Recent advances in the management of endocrine malignancies associated with hereditary hyperparathyroidism syndromes

• Published in: International journal of endocrine oncology Vol. 4; no. 2; pp. 85 - 102
• Main Authors: Li, Yulong, Simonds, William F
• Format: Journal Article
• Language: English
• Published: London Future Medicine Ltd 01.05.2017
• Subjects: Cancer therapies; CDC73; familial hyperparathyroidism syndromes; Lymphatic system; medullary thyroid cancer; MEN1; Metastasis; Mutation; Neuroendocrine tumors; nonfunctioning pancreatic neuroendocrine tumor; Pancreatic cancer; parathyroid carcinoma; peptide receptor radionuclide therapy; Radiation therapy; RET; Surgery; thymic neuroendocrine tumor; Thyroid cancer; tyrosine kinase inhibitor
• ISSN: 2045-0869; 2045-0877
• DOI: 10.2217/ije-2016-0018

Hereditary hyperparathyroidism syndromes, such as multiple endocrine neoplasm type 1, type 2A and the hyperparathyroidism-jaw tumor syndrome, are associated with an increased incidence of malignancies involving the neuroendocrine tissue of the pancreas and thymus, parathyroid and thyroid glands. The natural history of these endocrine tumors can differ from nonhereditary malignancies. The surgical approach, the only potentially curative treatment option for these endocrine malignancies, has evolved considerably in recent years. Newer targeted therapies, such as small molecule kinase inhibitors, somatostatin analogs and peptide receptor radionuclide therapy, are being developed. We provide here a comprehensive review of the current standards of treatment and emerging novel therapies for the endocrine malignancies commonly associated with hereditary hyperparathyroidism syndromes.