Acromegaly caused by growth hormone-relating hormone in a patient with multiple endocrine neoplasia type I.

A 51-year-old Caucasian man with multiple endocrine neoplasia (MEN) type I syndrome presented with clinical features of acromegaly. Exploration of the pituitary gland only revealed somatotrophic hyperplasia and his plasma growth hormone (GH) levels remained elevated. Production of growth hormone-releasing hormone (GHRH) by an ectopic tumor was suspected and, after additional investigations, a large pancreatic tumor was detected and removed. As the pancreatic tail contained multiple (occult) adenomas, lifelong follow-up was considered necessary. The patient has been recurrence-free for 10 years. All 19 living relatives of this patient were analysed for endocrine disorders related to MEN I syndrome. A brother was found to suffer from peptic ulcer disease caused by hyperparathyroidism and, during screening for other organ involvement associated with the MEN I syndrome, two tumors were found, one (4 cm) in the pancreatic tail region and one in the right adrenal gland. To date, six other family members have been found to suffer or have suffered from hyperparathyroidism and in a male subject, a prolactinoma and hyperparathyroidism were detected.

201Tl-99mTc subtraction scintigraphy in secondary hyperparathyroidism of chronic renal failure.

In a group of 14 patients with chronic renal failure and secondary hyperparathyroidism, subtraction scintigraphy with 201Tl and 99mTc for parathyroid localisation was performed prior to subtotal parathyroidectomy. Fourteen of 38 (37%) enlarged hyperplastic glands found at first operation corresponded with the hot spots seen on the preoperative scans. Four of five (80%) found at a second operation had also been detected this way preoperatively. Postoperative scans were performed in nine patients. All hot foci seen on the preoperative scans had disappeared. In two patients new hot spots emerged correlating with known residual parathyroid tissue, and also in one patient with recurrent secondary hyperparathyroidism. We conclude that this diagnostic method is of limited value prior to the first neck exploration in secondary hyperparathyroidism of chronic renal failure. However it might be a useful method in the evaluation of patients with signs of persistent or recurrent hyperparathyroidism.

Thallium-201-technetium-99m parathyroid subtraction scintigraphy: dual channel acquisition or sequential imaging?

Parathyroid subtraction scintigraphy has been performed in 108 patients. In 41 cases scintigraphic findings are correlated with surgical and histologic results. Comparison of sequential data acquisition and dual channel acquisition has been made. As the results of these two protocols are comparable, we prefer the dual channel acquisition because of the smaller chance of introducing subtraction artefacts caused by patient movement.

Primary melanoma in the female distal urethra.

A case of primary urethral oligomelanotic melanoma is presented. Melanoma of the female distal urethra is a rare tumor with a poor prognosis. Imprint cytology was used for detection. Electron microscopy and immunohistochemistry were used to confirm the histologic diagnosis.

Scintigraphic detection of adenoma in primary hyperparathyroidism.

Ten patients with biochemical and clinical evidence of primary hyperparathyroidism were studied scintigraphically using a double-radionuclide subtraction technique. Preoperative imaging was performed with Sodium pertechnetate Tc 99m and thallium chloride Tl 201 using a gamma camera with a pinhole collimator. The data were stored in a matrix of 64 X 64. The avidity of 201Tl for nodules of the parathyroid gland was demonstrated on analogue and digital images both with and without the subtraction of thyroid tissue. In our ten patients, this technique detected nine out of ten histologically proven adenomas, and missed a relatively small lesion embedded within the thyroid of one patient. This method may have considerable advantages over non-specific or invasive radiodiagnostic methods, and may facilitate the diagnosis and planning of the surgical approach in cases of primary hyperparathyroidism.

Sarcoidosis of the liver, complicated by bleeding oesophageal varices.

Case report of a 57-year-old woman with pulmonary and hepatic sarcoidosis. As a rare complication, portal hypertension developed and first became manifest as bleeding from oesophageal varices. A portocaval shunt was successfully performed and the patient remained in stable condition after a follow-up of two years.

Surgical aspects of primary hyperparathyroidism.

A series of 217 operations performed for primary hyperparathyroidism between 1965 and 1978, was studied to evaluate the peroperative findings and to assess the results of surgical treatment. There was a greater incidence in women than in men, a ratio of 2.5:1. Although occurring at any age, the incidence was higher for women over the age of 45. During all 217 neck explorations it was attempted to identify all parathyroid glands. In 89% of cases three or more glands were found. Of the 746 glands, 74% were situated normally and 12% were found inside the thymus. Histological examination confirmed hyperplasia in 50% of the patients, but one enlarged gland was found in each of 155 patients (70%). This shows a discrepancy based on microscopic findings by the pathologist and macroscopic findings by the surgeon concerned. Until today it has been our policy to remove the enlarged gland and to biopsy at least one other normally sized gland. Only when three or more glands are involved, a subtotal parathyroidectomy is recommended. The success rate of our primary operation is 96%. Complications due to severe hypocalcaemia or permanent vocal cord palsy have not been observed.