EPCephalic duodenopancreatectomy for neurofibromatosis associated with gastrointestinal stromal tumor. A case report

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COD: 3048_13_06_2019_ep Categorie: , ,

Florin Graur, Vlad I. Nechita, Sorana D. Bolboacă, Mihaela Dragotă,
Ana M. Cosma, Nadim Al Hajjar

Ann Ital Chir, Digital Edition 2019, 8
Epub, June 13

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BACKGROUND: Neurofibromatosis is a genetic autosomal dominant disease characterized by multiple skin nodules and hyperpigmentation. This condition is frequently associated with a large variety of neoplasia, including gastrointestinal stromal tumors (GIST) in about 6% of cases. We present a case of neurofibromatosis associated GIST.
CASE REPORT: A 57 year-old male patient with Von Recklinghausen disease was referred into our service after he was discovered with a 7 mm nodular formation in contact with the pancreatic head, during a routine abdominal ultrasonography.
Ultrasound examination performed into our service reveals a nodular formation with hypoechoic circumference, central translucency, with central necrosis, contrast medium uptake and dimensions at about 78/49/77 mm, without peritoneal fluid content. Trans-gastric biopsy specimen shows mesenchymal proliferation with spindle cells and elongated nuclei and c-kit intensely positive. The diagnosis was gastrointestinal stromal tumor. Delimitation towards pancreatic head was unclear. A cephalic duodenopacreatectomy was performed. Morphopathology confirmed a multifocal GIST of low grade G1, in the second stage.
CONCLUSION: A rare case of neurofibromatosis associated GIST with multifocal localization was successfully treated by cephalic duodenopancreatectomy.

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