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Objective To report the prevalence of parathyroid carcinoma ( PC) in patients with multiple endocrine neoplasia type 1 ( MEN1) and review of the literature. Background Primary hyperparathyroidism ( PHP) is the most common manifestation of MEN1. The occurrence of PC in patients with MEN1 is rare and the literature regarding the clinical manifestations - including the prevalence of the disease - is scarce. Context Single tertiary care centre experience from 1977 to 2013. Design Electronic search of the medical records to identify a cohort of patients with MEN1. Literature review based on current case reports. Patients Single case of PC in a cohort of 348 patients with MEN1. Ten cases of PC in patients with MEN1 reported in the literature. Measurement Clinical features of PC in patients with MEN1. Results The prevalence of PC in 348 patients with MEN1 was found to be 0·28% (95% CI, 0-1·4%). Based on the current published cases of PC in patients with MEN1, 54·5% were women, mean age at diagnosis was 48·3 years, and the serum PTH concentrations at least four times the upper limit of the reference range in 73% of the cases. Conclusion PC in patients with MEN 1 is rare with a prevalence of 0·28%, and the clinical features are similar to PC in patients without MEN1. [ABSTRACT FROM AUTHOR]

Patients with multiple endocrine neoplasia type 1 (MEN1) are commonly evaluated for clinical manifestations of this syndrome with the rationale that early diagnosis and adequate treatment will result in improved survival and quality of life. Thymic and bronchial carcinoid tumours are uncommon but important manifestations of MEN1. Current practice guidelines recommend evaluation with computed tomography or magnetic resonance imaging scan of the chest every 1-2 years to detect these neoplasms. However, the certainty that patients will be better off (increased survival or quality of life) as a result of this case detection strategy is based on evidence at moderate-high risk of bias that yields only imprecise results of indirect relevance to these patients. In order to improve the care that patients with MEN1 receive, co-ordinated efforts from different stakeholders are required so that large, prospective, multicentre studies evaluating patient important outcomes are carried out. [ABSTRACT FROM AUTHOR]