Endocrine Abstracts

Introduction: We present a rare case of 49-year-old patient with neurofibromatosis type I and somatostatin-secreting pancreatic neuroendocrine neoplasm. World literature describes about 30 cases of duodenal somatostatinoma in NFI1 and 1/3 of these cases with metastases to the lymph nodes or liver.

Case report: In October 2013 patient was operated in emergency due to gastrointestinal tract obstruction. During the procedure inoperable head of pancreas tumour, closing duodenum was confirmed. Gastrojejuniostomia was performed, specimens from pancreas tumour were taken. Neoplasm tissue wasn’t found in the examined specimens in the histopathological examination. In June 2014 core biopsy pancreas tumour was performed under control CT which resulted in discovering neuroendocrine neoplasm G2. Afterwards in 30th September 2014 patient was operated (using the method of Whipple’s). Diagnosis well-differentiated neuroendocrine neoplasm G2, Ki-67 5% was confirmed (positive somatostatin reaction/expression in immunohistochemical test). In abdominal CT 17th December 2014 local recurrence and metastases to liver and lymph nodes have been identified. Stomachaches, weight loss and loose stools (4/day) since October 2014 were appeared. Evaluated concentrations of neuroendocrine markers (CgA, insulin, gastrin, serotonin and 5-hydroxyindoleactic acid) were in reference ranges. Determination of serum somatostatin level was impossible. Due to symptomatic and metastatic characters neuroendocrine neoplasm patient was treated with the somatostatin analogue. Clinical improvement (weight stabilization, diarrhea withdrawal) was observed.

Conclusion: Somatostatin-secreting pancreatic neuroendocrine neoplasm occurs very rarely and affects patients with neurofibromatosis type I. Somatostatin analogues in neuroendocrine neoplasm treatment resulted in great effectiveness.