Medullary carcinoma of the thyroid with parathyroid adenoma and hypercalcaemia

Abstract

SYNOPSIS A patient with hyperparathyroidism due to a parathyroid adenoma was found to have metastatic medullary carcinoma in lymph nodes close to the thyroid gland. The finding of normal calcitonin and parathormone concentrations after removal of the adenoma suggested that the parathyroid lesion was not the result of the hypocalcaemic action of calcitonin produced by the tumour in the thyroid.Seventeen other examples of the association between medullary carcinoma and parathyroid enlargement are briefly reviewed.The pathogenesis of the parathyroid lesions is discussed, and it is suggested that some may be caused by the hypocalcaemic action of calcitonin, while others may be part of a syndrome of multiple endocrine adenomatosis.Medullary carcinoma is a special form of thyroid carcinoma characterized by the presence of amyloid in the tumour tissue (Williams, Brown, and Doniach, 1966) and by the production of calcitonin (Cunliffe, Black, Hall, Johnston, Hudgson, Shuster, Gudmundsson, Joplin, Williams, Woodhouse, Galante, and Maclntyre, 1968). Medullary carcinoma may be familial and is sometimes associated with phaeochromocytoma and multiple mucosal neuromata (Williams et al, 1966).In 17 cases taken from the literature, parathyroid adenoma or hyperplasia has been an additional feature (Table I) and in six of these there was evidence of hypercalcaemia (Table I, cases 12, 13, 14, 15, 16, 17).We wish to report a further example of the association between medullary carcinoma and parathyroid adenoma in a patient who presented with hypercalcaemia and nephrocalcinosis. Case ReportA 52-year-old woman was seen in 1967, complaining of two episodes of renal pain and the passage of small renal calculi. An intravenous pyelogram showed calcification in the medullary pyramids. The serum calcium was 11-6 mg/100 ml (normal 8-8-10-6 mg/100 ml) and the phosphorus 2-0 mg/100 ml Received for publication 26 May 1971.(normal 2 5-45 mg/100 ml). Inpatient investigation was advised, but the patient, who was by then feeling well, declined.A year later she was seen again having passed further stones. At this time the serum calcium was 9.4 mg/100 ml and the phosphorus 3-1 mg/100 ml.Early in 1970 she began to complain of extreme lassitude, tiredness, and backache. Radiographs in June 1970 again showed calcification in both kidneys. No definite phalangeal erosions were seen, but the skull showed irregular mottling and multiple translucent areas. The serum calcium was now 12-9 mg/ 100 ml and the phosphorus 2-1 mg/100 ml. A diagnosis of hyperparathyroidism was made and the neck was explored.At operation an enlarged right superior parathyroid was identified and removed. The right lower parathyroid was not identified, but the left superior and left inferior parathyroids were not enlarged. The thyroid was nodular and a circumscribed nodule found in the lower pole of the left lobe was enucleated. Three small nodules lying close to the left lobe of the thyroid were also removed. They were thought to be lymph nodes, but due to the lack of frozen sectio...