Islet Cell Tumors of the Pancreas

PURPOSE: Islet cell tumors are a rare disease that can be cured by surgical management if they are early diagnosed. However, diagnosis and localization are difficult due to their small size and varied clinical manifestations. We analyzed the clinicopathologic features, the diagnosis and the surgical management of islet cell tumors. METHODS: We retrospectively analyzed the case histories of 30 patients had undergone pancreatic surgery for islet cell tumors between April 1990 and December 1999. RESULTS: The islet-cell tumors included 16 insulinomas, 4 gastrinomas, 1 glucagonoma, one insulin-gastrin secreting tumor, and 8 nonfunctioning tumors. The major clinical manifestations were neuroglycopenic (94%) and adrenergic (75%) symptoms in cases of an insulinoma, abdominal ulcer symptoms (100%) in the cases of a gastrinoma, diabetis mellitus (100%) in the cases of a glucagonoma, and abdominal pain (63%) and a mass (25%) in nonfunctioning tumor. The preoperative tumor localization tools were angiography, transhepatic portal vein sampling, endoscopic ultrasonography, computed tomography, and octreotide scans which had sensitivities of 56%, 71%, 55.5%, 43.3%, and, 25% respectively. The surgical treatments were enucleation (38%) or segmental resection (25%) for insulinomas, pancreaticoduodenectomy with total gastrectomy (25%) or total pancreatectomy (25%) for gastrinomas, and pylorus preserving pancre aticoduodenectomy (38%) or regional pancreatectomy (26%) for nonfunctioning tumors. Malignant islet cell tumors were presenting cases (30%). Two patients died with postoperative complications on post operative day 3 and 35; the others survived during the follow-up period (1 month-10 years). Islet cell tumors with multiple endocrine neoplasm type I occurred in five (17%) cases; in three cases, the tumors were malignant. CONCLUSION: The early diagnosis and vigorous attempt to resect the lesion in islet cell tumors of the pancreas should be carried out for the long-term survival.

Surgically Correctable Hyperinsulinemic Hypoglycemia in Adults Insulinoma vs. Nesidioblastosis

BACKGROUNDS: Hyperinsulinemic hypoglycemia is caused by insulinoma mostly and by nesidioblastosis. While an insulinoma is the most common functional endocrine tumor of pancreas, nesidioblastosis primarily is a childhood disease and is rarely reported in adults. Nesidioblastosis has been defined as a diffuse islet cell hyperplasia accompanied by a differentiation of the islets arising from the pancreatic ductal epithelium. Preoperative localization and proper surgical treatment are crucial because these disases can induce critical and permanent neurologic sequela from the hypoglycemia. However, nesidioblastosis has to be considered differently from the insulinoma in terms of diagnosis and therapeutic aspects in adults. METHODS: We retrospectively analyzed 13 and 3 patients who had been diagnosed as having an insulinoma and nesidioblastosis and who had undergone operations during the 8-year period from 1990 to 1998 at Asan Medical Center. We compared the 2 diseases with respect to diagnosis and therapy. RESULTS: There were 3 men and 10 women with an insulinoma and their mean age was 45 (17~64 years). There were 2 men and 1 woman with nesidioblastosis and their mean age was 45 (22~58 years). The most common clinical manifestation was loss of consciousness, and all the patients had findings compatible with Whipple's triad. The median duration of symptoms before diagnosis was 28 months (6~120 months) in insulinoma and 1.1 months (7 days~2 months) in nesidioblastosis (p=0.009). Hyperinsulinemic hypoglycemia was confirmed during prolonged fasting and the concomitant insulin level was 3~130 U/ml (median=25) in the insulinoma patients and 37~202 U/ml (median=67) in the nesidioblastosis patients (p=0.03). Insulinoma can be localized in 12 patients (93%) preoperatively. The combination of negative angiography and a lack of difference in the insulin concentration gradientin THPVC (transhepatic portal vein catheterization) suggested preoperatively a nesidioblastosis in only one patient (33.3%). All the patients with nesidioblastosis was confirmed intraoperatively by a frozen biopsy. In terms of treating the insulinoma, an enucleation was performed in 5, and pancreatic resection in 8. In nesidioblastosis, subtotal pancreatectomy was done on 2 and pybrus preserving pancreaticoduodenectomy (70%) on one patient. Following the operation, the symtoms of hypoglycemia and the laboratory values were normal in all the patients. CONCLUSION: We observed 13 cases of insulinoma (81%) and 3 of nesidioblastosis (19%). Preoperative suspicion, proper utilization of diagnostic tools, and prudent intraoperative diagnostic procedures enhanced the diagnostic accuracy for hyperinsulinemic hypoglycemia and led to better treatment strategies.

The Significance of Urine Amylase in the Early Diagnosis of Allograft Rejection after Pancreas Transplantation / 대한이식학회지

Pancreas transplantation has became an accepted form of therapy for insulin dependent DM (IDDM). However, rejection remains the major cause of graft loss in pancreas allografts. To overcome the immunologic graft loss following pancreas allograft, early reliable method for rejection is crucial. The purpose of this study was to evaluate the significance of urine amylase (UA) levels as a reliable and sensitive indicator of pancreas allograft rejection retrospectively. Over a 15-month study period from August '97 to Cotover '98, 9 pancreas transplants with bladder drainage were performed at our center. Among which 6 pancreas transplantation alone (PTA) and 3 simultaneous pancreas-kidney transplantation (SPK) were performed. The diagnosis of rejection was based on clinical criteria (fever, tenderness, leukocytosis) and serology such as, a reduction in UA level. Rejection was developed in 5 patients (56%), including 4 PTA and 1 SPK recipients. Mean UA level during normal allograft function was 89,365 U/L, whereas level heralding rejection was 14,760 U/L (P<0.05). After steroid pulse therapy, first rejection episode result in 100% reversal of rejection and the UA level returned toward normal (mean 95,437 U/L). However more than one rejection episode resulted in poor outcome (all the graft were lost). Overall, reversal of rejection occurred in 63% of cases, with 2 PTA and 1 SPK lost due to rejection. Monitoring pancreas-allograft function by UA allows for the timely diagnosis and successful treatment of pancreas-allograft rejection. For more than one rejection episodes, more potent immunosuppressants are through needed to be improve the graft survival.