Basilar artery aneurysm simulating pheochromocytoma.

Stimulation of sympathetic vasomotor centers by lesions in the posterior fossa has been described in humans and animals. We describe a patient with a pheochromocytoma-like syndrome and a basilar artery aneurysm. Repair of the aneurysm was followed by disappearance of hypertensive episodes. Posterior fossa lesions must be considered in patients with hypertensive crises when diagnostic procedures do not indicate an adrenal or extra-adrenal pheochromocytoma.

Operative and chemotherapeutic management of malignant glucagon-producing tumors.

Glucagon-producing tumors of the pancreas are among the rarest forms of islet cell tumors. Two patients are described in whom the characteristic dermatitis, glucose intolerance, weight loss, and anemia of the glucagonoma syndrome were due to a metastasizing islet cell carcinoma. In both, removal of the primary tumor with a distal pancreatectomy brought marked relief of all clinical symptoms for 1- and 2-year periods. Because streptozocin, the usual chemotherapeutic agent for these tumors, is quite toxic and frequently unsuccessful dimethyltriazenoimidazole carboximide (DTIC) was used for recurrence after operation. The first patient began taking DTIC when his rash reappeared and his immunoreactive glucagon (IRG) level rose to 6,000 pg/ml (normal, less than 200 pg/ml). Within 3 months, his rash was gone, and IRG level was 75 pg/ml. The second patient developed a neoplastic gastrocolic fistula and was extremely emaciated. With DTIC, the fistula healed, and he gained weight and returned to work. His IRG level has decreased from 2,975 to 200 pg/ml. No side-effects were noted during chemotherapy. Temporary palliation of malignant glucagon-producing neoplasms can be achieved by cytoreductive surgery. When the life-threatening symptoms of this syndrome recur, DTIC chemotherapy seems indicated because of its safety and effectiveness.

Evaluation of thyroid nodules.

The first consideration in evaluating the thyroid nodule is whether it is functioning and causing hyperthyroidism. Autonomous nodules should be treated with either surgery or I-31, with surgery favored due to the possibility, although small, of malignancy. Thyroid scans are no longer recommended during the initial evaluation of the thyroid nodule. Ultrasound is useful in determining the size of the nodule and whether it is multinodular thyroid disease, but it cannot detect thyroid cancer. Fine needle aspiration biopsy is currently the procedure of choice for evaluating all thyroid nodules. For accuracy of the cytological analysis, it is important that adequate tissue samples be obtained. False negative findings are of most concern to the clinician and occur in 2-10% of reported fine needle aspiration biopsy series. Nodules thought to be benign will need continued follow-up.

Clinical aspects of glucagon-producing islet cell tumors.

Review of the 55 reported cases of glucagon-producing tumors reveals that a distinctive clinical syndrome consisting of diabetes, a peculiar dermatitis termed necrolytic migratory erythema, weight loss and an increased tendency for thrombosis is associated with these neoplasms. Normochromic normocytic anemia, hypocholesterolemia, hypoproteinemia and generalized hypoaminoacidemia are frequent laboratory findings. Definitive diagnosis of a glucagonoma requires elevation of the fasting serum glucagon level. Selective arteriography of the pancreas has been the best method for localizing these neoplasms preoperatively, but the noninvasive technics of ultrasound and CAT scanning can also be helpful. When the tumor is benign, complete surgical excision can completely reverse all the clinical manifestations of the glucagonoma syndrome and result in lasting cure. Since, however, approximately three-fourths of these tumors are malignant, palliative therapy is frequently required. Cytoreductive surgery can decrease the amount of hormone-producing tissue and can improve or even temporarily reverse the clinical symptomatology. For disseminated disease, chemotherapy is necessary. The best results have been obtained with DTIC although streptozotocin has also been used.

45,X/46,XY/47,XY, +21 mosaicism in a hypogonadal phenotypic male.

A phenotypically normal male was found to have a chromosomal complement of 45,X/46,XY/47,XY, +21. This mosaic pattern has been reported only twice before. Although the patient had apparently fathered two children, he now has progressive impotence, absence of sperm in the seminal fluid, atrophic testes, almost complete absence of germ cells in testicular biopsies, high plasma LH and FSH, and a low normal testosterone. There were no physical characteristics of Turner's or Down's syndromes except for dermatoglyphic features commonly associated with the latter. These observations in this patients emphasise the value of chromosomal studies in multiple tissues in cases of mosaicism with atypical clinical features.