Steven E Rodgers1, Nancy D Perrier. 1. Department of Surgical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas 77230-1402, USA.
Abstract
PURPOSE OF REVIEW: This article reviews current knowledge on the etiology, diagnosis and treatment of parathyroid carcinoma. RECENT FINDINGS: Due to its rarity, research on the molecular etiology and treatment of parathyroid carcinoma has been slow. Mutations of the tumor suppressor gene, HRPT2, and resultant loss of expression of its gene product have been found in the majority of parathyroid cancers studied. Recent advances in the field have identified regions on several chromosomes that demonstrate loss of heterozygosity more commonly in parathyroid carcinoma than in benign parathyroid lesions. This has provided clues to the location and identity of additional tumor suppressor genes associated with the development of this cancer. SUMMARY: Parathyroid carcinoma is an extremely rare cause of primary hyperparathyroidism, accounting for fewer than 1% of cases. The etiology of parathyroid cancer is largely unknown. Associations have been made with several inherited syndromes and with specific genetic lesions. Little is known about the most appropriate management of this disease. En bloc resection at the time of initial surgery appears to provide the best chance of cure. Anecdotal experience with adjuvant chemotherapy has shown a modest and short-lived effect. External beam radiation following surgical resection, however, may increase long-term survival compared with surgery alone. Bisphosphonates and a new class of drugs known as calcimimetics have been used effectively in some patients to control the symptoms of severe hypercalcemia in a palliative setting.
PURPOSE OF REVIEW: This article reviews current knowledge on the etiology, diagnosis and treatment of parathyroid carcinoma. RECENT FINDINGS: Due to its rarity, research on the molecular etiology and treatment of parathyroid carcinoma has been slow. Mutations of the tumor suppressor gene, HRPT2, and resultant loss of expression of its gene product have been found in the majority of parathyroid cancers studied. Recent advances in the field have identified regions on several chromosomes that demonstrate loss of heterozygosity more commonly in parathyroid carcinoma than in benign parathyroid lesions. This has provided clues to the location and identity of additional tumor suppressor genes associated with the development of this cancer. SUMMARY:Parathyroid carcinoma is an extremely rare cause of primary hyperparathyroidism, accounting for fewer than 1% of cases. The etiology of parathyroid cancer is largely unknown. Associations have been made with several inherited syndromes and with specific genetic lesions. Little is known about the most appropriate management of this disease. En bloc resection at the time of initial surgery appears to provide the best chance of cure. Anecdotal experience with adjuvant chemotherapy has shown a modest and short-lived effect. External beam radiation following surgical resection, however, may increase long-term survival compared with surgery alone. Bisphosphonates and a new class of drugs known as calcimimetics have been used effectively in some patients to control the symptoms of severe hypercalcemia in a palliative setting.
Authors: C Christofer Juhlin; Inga-Lena Nilsson; Kenth Johansson; Felix Haglund; Andrea Villablanca; Anders Höög; Catharina Larsson Journal: Endocr Pathol Date: 2010-09 Impact factor: 3.943
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