Endocrine Abstracts

Background: Multiple endocrine neoplasia type 1 (MEN1) is an inherited syndrome, characterized by primary MEN1 tumors: parathyroid, pancreatic and pituitary. We describe the case of a patient with MEN1 syndrome, initially presented with a gastric neuroendocrine tumor (NET).

Case presentation: A 33-year-old male patient, initially treated for perforated gastric ulcer, presented 7 years later with dyspepsia, diarrhea and weight loss. Abdominal CT scan revealed marked thickening of the whole gastric wall and several enlarged lymph nodes. The patient underwent total gastrectomy without lymph nodes resection. Histopathological examination and immunochemistry showed well differentiated G2 NET, with Ki-67 of 3.8%, positive for chromogranin A, synaptophysin and somatostatin receptor type 2. The serum gastrin and chromogranin A levels were elevated, thereby treatment with lanreotide was initiated. Furthermore, the patient presented elevated serum calcium and PTH levels. Sestamibi scintigraphy identified a right parathyroid adenoma and subtotal parathyroidectomy was performed. Following a pituitary CT, a double non-functioning pituitary adenoma was found. The genetic test confirmed the MEN1 diagnosis. The ⁶⁸Gallium-DOTATATE PET/CT scan showed high uptake in several abdominal lymph nodes, and also in the pancreatic head. The patient was treated with 4 cures of peptide radio receptor therapy (PRRT) with lymph node shrinkage. Further assessment of the possibility of pancreatic tumor resection is considered.

Conclusions: Up to 30–50% of patients with MEN1 will develop gastric NETs. We presented a case of a patient with confirmed MEN1 syndrome with hyperparathyroidism, pituitary adenoma and a pancreatic tumor, who also associated a gastric NET with metastatic lymphadenopathies.