WILLIAM P. LONGMIRE JR., M.D.; JOSIAH BROWN, M.D.; GERALD D. BUCKBERG, M.D.; ALLAN COOKE, M.D.; GARY GLOBER, M.D.; WILLIAM N. HANAFEE, M.D.; GARY KANTOR, M.D.; KENNETH K. MATSUMOTO, M.D., F.A.C.P.; WILLIAM G. PLESTED, M.D.; DONALD B. ROCHLIN, M.D.; JAMES A. WILKERSON, M.D.
The features of the three types of functioning islet cell tumors of the pancreas are illustrated in the cases presented.
Hypoglycemia caused by the classical insulin-producing islet cell adenoma must be distinguished from reactive or functional hypoglycemia before surgery is recommended. The hypoglycemia of the insuloma occurs after fasting and produces elevated blood insulin in two thirds of the patients. Surgical practice has favored the "blind" distal pancreatectomy when islet cell adenoma seems certain but cannot be located at laparotomy. Because of the increased complications resulting from a secondary total pancreatectomy and because of the frequency with which the tumor has been found in the head of the gland on pathologic examination, it is possible that resection of the head and uncinate process should be the initial procedure in these cases. More accurate localization of these tumors by angiography may make such considerations unnecessary.
The typical diagnostic features of the Zollinger-Ellison tumor are gastric hypersecretion and fulminating ulcer diathesis refractory to conventional therapy. In addition, the Zollinger-Ellison tumor (and its metastasis) yields an extract with the physiologic properties of the hormone gastrin, a stimulator of gastric secretion 1,000 times more powerful than histamine. Best results in surgical therapy of the Zollinger-Ellison tumor have followed total gastrectomy combined with resection of the tumor (85% satisfactory results). A test is under evaluation by Zollinger and his associates to detect gastrin remaining in the circulation after tumor resection. Proof of complete eradication of the tumor could obviate gastrectomy.
The rarest type of functioning islet cell tumor is recognized by a debilitating severe diarrhea and hypokalemia unaccompanied by gastric hypersecretion or peptic ulceration. Although secretion of a hormone by the tumor is probable, none has been extracted. A few patients may manifest both this syndrome and the Zollinger-Ellison syndrome, just as some have manifested a Zollinger-Ellison syndrome accompanied by the hypoglycemia of the classical insuloma.
Chemotherapeutic treatment of functioning and nonfunctioning islet cell tumors has been under way for many years. Recently some responses have been achieved in cases of malignant tumors with 5-fluorouracil and a new drug, Tubercidin®. Successful chemotherapeutic treatment of islet cell tumors will depend upon finding a way to control the drug toxicity or to allow the drug to be excreted by the body so that severe bone marrow depression need not dictate cessation of treatment.
WILLIAM P. LONGMIRE, JOSIAH BROWN, GERALD D. BUCKBERG, ALLAN COOKE, GARY GLOBER, WILLIAM N. HANAFEE, et al. Islet Cell Tumors of the Pancreas. Ann Intern Med. 1968;68:203–221. doi: 10.7326/0003-4819-68-1-203
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Published: Ann Intern Med. 1968;68(1):203-221.
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