Cases reported "Adenoma, Islet Cell"

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11/74. Idiopathic hypoglycaemia in sibs with morphological evidence of nesidioblastosis of the pancreas.

    Two sibs who sustained severe hypoglycaemia in the neonatal period are reported. In spite of treatment with frequent feeds intravenous glucose, glucagon, hydrocortisone, and diazoxide, hypoglycaemia persisted, and both infants eventually required subtotal pancreatectomy. Tests for leucine toleranct were normal though the second case showed some protein sensitivity. Histological and immunohistochemical studies indicated nesidioblastosis in both specimens of pancreata. The children are presently performing at mildly retarded levels, and required diazoxide and anticonvulsant medication for some time postoperatively. Because both sexes are represetned, an autosomal recessive inheritance pattern is suggested. The theory of a gut hormone stimulating insulin production is briefly discussed.
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12/74. The significance of gastrinomas found in peripancreatic lymph nodes.

    A patient with zollinger-ellison syndrome (ZES) was found to have a solitary, extrapancreatic, extraintestinal gastrinoma in a peripancreatic lymph node. Preoperative studies did not show the location of the tumor. After excision of the gastrinoma the patient's fasting serum gastrin level dropped from preoperative levels of 596 pg/ml to 120 pg/ml (normal, less than 200 pg/ml). fasting and stimulated gastrin levels have remained within the normal range at 18 months follow-up. This patient had previously undergone antrectomy, so it could not be determined if this tumor represented a primary lymph node gastrinoma or metastasis from an unrecognized tumor that may have been present in the resected stomach or duodenum. The outcome of this case confirms the previous reports that ZES can be controlled after resection of gastrinomas that were contained within abdominal lymph nodes, even if a primary enteropancreatic tumor is not found. Our results also support the use of an aggressive operative search aimed at eradication of the tumor in patients with ZES even if preoperative localization studies fail to identify the site of the gastrinoma.
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13/74. Clinical correlations of serum proinsulin-like material in islet cell tumours.

    To examine the possibility that the concentration of circulating proinsulin-like material (PLM) might be helpful in evaluating the therapeutic response of patients with islet cell tumours, serum levels of PLM in three patients with islet cell tumours were correlated with hypoglycaemic symptoms and plasma glucose concentrations before and after treatment. In two patients ranges of fasting PLM concentration were 0.21-0.29 and 0.91-0.93 ng/ml, respectively, before treatment. After surgical excision of their islet cell adenomas, PLM concentrations decreased to 0.06-0.09 and 0.03-0.05 ng/ml. Insulin concentrations were low preoperatively in both patients and were unchanged postoperatively. The resulting relief from hypoglycaemia was paralleled by a reduction of PLM, with no significant change in insulin. In a third patient, treatment with streptozotocin resulted in marked symptomatic improvement, a 65% reduction in PLM concentration, but no significant change in insulin levels. Relapse was associated with increasing frequency of hypoglycaemic symptoms and increasing PLM concentrations. These findings suggest that changes in the levels of serum PLM may prove to be a sensitive indicator of the response of islet cell tumours to therapy.
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ranking = 2
keywords = operative
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14/74. Nonautonomous function of a pancreatic insulinoma.

    A 56-year-old woman with symptoms of weakness, visual blurring, and sweating underwent diagnostic studies to evaluate the etiology of her hypoglycemia. fasting hypoglycemia was never documented; in diagnostic studies performed during her two hospitalizations and several outpatient glucose tolerance tests, the lowest fasting plasma glucose recorded was 56 mg/dl. The patient displayed exaggerated plasma insulin responses following oral glucose (peak response: 447 muU/ml at 30 min) and following 1 gm of iv tolbutamide (peak response: 719 muU/ml at 5 min) with symptomatic profound hypoglyceria during both tests. Basal per cent proinsulin was elevated at 49% (normal range 5-22%). Throughout a 72 h fast, values for plasma glucose, insulin, and glucose/insulin ratios were all within the normal range. During the infusion of exogenous insulin (0.1 U/kg for 60 min) serum c-peptide reactivity suppressed to less than 1.3 ng/ml when the plasma glucose fell below 40 mg/dl representing normal suppression. At surgery, a pancreatic beta cell adenoma was found and removed. This patient represents the uncommon circumstances in which stimulation tests with tolbutamide and glucose were more helpful in establishing a preoperative diagnosis than were the suppression tests.
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15/74. Diagnostic and operative problems in multiple pheochromocytomas.

