Please use this identifier to cite or link to this item: https://observatorio.fm.usp.br/handle/OPI/16978
Title: Pancreaticoduodenectomy in patients with type 1 Neurofibromatosis: Report of two cases and literature review
Authors: TEIXEIRA, FredericMENEGOZZO, Carlos Augusto MetidieriCOUTO NETTO, Sergio Dias doSCAPINI, GustavoAKAISHI, Eduardo HiroshiVASCONCELOS, Marcela Pereira SilvaUTIYAMA, Edivaldo Massazo
Citation: INTERNATIONAL JOURNAL OF SURGERY CASE REPORTS, v.27, p.36-40, 2016
Abstract: INTRODUCTION: Type 1 Neurofibromatosis (NF1) is one of the most common autosomal dominantly inherited multisystem disorders. It is associated with an increased risk of developing neurologic and gastrointestinal (GI) malignant neoplasms. The incidence of GI involvement is reported in 10-25% of patients. Less than 5% of NF1 patients with GI neoplasms manifest symptoms. The presence of synchronic gastrointestinal stromal and neuroendocrine tumors is rare in these patients. PRESENTATION OF CASES: The first case is a 37 year-old male patient with a history of abdominal pain for a few months. Imaging study showed a periampullary mass and a solid lesion at the third duodenal portion. He was submitted to a pancreatoduodenectomy and histological anaylisis showed two low-grade neuroendocrine tumors and a gastrointestinal stromal tumor. The second case is a 47 year-old female patient with a routine computed tomography scan showing a duodenal and a jejunal lesion. Duodenopancreatectomy was performed and histological analysis showed a neuroendocrine adenocarcinoma of the duodenum and two jejunal lesions compatible with GI tumors. DISCUSSION: GI symptoms such as jaundice, pain and bleeding in NF1 patients should prompt urgent admission Occasionally, associated gastrointestinal tumors may be incidentally found in asymptomatic NF1 patients. The presence of a periampullary or duodenal neoplasia such as neuroendocrine tumors should be evaluated. CONCLUSION: Although rare, the synchronic presentation of gastrointestinal tumors in patients with NF1 should be ruled out since it can lead to higher morbidity and mortality rates. Single-stage surgical management is feasable and yields satisfactory results. (C) 2016 The Authors.
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Artigos e Materiais de Revistas Científicas - FM/MCG
Departamento de Cirurgia - FM/MCG

Artigos e Materiais de Revistas Científicas - HC/ICESP
Instituto do Câncer do Estado de São Paulo - HC/ICESP

Artigos e Materiais de Revistas Científicas - HC/ICHC
Instituto Central - HC/ICHC

Artigos e Materiais de Revistas Científicas - LIM/62
LIM/62 - Laboratório de Fisiopatologia Cirúrgica

Artigos e Materiais de Revistas Científicas - ODS/03
ODS/03 - Saúde e bem-estar


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