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Revista Portuguesa de Cirurgia

Print version ISSN 1646-6918

Abstract

BARBOSA, Ana  and  COSTA, João Capela. Parathyroid Carcinoma. Rev. Port. Cir. [online]. 2018, n.44, pp.25-36. ISSN 1646-6918.

Parathyroid carcinoma (PC) is a very rare endocrine tumor, accounting for less than 1% of cases of primary hyperparathyroidism (P-HPT). As in many other tumors, its etiology is unknown, however, mutations in gene HRPT2/CDC73 have recently been found to play a key role in the pathogenesis of this neoplasm. PC is usually a sporadic disease but may occur in the context of genetic syndromes and is reported in patients with secondary hyperparathyroidism. Its diagnosis is a challenge, both clinically and histologically due to the absence of pathognomonic characteristics that allow early distinction between benign and malignant disease. The treatment with the best curative potential is the en bloc resection of the primary tumor with clear margins, so its success depends on the preoperative or intraoperative suspicion of carcinoma. PC has an extended clinical course with multiple recurrences, most of which occur locally, so patients should be monitored for the rest of their lives. Given the limited value of adjuvant therapies, the recommended treatment in cases of recurrence and metastasis is, a surgical resection, whenever possible.

Keywords : Parathyroid Carcinoma; Primary Hyperparathyroidism; Gene HRPT2/CDC73; Parafibromin; Parathyroidectomy.

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