The spectrum and extent of islet cell histopathological findings in patients with multiple endocrine neoplasia, type I (MEN I) syndrome has never been clearly defined. Although some patients have discreet tumors causing clinically evident syndromes, others may have no symptoms until metastatic islet cell carcinoma is apparent. Whether diffuse islet cell disease occurs in all patients with grossly apparent tumors is not known. This study is an attempt to define both the functional and anatomical extent of islet cell disease and its relationship with the clinical course of patients with MEN I syndrome.The resected specimens of pancreas from 14 patients with MEN I syndrome were evaluated for hyperplasia, nesidioblastosis, multiple tumors, and evidence of malignancy. In 12 cases, specimens consisted of distal pancreas and, in 2 cases, the entire pancreas was available. Multiple sections were taken from each specimen. Immunoperoxidase staining was done for gastrin, pancreatic polypeptide, glucagon, serotonin, VIP, somatostatin, and neuron-specific enolase in sections of 24 tumors from 10 patients. Five of the 10 patients with Zollinger-Ellison syndrome underwent total gastrectomy and 3 others underwent only pancreatic procedures to control their acid hypersecretion.The following is concluded. All MEN I patients with pancreatic neoplasms have diffuse islet cell involvement consisting of nesidioblastosis, micro-and macronodular hyperplasia. Some tumors produce multiple hormones and these patients are at risk to develop new tumors, but complete excision of grossly apparent tumors may result in

We describe nine patients with underlying hematologic cancer in whom primary cutaneous aspergillosis developed at the sites of Hickman intravenous catheters. Our patients, 17 to 74 years of age, were all immunocompromised either from their primary disease or from chemotherapy, and the Hickman catheters had been placed to provide venous access for chemotherapy or hyperalimentation or both. Clinical signs of infection included erythema, induration, and cutaneous or subcutaneous necrosis at the point of entry into the subclavian vein, in the subcutaneous tunnel, or at the exit site from the skin. Diagnosis was confirmed by positive wound culture for Aspergillus flavus in all but one patient. Treatment consisted of intravenous amphotericin B, oral flucytosine, and local wound care. Three patients recovered completely without operative débridement; three others recovered after operative débridement and delayed grafting. Two patients died of disseminated aspergillosis, and one died of unrelated causes while recovering from primary cutaneous aspergillosis. Successful treatment required resolution of aplasia or leukopenia, catheter removal, systemic treatment with amphotericin B, and local wound care. We conclude that primary cutaneous aspergillosis, a rare infection, may occur at the sites of Hickman catheters in immunocompromised patients, and that it is a serious complication requiring prompt diagnosis and treatment.

Primary hyperparathyroidism (HPT) is considered a rare disease in children and an uncommon one in adolescents and young adults. Until 1975, only 60 children under the age of 16 years had been reported with proven HPT. Most had symptoms of weakness, irritability, anorexia, and weight loss. Severe hypercalcemia (serum calcium > 15 mg/ 100 ml) and radiologic evidence of bone changes were common findings, and suggested delayed diagnoses. This report is based on the clinical and laboratory findings in 53 young patients with proven primary HPT (range: 1-30 years of age) from 1971 to 1980, treated in one hospital. There were 29 male and 24 female patients, 26 of whom developed symptoms before age 18. Common symptoms included hematuria and renal colic (50%), renal calculi (50%), and hypertension with (6%) and without (3%) severe headaches. Although 64.2% of patients had adenomas, only 54% of patients under 18, and as many as 77.8% over 18, had them. The incidence of hyperplasia was markedly increased in the patients under 18 (38%) as compared to the patients over 18 (18.5%) or the group taken as a whole (30.2 %). The following associated diseases were identified: MEA I syndrome (4); MEA II syndrome (4); von Recklinghausen's neurofibromatosis; papillary carcinoma of thyroid; craniopharyngioma; and multiple metaphyseal chondromatosis. One child had hereditary neonatal parathyroid hyperplasia. Primary hyperparathyroidism is more common than previously suspected in young people. Symptoms of renal stones, hypertension, persistent headaches, unexplained anorexia, and weight loss should prompt evaluation for primary HPT. If hyperplasia is found, the patient and family should be investigated for associated endocrinopathies.

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These data demonstrate that the size of a Hürthle cell neoplasm is predictive of malignancy. Therefore, at the time of initial exploration for large Hürthle cell neoplasms (>4 cm), definitive resection involving both thyroid lobes should be considered because of the higher probability of malignancy.