MEN type 1 is characterized primarily by the presence of functioning and nonfunctioning tumors or hyperplasia of the pituitary gland, parathyroid glands, and pancreatic islet cells. Pancreatic islet tumors in MEN type 1 produce different kinds of hormone which were pancreatic polypeptide, gastrin, glucagon, insulin and so on. To date, ten cases had been reported in Korea. We report another case with MEN type 1 having prolatin-secreating pituitary adenoma, parathyroid hyperplasia and insulinoma. A 36-year-old woman was admitted because of long-standing amenorrhea and recently diagnosed osteoporosis. Otherwise, she had been in good health except experiencing one episode of loss of consciousness after skipped meal. The blood chemistries were normal except hypercalcemia and hypophosphatemia. Hormonal studies revealed elevated levels of intact PTH and prolactin and decreased value of estradiol with low LH and FSH. The neck CT revealed 1 cm-sized nodule at posterior portion of right thyroid gland and 99mTc-sestamibi sintigraphy showed a increased uptake in left lower and right lower parathyroid glands. The sella MRI showed 0.7 cm-sized enhanced lesion in the left pituitary gland. The ratio of immunoreactive insulin to glucose was elevated and 3-4 pancreatic masses of variable size were identified by endoscopic ultrasonography and angiography. Subtotal parathyroidectomy and pyrolus-preserving pancreaticoduodenectomy with spleen-preserving distal pancreatectomy was done. Postoperative she had been doing well with normocalcemia and normoglycemia. Transsphenoidal adenonectomy was done 5 months later. Histologic examination of removed tissues revealed a single insulinoma, prathyroid hyperplasia and prolactin-secreating pituitary adenoma.
Adult ; Amenorrhea* ; Angiography ; Endosonography ; Estradiol ; Female ; Gastrins ; Glucagon ; Glucose ; Humans ; Hypercalcemia ; Hyperplasia ; Hypophosphatemia ; Insulin ; Insulinoma ; Islets of Langerhans ; Korea ; Magnetic Resonance Imaging ; Male ; Meals ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Neck ; Osteoporosis* ; Pancreatectomy ; Pancreatic Polypeptide ; Pancreaticoduodenectomy ; Parathyroid Glands ; Parathyroidectomy ; Pituitary Gland ; Pituitary Neoplasms ; Prolactin ; Technetium Tc 99m Sestamibi ; Thyroid Gland ; Unconsciousness

We report a case of somatostatinoma, which manifested as insulinoma after liver metastasis. A 74-year-old man suffered from diabetes mellitus and jaundice. The abdominal CT scan of this patient showed a mass in the pancreas head, which obstructed biliary duct. He underwent Whipples procedure. Immunohistochemical staining of postoperative specimen disclosed that this tumor was strongly positive for somatostatin. After 4 months, follow up CT scan showed multiple metastatic lesions in the liver. We performed transarterial chemoinfusion two times, but the response was disappointing. After 2 months, he suffered from altered mentality, which was relieved by intake of sugar. Biochemical laboratory findings and immunohistochemical staining of liver biopsy disclosed that the metastatic lesion in this patient was insulinoma. We performed embolization of hepatic artery with gelform. The biochemical response was dramatic, but he died of septic shock, which was caused by gas forming liver abscess. In summary, we report a case of somatostatinoma which manifested as insulinoma after metastasis to the liver.
Aged ; Biopsy ; Diabetes Mellitus ; Follow-Up Studies ; Head ; Hepatic Artery ; Humans ; Insulinoma* ; Jaundice ; Liver Abscess ; Liver* ; Neoplasm Metastasis ; Pancreas ; Shock, Septic ; Somatostatin ; Somatostatinoma* ; Tomography, X-Ray Computed