Incidence rate of primary cardiac tumors: a 14-year population study

Abstract

Aims: Incidence of primary cardiac tumors (PCTs) is not known. Literature data derive from autoptic studies or echocardiographic registries. An incidence of 1 of 1000 autoptic and 1.5 of 1000 echocardiographic study is reported but data from a general population are not available. The aim of our study was to evaluate the incidence rate of PCTs in the general population.

Methods: All patients with suspected cardiac mass were evaluated with basal echocardiogram and/or transesophageal echocardiogram/cardiac magnetic resonance by Grosseto's cardiology department, the county referral center for both adult and pediatric populations. Diagnosis was confirmed at surgical excision (32), autoptic specimens (3) or by multimodal imaging when surgery was not indicated (7). The database of the county health system was interrogated to identify residents with The International Classification of Diseases-9 codes of PCT. Forty-two consecutive cases of PCTs were diagnosed from 1 January 1998, through 31 December 2011, among residents in Grosseto's county.

Results: Incidence rate of PCTs was 1.38 of 100,000 inhabitants per year. PCTs were benign in 38 patients (90.5%) and malignant in four (9.5%). Twenty myxomas were found (48%), followed by seven fibroelastomas (15%), six lipomas (15%), three rhabdomyomas (8%), two hemangioma (5%), two sarcomas (5%), one lymphoma (2%) and one pericardial hemangiopericytoma (2%). Incidence of benign PCT was 1.24 of 100,000/year; referring only to myxomas we found an incidence of 0.68 of 100,000/year.

Conclusion: This is the first population study on PCT, a rare disease with an incidence rate of 1.38 new cases per 100,000 residents per year.