Pancreaticoduodenectomy in patients with type 1 Neurofibromatosis: Report of two cases and literature review

• Type 1 Neurofibromatosis is an autosomal disorder associated with gastrointestinal tumors in almost one quarter of the cases.
• The presence of synchronous malignant gastrintestinal tumor in patients with type 1 Neurofibromatosis is rarely reported in the literature.
• Patients with type 1 Neurofibromatosis with gastrointestinal complaints should be thoroughly evaluated to rule out malignancy.
• Optimal management of patients with synchronous neoplasms is single-stage surgical resection.

IntroductionType 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 casesThe 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.DiscussionGI 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.ConclusionAlthough 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.

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