Clinical Endocrinology (2016) 84, 244–249
doi: 10.1111/cen.12714
ORIGINAL ARTICLE
Prevalence of parathyroid carcinoma in 348 patients with multiple endocrine neoplasia type 1 – case report and review of the literature Naykky Singh Ospina*, Thomas J. Sebo†, Geoffrey B. Thompson‡, Bart L. Clarke* and William F. Young Jr* *Division of Endocrinology, Diabetes, Metabolism and Nutrition, Department of Internal Medicine, Mayo Clinic, Rochester, MN, †Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN and ‡Section of Endocrine Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA
Introduction Summary 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 028% (95% CI, 0–14%). Based on the current published cases of PC in patients with MEN1, 545% were women, mean age at diagnosis was 483 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 028%, and the clinical features are similar to PC in patients without MEN1. (Received 20 October 2014; returned for revision 25 November 2014; finally revised 5 December 2014; accepted 27 December 2014)
Correspondence: William F. Young Jr, Mayo Clinic Rochester, 200 First Street Southwest, Rochester, Minnesota-55905, USA. Tel.: 507 284 2511; E-mail: [email protected]
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Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disease characterized by the presence of endocrine tumours affecting the parathyroid, pituitary and pancreas. Primary hyperparathyroidism (PHP) is the most common manifestation of this syndrome occurring in more than 95% of the cases and it involves all of the parathyroid glands.1 PHP is also the most common cause of hypercalcaemia in the outpatient setting and is usually caused by overactivity of a single parathyroid adenoma. In contrast, parathyroid carcinoma (PC) is a rare malignancy that accounts for
doi: 10.1111/cen.12714
ORIGINAL ARTICLE
Prevalence of parathyroid carcinoma in 348 patients with multiple endocrine neoplasia type 1 – case report and review of the literature Naykky Singh Ospina*, Thomas J. Sebo†, Geoffrey B. Thompson‡, Bart L. Clarke* and William F. Young Jr* *Division of Endocrinology, Diabetes, Metabolism and Nutrition, Department of Internal Medicine, Mayo Clinic, Rochester, MN, †Division of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN and ‡Section of Endocrine Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA
Introduction Summary 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 028% (95% CI, 0–14%). Based on the current published cases of PC in patients with MEN1, 545% were women, mean age at diagnosis was 483 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 028%, and the clinical features are similar to PC in patients without MEN1. (Received 20 October 2014; returned for revision 25 November 2014; finally revised 5 December 2014; accepted 27 December 2014)
Correspondence: William F. Young Jr, Mayo Clinic Rochester, 200 First Street Southwest, Rochester, Minnesota-55905, USA. Tel.: 507 284 2511; E-mail: [email protected]
244
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disease characterized by the presence of endocrine tumours affecting the parathyroid, pituitary and pancreas. Primary hyperparathyroidism (PHP) is the most common manifestation of this syndrome occurring in more than 95% of the cases and it involves all of the parathyroid glands.1 PHP is also the most common cause of hypercalcaemia in the outpatient setting and is usually caused by overactivity of a single parathyroid adenoma. In contrast, parathyroid carcinoma (PC) is a rare malignancy that accounts for
