Parathyroid Carcinoma and Ectopic Secretion of Parathyroid hormone

Filomena Cetani; Elena Pardi; Claudio Marcocci

doi:10.1016/j.ecl.2021.07.001

Parathyroid Carcinoma and Ectopic Secretion of Parathyroid hormone

Endocrinol Metab Clin North Am. 2021 Dec;50(4):683-709. doi: 10.1016/j.ecl.2021.07.001.

Authors

Filomena Cetani¹, Elena Pardi², Claudio Marcocci³

Affiliations

¹ University Hospital of Pisa, Endocrine Unit 2, Via Paradisa, 2, Pisa 56124, Italy. Electronic address: cetani@endoc.med.unipi.it.
² Department of Clinical and Experimental Medicine, University of Pisa, Via Paradisa, 2, Pisa 56124, Italy.
³ University Hospital of Pisa, Endocrine Unit 2, Via Paradisa, 2, Pisa 56124, Italy; Department of Clinical and Experimental Medicine, University of Pisa, Via Paradisa, 2, Pisa 56124, Italy.

PMID: 34774241
DOI: 10.1016/j.ecl.2021.07.001

Abstract

The most common causes of hypercalcemia are primary hyperparathyroidism (PHPT) and malignancy. Parathyroid carcinoma (PC), causing a severe PHPT, is the rarest parathyroid tumor. A diagnosis of PC is challenging because the clinical profile overlaps with that of benign counterpart. Surgery is the mainstay treatment. CDC73 mutations have been detected in up to 80% of sporadic PCs. Ectopic production of parathyroid hormone (PTH) by malignant nonparathyroid tumors is a rare condition accounting for less than 1% of hypercalcemia of malignancy. PTH secretion can be considered an aberration in the tissue specificity of gene expression and may involve heterogeneous molecular mechanisms.

Keywords: CDC73; HPT-JT; Hypercalcemia; PI3K/AKT/mTOR; PTH; Parafibromin; Primary hyperparathyroidism.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Humans
Hypercalcemia* / etiology
Parathyroid Hormone
Parathyroid Neoplasms* / complications
Parathyroid Neoplasms* / diagnosis
Parathyroid Neoplasms* / genetics

Substances

Parathyroid Hormone