CLINICALLY SILENT PANCREATIC SOMATOSTATINOMA IN MEN-I SYNDROME, AND LITERATURE-REVIEW

A 59 year old female with a somatostatinoma associated with the MEN-I syndrome is presented. Transabdominal ultrasonography, performed for u nexplained epigastric pain, showed a tumor of the head of the pancreas . A diagnosis of somatostatinoma was made from immunohistochemical exa mination of the resected tumor. Twenty-four days after surgery, the pa tient become drowsy and stuporous due to acute hyperparathyroidism. Mi nimal improvement occurred with medical treatment. Parenteral infusion of somatostatin, added on the basis of the immunohistochemical diagno sis of the resected pancreatic tumor, did not relieve hyperparathyroid ism. The patient then had a left inferior parathyroid adenoma resected with complete recovery. One month later, a cerebral CT scan showed a silent, not functioning, 1 cm pituitary adenoma. Two years later chole cystectomy, because of lithiasis, was performed. The patient is alive and symptom free 7 years after surgery. Literature review shows only o ne other patient with MEN-I among 136 somatostatinomas (55 pancreatic, 2 bronchial and 79 intestinal).