ArticleDiagnostic pathology2019
Triple malignancy (NET, GIST and pheochromocytoma) as a first manifestation of neurofibromatosis type-1 in an adult patient.
Article in Diagnostic pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 14 citations in OpenAlex.
- [Neurofibromatosis 1-related tumors in pediatric patients].Problemy endokrinologii · 2025Article
- Neurofibromatosis Type 1: Optimizing Management with a Multidisciplinary Approach.Journal of multidisciplinary healthcare · 2024Review
- Periampullary tumors in a patient with pancreatic divisum and neurofibromatosis type 1: a case report.Hereditary cancer in clinical practice · 2023Article
- Article
- Neuroendocrine Tumor with Metachronous Gastrointestinal Stromal Tumor in a Patient: A Case Report.The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi · 2022Article
- Laparoscopic retroperitoneal resection of the duodenal gastrointestinal stromal tumors in neurofibromatosis type 1; Case Report and literature review.Frontiers in surgery · 2022Article
- Pancreatic Gastrinoma, Gastrointestinal Stromal Tumor (GIST), Pheochromocytoma, and Hürthle Cell Neoplasm in a Patient with Neurofibromatosis Type 1: A Case Report and Literature Review.The American journal of case reports · 2021Review
- Identification and Validation of Circulating Micrornas as Prognostic Biomarkers in Pancreatic Ductal Adenocarcinoma Patients Undergoing Surgical Resection.Journal of clinical medicine · 2020Article
- Pheochromocytoma and gastrointestinal stromal tumours in an adult neurofibromatosis type 1 patient: a rare co-occurrence.BMJ case reports · 2020Review
- Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1.Frontiers in genetics · 2020Article
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
Abstract
backgroundNeurofibromatosis type-1 (NF1), also called von Recklinghausen disease, is a rare genetic disease which can lead to the development of benign or even malignant tumors. NF1 is mostly diagnosed in children or early adolescents who present with clinical symptoms. A curative therapy is still missing and the management of NF1 is based on careful surveillance. Concerning tumors which affect the gastrointestinal tract in patients with NF1, the most common is a gastrointestinal stromal tumor (GIST). CASE PRESENTATION: We present a case of a 58-year-old adult patient with dyspeptic symptoms who was incidentally diagnosed with triple malignancy (pheochromocytoma, multiple GISTs of small intestine and an ampullary NET) as a first manifestation of NF1. The patient underwent surgical treatment (adrenalectomy and pancreaticoduodenectomy) with no complications and after 2 years remains in oncological remission.
conclusionNF1 is a rare genetic disease which can cause various benign or malignant tumors. The coincidence of GIST and NET is almost pathognomonic for NF1 and should raise a suspicion of this rare disorder in clinical practice.
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