Endocrine Neoplasia pp 105-116
Parathyromatosis and Parathyroid Cancer
Parathyroid carcinoma is a rare entity, accounting for less than 1% of all cases of primary hyperparathyroidism [1–5]. This chapter provides an overview of the clinical presentation, pathophysiology, and medical and surgical management of parathyroid carcinoma and highlights some of the ongoing controversies and areas of current research interest involving this uncommon disease, including: difficulties in distinguishing benign from malignant disease both macroscopically and histologically; recent developments in the understanding of the genetic basis for this disease; the use of calcimimetics and other medical therapies for nonoperative management; and the utility of multiple reoperations in patients with recurrent disease. This chapter also summarizes the current state of knowledge regarding parathyromatosis, defined as an overgrowth of multiple hyperfunctioning nodules of the parathyroid tissue, a condition which may develop after capsular fracture and intraoperative seeding during parathyroid tumor resection.