MEN1 patients at risk for venous thromboembolism

Patients with multiple endocrine neoplasia type 1 (MEN1) are at greater risk of experiencing venous thromboembolism (VTE), suggests a recent study.

“MEN1 is a rare inherited disorder predisposing the development of multiple functional and nonfunctional neuroendocrine tumours (NETs),” the authors said. “Only uncommon MEN1-associated functional NETs such as glucagonomas (<1 percent) and adrenocorticotropic hormone-producing tumours (<5 percent) are known to be associated with hypercoagulability.”

In this study, the authors looked into a prospective natural history study of germline mutation-positive MEN1 patients (n=286) between 1991 and 2019 for all lifetime events of VTE. The search terms used were as follows: DVT, thromb, embol, PE, pulmonary embolism, clot, haematology consult, anticoagulant, coumadin, lovenox, xarelto, warfarin, aspirin, rivaroxaban, and apixaban.

The authors then calculated the incidence rates, accounting for both age and sex, and compared these to published incidence rates in healthy populations, different cancer types, and Cushing’s syndrome.

Of the patients, 36 (median age, 45 years; range, 16–75) had a VTE event, resulting in a prevalence rate of 12.9 percent. The age–sex adjusted incidence rate of VTE stood at 9.11 per 1,000 patient-years, with a sex-adjusted lifetime incidence rate of 2.81 per 1,000 patient-years.

MEN1-associated lifetime incidence rates were nearly twofold higher than the estimated annual incidence rate in the general population and were comparable to the known risk in the setting of various cancer types.

Furthermore, about four in five patients who had a VTE were diagnosed with pancreatic NETs, of whom 24 percent were insulinomas. Fourteen patients (42 percent) experienced perioperative VTE events.

“Further mechanistic investigation and validation from other MEN1 cohorts are needed to confirm the increased prevalence of VTE in MEN1.