Parathyroid carcinoma

Antonio Stefano Salcuni, Filomena Cetani, Vito Guarnieri, Vincenzo Nicastro, Elisabetta Romagnoli, Danilo de Martino, Alfredo Scillitani, David E.C. Cole

Research output: Contribution to journalReview article

Abstract

Parathyroid carcinoma (PC) is a rare disease with an indolent behavior due to the low malignant potential. The etiology is unknown. Somatic mutations of CDC73 gene, the same gene involved in the hyperparathyroidism-jaw tumor syndrome, can be identified in up to 70% of patients with PC and in one-third of cases the mutations are germline. Therefore, in patients who carry germline CDC73 gene mutations, its finding permits to identify the carriers among relatives and sometimes to early detect a parathyroid lesion in such subjects. The diagnosis of PC is commonly made after surgery, however there are some clinical/biochemical features that should raise the suspicion of PC, namely markedly elevated serum calcium and PTH levels, a large parathyroid lesion with suspected ultrasonographic features of malignancy, the damages of kidney and bones. The best chance of cure is the complete surgical resection with the en-bloc excision at the first operation, however several recurrences are often observed during the follow-up. Since PC is an indolent tumor with long-lasting survival and the death is due to complications of untreatable hypercalcemia, multiple surgical interventions with debulking of tumoral tissues along with medical treatment for reducing hypercalcemia are often needed. Patients with PC should be followed up along their lifetime.

Original languageEnglish
Pages (from-to)877-889
Number of pages13
JournalBest Practice and Research: Clinical Endocrinology and Metabolism
Volume32
Issue number6
DOIs
Publication statusPublished - Dec 1 2018

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Keywords

  • CDC73 gene
  • HPT-JT syndrome
  • hypercalcemia
  • parathyroid carcinoma

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

Salcuni, A. S., Cetani, F., Guarnieri, V., Nicastro, V., Romagnoli, E., de Martino, D., Scillitani, A., & Cole, D. E. C. (2018). Parathyroid carcinoma. Best Practice and Research: Clinical Endocrinology and Metabolism, 32(6), 877-889. https://doi.org/10.1016/j.beem.2018.11.002