Surgical Management of Large Gastrinomas in a Young Patient With MEN1

Abstract

Multiple Endocrine Neoplasia 1 (MEN1) is a well-described hereditary disorder that requires a multidisciplinary approach. Gastrinomas are the most common enteropancreatic tumors found in MEN1. They often appear as small (< 0.5 cm) multifocal lesions and are rarely found as large masses in the pancreas. The crossroads of deciding between medical versus surgical management when treating these tumors requires an evidenced-based- and patient-centered approach. We describe a rare case of a young patient with MEN1 and large pancreatic gastrinomas. A 23-year-old female patient with MEN1 (prolactinoma, primary hyperparathyroidism) was evaluated for the development of hypocalcemia after surgical excision of 3 parathyroid glands. A prior history of a perforated peptic ulcer prompted further evaluation that revealed gastrin levels of 481 pg/ml (13 - 115 pg/ml) off any acid suppression therapy, and an abdominopelvic MRI that revealed two T1 hypointense lesions measuring 2.4 cm and 1.4 cm at pancreatic head and tail, respectively. Both lesions resulted to be grade 2/3 neuroendocrine tumors consistent with gastrinoma. Abdominopelvic CT scan for staging showed 6 isodense lesions distributed within the pancreas measuring up to 3.2 cm without lymphadenopathy nor metastatic liver lesions. After extensive discussion regarding management, the patient opted for a surgical approach. A total pancreaticoduodenectomy was performed. The pathology revealed 7 grade 2 well-differentiated neuroendocrine tumors (6 in pancreas and 1 in duodenum) and 18 of 22 positive lymph nodes. Post-surgically she experienced multiple admissions due to surgical complications and the expected endocrine and exocrine dysfunction. Eight weeks after surgery gastrin remained elevated [652 pg/ml (13 - 115 pg/ml)]. The surgical management of gastrinomas in MEN1 remains without consensus. Due to the multifocality of gastrinomas associated with MEN1 and the high probability of persistent hypergastrinemia, surgery is not usually recommended. However, most guidelines are in favor of surgical excision for tumors > 2 cm to decrease their associated risk for liver metastasis and improve long-term survival. In this young patient with a 3.2 cm tumor, a surgical approach was favored to improve prognosis. Persistent hypersecretion may occur, as in our patient, due to residual hypertrophy of gastric parietal cells, although the possibility of remaining tumors still exists, especially in view of high lymph node positivity. The best available evidence-based treatment alternatives were offered to our patient; unfortunately, both the disease itself and surgical treatment options impose high morbidity and decreased quality of life. More studies reporting on the long-term outcomes after surgical resection of gastrinomas in MEN1 are needed to identify predictors to help recognize patients which benefit from a surgical approach.