A Rare Case of Cardiac Mesenchymal Hamartoma and Comprehensive Review of the Literature With Emphasis on Histopathology

Abstract

Hamartomas are primary, benign neoplastic lesions that most commonly derive from a single variably differentiated cell lineage. Here, we report an unusual case of a cardiac hamartoma. A 62-year-old woman presented with chest pain and palpitations. Serial imaging revealed a large slowly growing and highly vascularized left ventricular mass, which required surgical resection. Microscopically, the lesion was composed of nodular fibrovascular proliferation with haphazardly embedded muscle bundles and peripheral calcifications. Immunohistochemical studies revealed prominent muscle-specific actin positive and smooth muscle actin positive muscle fiber bundles within a disorganized fibrovascular stroma. This characterization is most consistent with cardiac mesenchymal hamartoma. Relevant differential diagnoses for this lesion include hamartoma of mature cardiac myocytes (HMCMs) and intramuscular hemangioma. The prominent smooth muscle differentiation of muscle bundles was incompatible with defining features of HMCM. Absence of S100-positive nerve and mature adipose cells distinguished this lesion from the recently defined, heterogeneous cardiac mesenchymal hamartoma. Forty-seven cases of cardiac hamartoma reported from 1970 to 2020 were reviewed to provide histopathologic context.

Hamartoma of mature cardiomyocytes presenting with atypical angina, 18F-fluorodeoxyglucose positron emission tomography uptake, and myocardial bridging: a case report

“…Hamartoma is broadly defined as a benign abnormal polyclonal proliferation of cells native to the affected organ. Hamartomatous lesions that are composed of mature muscle fibre bundles that occur in cardiac tissue have been called hamartoma of mature cardiac myocytes (HMCMs); HMCMs are rare tumours; only 47 cases have been described to date in both paediatrics and adults, 1 and its diagnostic workup is a challenging scenario. The multidisciplinary approach has been the key to the final diagnostic findings and treatment option strategy.…”