case of neurofibromatosis type 1: a case report.Frontiers in oncology · 2024Article
- Overview of the 2022 WHO Classification of Familial Endocrine Tumor Syndromes.Endocrine pathology · 2022Review
- What Are the Place and Modalities of Surgical Management for Pancreatic Neuroendocrine Neoplasms? A Narrative Review.Cancers · 2021Review
- A Case of Duodenal Neuroendocrine Tumor Accompanied by Gastrointestinal Stromal Tumors in Type 1 Neurofibromatosis Complicated by Life-Threatening Vascular Lesions.The American journal of case reports · 2021Article
- Phakomatoses and Endocrine Gland Tumors: Noteworthy and (Not so) Rare Associations.Frontiers in endocrinology · 2021Review
- Somatostatinoma and Neurofibromatosis Type 1-A Case Report and Review of the Literature.Diagnostics (Basel, Switzerland) · 2020Article
- Abdominal neoplastic manifestations of neurofibromatosis type 1.Neuro-oncology advances · 2020Review
- Advanced pharmacological therapies for neurofibromatosis type 1-related tumors.Acta bio-medica : Atenei Parmensis · 2020Article
- Inherited syndromes involving pancreatic neuroendocrine tumors.Journal of gastrointestinal oncology · 2020Review
- Advances in the Diagnosis and Management of Well-Differentiated Neuroendocrine Neoplasms.Endocrine reviews · 2020Review
- Clinical and Molecular Risk Factors for Recurrence Following Radical Surgery of Well-Differentiated Pancreatic Neuroendocrine Tumors.Frontiers in medicine · 2020Review
- Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1.Frontiers in genetics · 2020Article
- Synchronous Gastrointestinal Stromal Tumor and Ampullary Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Report of Three Cases.The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi · 2019Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients with neurofibromatosis type 1 (NF1) are at increased risk to develop tumors throughout the gastrointestinal tract, including neuromas, gastrointestinal stromal tumors (GIST), and periampullary somatostatin-rich carcinoids. Here, we briefly describe two male patients with NF1 and review the recent literature on this topic.
methodsDatabases for PubMed and MEDLINE were searched for English-language articles since 1989 using a list of keywords, as well as references from review articles.
resultsThe results generated by the search yielded 50 articles and 74 cases. Patients most commonly presented with jaundice, weight loss, GI bleeding, or anemia. The mean age at presentation was 47.9 years, with 59% of patients being female. Mean tumor size was 3.8 cm (range 0.9-27 cm). Tumor location was the duodenum (60%), ampulla (31%), pancreas (5%), or bile duct/gallbladder (4%). Tumor type was reported as somatostatinoma (40%), GIST (34%), adenocarcinoma (8%), carcinoid (6%), neurofibroma (5%), schwannoma (4%), or gangliocytic paraganglioma (3%). Treatment included classic Whipple procedure (42%), local excision (25%), pylorus-preserving pancreaticoduodenectomy (17%), and other resection (6%). Mean follow-up was 31 months postresection (range 0-99 months): 75% of patients were alive with no evidence of disease.
conclusionsThese results underscore the importance of a thorough evaluation for tumors in NF1 patients with gastrointestinal symptoms, as well as subsequent surgical management when findings suggest a tumor in the periampullary region, as resection remains the mainstay of treatment.
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Registered trials
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