Session: MON 199-237-Disorders of Parathyroid Hormone & Calcium Homeostasis
Poster Board MON-207
Parathyroid carcinoma is a rare cause of primary hyperparathyroidism, accounting for less than 1% of cases (1). Concurrent parathyroid carcinoma of a single gland and hyperplasia has been previously reported. We describe a case of sporadic parathyroid carcinoma involving two glands with hyperplasia of a third parathyroid gland.
A 52 year-old man presented to the emergency department with chronic fatigue, hoarseness, bone pain and a palpable neck mass in the region of the right lobe of the thyroid. Chest x-ray showed tracheal deviation to the left. Past medical history was notable for recurrent nephrolithiasis, chronic hypercalcemia and osteoporosis of the forearm, hip and spine. PTH was elevated to 712 ng/L (normal 7-53 ng/L) with a calcium of 12.1 mg/dL (normal 9.0-11.0 mg/dL). Neck ultrasound revealed a 3.4 x 2.0 cm mixed solid and cystic nodule inferior to the right thyroid lobe. Fine needle aspiration of the lesion yielded a markedly elevated level of PTH in the needle rinse (> 1900 ng/L). Tc-99m-Sestimibi (MIBI) scan confirmed a large focus of delayed MIBI retention in the region of the right inferior parathyroid. A second focus was seen in the region of the left upper parathyroid. Upon surgical exploration, the right inferior gland was enlarged, highly vascular with multiple adhesions to the surrounding tissue and to the thyroid gland. The left superior parathyroid gland was also enlarged and intimately associated with the left upper pole of the thyroid. The right superior parathyroid though enlarged, was normal in appearance. These three parathyroid glands along with the thyroid were resected. Pathology confirmed carcinoma of the right inferior and left superior parathyroid glands, possessing the features first described by Schantz and Castleman (2). The right superior parathyroid showed chief cell hyperplasia. The patient is currently receiving calcitriol and calcium supplementation and is without evidence of recurrence 6 months post-operatively. Genetic evaluation was negative for MEN1, MEN2 A/B and for HRPT2 gene mutations.
This case illustrates that multiple parathyroid glands can be involved in parathyroid carcinoma, and can occur in the presence of parathyroid hyperplasia. It also highlights the need to consider a four-gland exploration when parathyroid carcinoma is suspected.
Nothing to Disclose: RMP, MAA, UKS
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