We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Approach to the Patient With Parathyroid Carcinoma.
- Authors
Cetani, Filomena; Pardi, Elena; Torregrossa, Liborio; Borsari, Simona; Pierotti, Laura; Dinoi, Elisa; Marcocci, Claudio
- Abstract
Parathyroid carcinoma (PC) is usually associated with severe symptomatic primary hyperparathyroidism (PHPT) and accounts for less than 1% of all cases of PHPT and approximately 0.005% of all cancers. PC most commonly occurs as a sporadic disease and somatic CDC73 mutations can be detected in up to 80% of cases. Approximately 30% of patients harbor a germline mutation of the CDC73 gene. Preoperative diagnosis of PC is difficult because no disease-specific markers are available, and PC should be suspected in patients with severe hypercalcemia and end-organ complications. The diagnosis is based on the evidence of invasive tumor growth at histology and/or metastases. En bloc resection of the tumor, together with the ipsilateral thyroid lobe and adjacent structures, should be performed by an experienced surgeon when PC is suspected. This surgical approach reduces the risk of recurrence and metastasis and offers the highest chance of cure. Nonetheless, PC has a recurrence rate of 40% to 60% and, if feasible, multiple surgical procedures should be performed. When surgery is no longer an option, medical treatment is aimed to reduce hypercalcemia and target organ complications. Targeted agents have been effectively used in a few cases. We describe herein a patient with severe PHPT due to PC and provide a systematic diagnostic and treatment approach. A thorough review of the medical history, a typical clinical and biochemical phenotype and, in some cases, the revision of the histological examination provide the clues for the diagnosis of PC.
- Subjects
PARATHYROID gland cancer; CANCER treatment; HYPERPARATHYROIDISM
- Publication
Journal of Clinical Endocrinology & Metabolism, 2024, Vol 109, Issue 1, p256
- ISSN
0021-972X
- Publication type
Article
- DOI
10.1210/clinem/dgad455