Parathyroid carcinoma in multiple endocrine neoplasm type 1 syndrome: case report and systematic literature review
- 158 Downloads
The aim of this report was to illustrate a case of parathyroid carcinoma (PC) in a patient with multiple endocrine neoplasia type 1 (MEN1) along with a comprehensive literature review. A 61-year-old man presented with 9-cm PC causing primary hyperparathyroidism (PHPT). His pre-operative corrected calcium and intact PTH serum levels were 2.92 mmol/L and 391.7 pg/mL, respectively. The neoplastic gland was removed in bloc with thyroid and central compartment lymph nodes. A literature review was run by searching PubMed MEDLINE from 1977 to 2018 for studies of all types, in the English language only, using the terms “Parathyroid, carcinoma, Multiple endocrine neoplasia, type 1, (MEN1).” Pathology confirmed PC. Post-operative calcium and PTH levels were normal. A diagnosis of MEN1 was established post-operatively. Seventeen cases of PC in patients with MEN1 have been reported in the literature. 59% of patients were men, and median age at diagnosis was 50 years, with median serum PTH of 379 pg/mL and median serum calcium level of 3.2 mmol/L. The occurrence of PC in the context of MEN1 is extremely rare. Diagnosis and treatment may represent a challenge, so opportune identification or suspicion of malignancy and adoption of correct surgical approach may offer affected patients the best outcome.
KeywordsParathyroid carcinoma Multiple endocrine neoplasia type 1 MEN1 Primary hyperparathyroidism Cancer Surgery
Multiple endocrine neoplasia type 1
GDM contributed to conception and design of the study, case and literature review, draft writing, manuscript review, and intellectual input. LIS and VF contributed to case and literature review, draft writing, and manuscript review. FPP and AG contributed to draft writing, manuscript review, and intellectual input. MT helped in design and coordination of the study, draft writing, manuscript review, and intellectual input. All the authors read and approved the final manuscript.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflicts of interest.
- 1.Hundahl SA, Fleming ID, Fremgen AM, Menck HR. Two hundred eighty-six cases of parathyroid carcinoma treated in the U.S. between 1985 and 1995: a National Cancer Data Base Report. The American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer. 1999;86:538–44.CrossRefGoogle Scholar
- 8.Kalavalapalli S, Talapatra I, Patrick I. A complex case of multiple endocrine neoplasia type 1 with metastatic parathyroid carcinoma. Cent Eur J Med. 2010;5:53–8.Google Scholar
- 20.LiVolsi VA. Pathology of the parathyroid gland. In: Barnes L, editor. Surgical pathology of the head and neck. New York: Marcel Dekker; 1985. p. 1487.Google Scholar