Sizemore, Glen W.

A healthy 27‐year‐old man with a lump in the lateral midneck was examined. Results of a recent biopsy performed elsewhere indicated amelanotic melanoma. A careful search for a primary integumentary or upper respiratory site of origin, including appropriate roentgenograms and a thyroid scan, was negative.At right radical neck dissection, the pathologic diagnosis was expanded to include anaplastic carcinoma. Subsequent examinations of the head and neck were negative. The neck and upper aerodigestive system were subjected to a full course of radiotherapy.Pathologic study of this tumor was continued. Electron microscopy indicated the tumor was epithelial, possibly of glandular epithelial origin.A tumor nodule reappeared in the right tracheoesophageal groove. Surgery was performed and the nodule was removed. The right thyroid lobe was removed also and submitted for histopathologic study. A solitary microscopic tumor focus, presumably anaplastic thyroid carcinoma, was found within the thyroid. Further pathologic studies were undertaken. Eventually, amyloid deposits were discovered, and a final diagnosis of cellular medullary thyroid carcinoma was established. Further studies of this patient and his family for multiple endocrine adenoma have been negative.

An enzymatic activity that catalyzes the conversion of glandular parathyroid hormone to a smaller-molecular-weight, biologically and immunologically active form of this hormone has been extracted from normal parathyroid and other porcine tissues. The product of the conversion has the immunologic characteristics of the hormone that occurs in peripheral serum and in tissue culture medium from parathyroid explants. The enzyme is activated by chelating agents and is inhibited by high calcium concentrations, suggesting that this enzyme may be important in the regulation of secretion or metabolism of the hormone.

AbstractDuring a 30‐year period (1951–1981), seventeen patients underwent bilateral adrenalectomy for established adrenal medullary disease with catecholamine excess. Fourteen patients had the MEN IIa syndrome and 3 had the MEN IIb syndrome. There was no major operative morbidity and no operative mortality.One patient died 23 months after initial operation because of metastatic pheochromocytoma. The remaining patients were followed for a mean of 129 months and all were personally interviewed. Three patients had 7 uneventful pregnancies. Twelve patients underwent 23 other surgical procedures requiring general anesthesia during the followup period without any morbidity. Two patients have metastatic pheochromocytoma; 3 have clinical metastatic medullary thyroid carcinoma; 5 patients have high calcitonin values and 6 patients are in excellent health at intervals of 15, 27, 58, 110, 134, and 373 months following resection. The need for adrenal replacement therapy has not caused significant problems in any patient.The results of this study confirm that bilateral total adrenalectomy is a safe modality for the treatment of adrenal medullary disease in this syndrome and that it does not produce significant long‐term morbidity.