    Two children, aged 14 yr, with multiple pheochromocytomas are presented. Both patients had a positive family history. In the preoperative aortographies the intra-adrenal pheochromocytomas of both patients were well visualized, but not the extra-adrenal tumors of the first case. chlorpromazine as an adrenergic blocking agent was successfully used in the preoperative treatment. Postoperative catecholamine excretion in the first case was repeatedly slightly increased indicating residual pheochromocytoma. In addition to the bilateral adrenal pheochromocytomas, multiple islet cell adrenomas and cholecystolithiasis were revealed at the operation of the second case. After bilateral adrenalectomy and total pancreaticoduodenectomy, regular follow-up examinations were carried out for 28 months. hyperparathyroidism and signs of possible medullary thryoid carcinoma were discovered. Thus the patient had an unique pattern of MEA syndrome.
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ranking = 7
keywords = operative
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16/74. Anaesthetic management of insulinoma.

    The anaesthetic management of two cases of insulinoma treated by distal pancreatectomy is presented. Stress is laid on pre-operative and intra-operative glucose administration in sufficiently high quantity. Pre-operative corticosteroid therapy is better avoided. Dextrose infusion should be stopped soon after removal of the tumour to prevent excessive hyperglycemic response in the postoperative period. Methyoxyflurane was used because it tends to raise the blood sugar level. pancuronium bromide was preferred because of the stable cardiovascular conditions it produces.
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ranking = 4
keywords = operative
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17/74. Subselective angiography in localizing insulinomas of the pancreas.

    Preoperative angiography was performed on 10 patients who had insulin-producing islet cell adenomas. Eight of the 10 islet cell tumors were localized. Seven of the eight adenomas were demonstrated only on subselective injections into the small intrapancreatic arteries, and one was demonstrated by a selective injection into a large artery. Failure to localize two islet cell adenomas was probably caused by the inability to subselect the small intrapancreatic arteries supplying the tumor. One tumor was seen only with subtraction techniques, which were used in all cases. Localization was not related to vascularity, size of tumor, or location within the pancreas. Vasoconstrictive pharmacoangiography was not helpful; magnification was helpful but not essential. The most important factor in localizing islet cell tumors is demonstrating the complete pancreatic blood supply with subselective injections into the small intrapancreatic arteries supplying the tumor (inferior and superior pancreaticoduodenal and dorsal arteries).
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ranking = 1
keywords = operative
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18/74. Cure of acromegaly by operative removal of an islet cell tumor of the pancreas.

    We studied a 30 year old woman in whom acromegaly was cured by operative removal of a large cystic beta cell adenoma of the pancreas. We detected substantial amounts of immunoreactive human growth hormone (hGH)-like activity in a tumor tissue extract. Extracts of the tumor and a normal human pituitary gland eluted from a Sephadex G-75 column in two identical peaks. Serial dilutions of the tumor extract displaced radioactive 125I hGH parallel to a standard curve. Surprisingly, an extract of a normal human pancreas contained large amounts of hGH-like activity and gave results similar to those of the tumor extract on gel chromatography and on serial dilution displacement in the growth hormone immunoassay. paper electrophoretic studies of 125I hGH after incubation with normal pancreatic and tumor extracts with and without enzyme inhibitors suggested that pancreatic proteolytic enzymes damaged the 125I hGH used in growth hormone radioimmunoassay and produced a false detection of hGH.
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ranking = 5
keywords = operative
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19/74. insulinoma with hypoglycemia and normal immunoreactive insulin but with an insulin-like activity restricted to the portal vein.

    In a 46-year old Caucasian woman, the authors report a B-cell adenoma with plasma immunoreactive insulin (IRI) ranging from 10 to 32 microunits/ml, despite severe spontaneous hypoglycemia. In a peroperative sample withdrawn from the portal vein, normal IRI (40 micromicron/ml) in the presence of high insulin-like activity (290 microunits/ml) was observed by using a biological assay performed on rat epididymal fat tissue. Furthermore, this material did not cross-react with insulin antibodies and was undetectable in systemic venous samples. Although further identification by chromatographic extraction was not performed, the substance secreted by the tumor is probably identical to the non-suppressible insulin-like activity (NSILA) isolated by Froesch and responsible for hypoglycemia in a few cases of extrapancreatic tumors. The absence of this material in systemic samples indicates an immediate removal by a single passage through the liver.
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ranking = 1
keywords = operative
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20/74. insulinoma in a patient with annular pancreas.

    Intraoperative ultrasonography has facilitated the localization of insulinomas. In this report, we describe a patient who was found to have an insulinoma in association with an annular pancreas. Because such tumors can be in close approximation to major pancreatic ductal structures, intraoperative ultrasonography can be useful for not only localizing the lesion but also delineating the related anatomic features. Although the optimal management of an asymptomatic annular pancreas has not been determined, our patient had no evidence of chronic pancreatitis and no narrowing of the duodenal lumen; therefore, no bowel bypass procedure was done.
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ranking = 2
keywords = operative
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