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Endocrinology

Parathyroid Carcinoma: Current Understanding and New Insights into Gene Expression and Intraoperative Parathyroid Hormone Kinetics

Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA

Key Words. Parathyroid carcinoma • Intraoperative PTH • Gene expression

Correspondence: John A. Olson, Jr., M.D., Ph.D., Duke University Medical Center, Durham, North Carolina 27710, USA. Telephone: 919-668-1767; Fax: 919-668-4777; e-mail: jaomd{at}duke.edu

Received August 17, 2009; accepted for publication November 30, 2009; first published online in THE ONCOLOGIST Express on January 5, 2010.

Disclosures

Mohamed Abdelgadir Adam: None; Brian R. Untch: None; John A. Olson, Jr.: None.

Section editors Herbert Chen and Stan B. Sidhu have disclosed no financial relationships relevant to the content of this article.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias.

Parathyroid carcinoma is an indolent but ultimately life-threatening malignancy. Due to the lack of definitive diagnostic markers and overlapping clinical features of benign primary hyperparathyroidism (PHPT), this disease is often misdiagnosed as parathyroid adenoma. Therefore, a high index of suspicion preoperatively and early intraoperative recognition with en bloc surgical resection are crucial for favorable outcome. Owing to the rarity of the disease, little is known about the molecular pathogenesis of parathyroid carcinoma. Here, we review the literature to present current understanding of the disease and provide new information on gene expression and use of intraoperative parathyroid hormone (PTH) monitoring in the surgical management of this rare malignancy. Specifically, using microarray transcriptome analysis of an unequivocal case of parathyroid carcinoma and a biopsy from the same patient's normal parathyroid gland, we identify APP, CDH1, KCNJ16, and UCHL1 as differentially expressed genes in parathyroid carcinoma. Further, using case records from four cases of unequivocal parathyroid carcinoma, we compared intraoperative PTH kinetics of these patients to 475 patients with benign PHPT, and show that intraoperative PTH monitoring is accurate in predicting postoperative normocalcemia in initial en bloc operations for parathyroid carcinoma.