title: Pancreaticoduodenectomy in patients with type 1 Neurofibromatosis: Report of two cases and literature review.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: fredteixeirajr@gmail.com.
authors.forename: Frederico
authors.initials: F
authors.lastname: Teixeira
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: carlosmenegozzo@gmail.com.
authors.forename: Carlos Augusto Metidieri
authors.initials: CAM
authors.lastname: Menegozzo
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: sergiodcnetto@gmail.com.
authors.forename: Sérgio Dias do
authors.initials: SDD
authors.lastname: Couto Netto
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: gustavo.scapini@hc.fm.usp.br.
authors.forename: Gustavo
authors.initials: G
authors.lastname: Scapini
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: eduardoakaishi@gmail.com.
authors.forename: Eduardo Hiroshi
authors.initials: EH
authors.lastname: Akaishi
authors.affiliation: Departament of Pathology, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: marcelavasconcelos@gmail.com.
authors.forename: Marcela Pereira Silva
authors.initials: MPS
authors.lastname: Vasconcelos
authors.affiliation: Departament of Surgery, Division of Surgical Clinic III, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Enéas de Carvalho Aguiar, 255 - Cerqueira César, Sao Paulo, 05403-000, Brazil. Electronic address: edivaldo.utiyama@hc.fm.usp.br.
authors.forename: Edivaldo Massazo
authors.initials: EM
authors.lastname: Utiyama
keywords: Case report; Gastrointestinal tumors; Neurofibromatosis