Of 565 patients with thrombocytopenia admitted to Duke University Hospital between 1975 and 1985, 100 had splenectomy. Ninety-eight patients had failed chronic immunosuppressive therapy and three patients had acute intracranial bleeding or total absence of platelets in the peripheral blood smear, and had urgent splenectomy. At primary splenectomy, accessory spleens were identified and resected in 18% of patients. There was no operative mortality. Fifty-eight patients had an excellent response to splenectomy and their steroids were tapered off within 3 weeks. Thirteen patients had a poor response to primary splenectomy of whom eight remitted spontaneously and five required accessory splenectomy resulting in complete remission in three patients. Twenty-nine patients were considered nonresponders, 25 of whom had radionuclide scanning for accessory spleens. Seven of these patients had accessory spleens identified but only four consented to accessory splenectomy. In three of the four patients, a complete remission was achieved. Neither platelet antibody titers nor measurements of platelet survival or turnover predicted platelet response to splenectomy. However, immune thrombocytopenic purpura (ITP) in older patients was significantly less likely to respond to splenectomy. These data support continuing use of splenectomy in selected patients with ITP and an aggressive search for accessory spleens in patients who relapse since they are easily localized at operation by hand-held isotope detector probe.

From 1950 through 1974, a total of 108 cases of primary intestinal leiomyosarcoma were seen at the Mayo Clinic. Most of these uncommon tumors occurred in the fifth and sixth decades of life, and more of them in men than in women (2.6:l). There were 73% in the small bowel, 25% in the large bowel, and 2% in the anus. Gastrointestinal bleeding and pain were the two most common signs at presentation, and they led to surgical exploration in all cases where they appeared. By the time surgery was performed, only 48% of the tumors could be resected with hope of cure. Within that group of cases, 50% of the patients survived 5 years, but only 35% survived 10 years, late recurrence being common. The histologic grade of the tumor affected survival strongly. Lack of recognition of the high late recurrence rate probably led to erroneous early optimism in prognosis.

In a detailed review of cystic hepatobiliary neoplasms, we identified a subset of 50 cases in which tumors were characterized by the presence of a mesenchymal cell layer interposed between an inner epithelial lining and an outer connective tissue layer. We have recently seen three such patients, making a total of 53 patients reported in the English literature. All of the patients were female, 44 of whom, with an average age of 41 years, had benign tumors. The average age of the remaining nine patients was 57 years and these patients had malignant tumors. In seven patients, the malignancy arose from the epithelial layer, but in two patients sarcomatous changes were identified in the mesenchymal tissues. The older age of the patients with malignant tumors with adequate serial biopsies in two cases supported the thesis that malignant transformation may occur in the benign tumors. Moreover the location of the tumor in one of our patients in whom the resected tumor was associated with anomalous right hepatic ducts and portal veins supported the theory that these tumors develop embryologically from nests of primitive hepatobiliary endodermal and mesodermal cells. Although surgical treatment was performed in all patients, 25% of the patients with benign hepatobiliary cystadenoma with mesenchymal stroma (CMS), and 33% of the patients with malignant CMS had tumor recurrence after primary resection. Ninety per cent of these patients had an incomplete resection at the time of their initial operations. Forty-four per cent of the patients with malignant CMS died after a mean follow-up of 17 months. We conclude that CMS (Edmonson's tumor) occurs uniquely in young female patients, develops from nests of primitive embryonal cells, has the potential for malignant transformation, and should be completely resected at primary operation to avoid recurrence.

The purpose of the present study was to examine the role played by structural differences among fatty acids in their effect on insulin secretion by isolated perifused murine islets. Insulin secretion measured by radioimmunoassay was assessed either as total insulin output (ng.6 islets-1.20 min-1) or as percent of basal insulin secretion. Raising the glucose concentration from a basal 5.5 to 27.7 mM caused an increase of insulin output from 6.69 +/- 1.59 to 19.92 +/- 4.99 ng.6 islets-1.20 min-1 (P < 0.05) in control (untreated) islets. However, after 20-min exposure of islets to 5 mM 16:0 or 18:2, the effect of 27.7 mM glucose was enhanced or diminished, respectively. Basal insulin output (100% basal) changed to 44 +/- 10% basal (P < 0.005) with the addition of 5 mM 4:0 but was not altered when 4:0 was replaced by 6:0. Insulin output increased modestly with 5 mM 8:0 but significantly (P < 0.05) with 10:0 until a maximal of 280 +/- 24% basal with 12:0 (P < 0.01), then fell to 110 +/- 18 and 93 +/- 15% basal (P < 0.05) with 14:0 and 16:0, respectively. The addition of 5 mM 18:0 inhibited insulin secretion to 30 +/- 10% of basal (P < 0.003), and this effect was not caused by fatty acid interference with insulin assay.(ABSTRACT TRUNCATED AT 250 WORDS)