Gastric GIST with synchronous neuroendocrine tumour of the pancreas in a patient without neurofibromatosis type 1.
The gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the gastrointestinal tract. These are rare tumours with an incidence of 15 new cases per million per year. The occurrence of neuroendocrine tumours of the pancreas is rare, representing 1-5% of pancreatic cancers, and it is estimated that its incidence does not exceed five to one million. GISTs are common in patients with neurofibromatosis type 1 (NF1); there are few reported cases of synchronous neuroendocrine tumours in these patients and most are pheochromocytomas. The case reports a 64-year-old woman referred to the General Surgery Outpatient for incidental finding of gastric and pancreatic tumours. She underwent a radical subtotal pancreatectomy + partial gastrectomy with jejunal transposition. The pathological examination revealed: gastric GISTs and a well-differentiated neuroendocrine carcinoma of the pancreas. This is the second case published so far of a patient with both tumours and without NF1. Posterior studies must be performed to evaluate if some other genetic disorder is involved in these patients without NF1.
Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal. ameliassbtavares@gmail.com
Amelia Brandao
AB
Tavares
Fernando Arruda
FA
Viveiros
Cassilda Neves
CN
Cidade
Jorge
J
Maciel
D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D016099:Endoscopy, Gastrointestinal; D019160:Endosonography; D005260:Female; D005743:Gastrectomy; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D033162:Incidental Findings; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000601:surgery; D009456:Neurofibromatosis 1; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery; D049268:Positron-Emission Tomography