Paper # 008

Versión en Español

Purkinje cell hamartoma (Histiocytoid cardiomyopathy). Study of a case in an 18 months infant.

Marcial Garcia-Rojo, Carlos Gamallo, Felipe Moreno

[Title] [Materials & Methods] [Results] [Pictures] [Discussion] [Bibliography]

INTRODUCTION

During pediatric age, myocardial alterations comprises a series of metabolic, infectious, congenital and acquired processes. Amongst these, Purkinje cell hamartoma (histiocytoid cardiomyopathy) appears.

The first description of the Purkinje cell hamartoma was made by Wegman y Egbert, in 1935, that they described as a congenital rhabdomyoma⁽¹⁾. The first ultrastructural study corresponds to Haese et al (1972), determining the characteristics of the lesion: myocardial cells with large amount of mitochondria and few myofibrils ⁽²⁾. Until now, about 70 patients with this disease have been published, with many different eponyms, such as cardiac lipidoses, myocardial foamy transformation, histiocytoid cardiomyopathy, oncocytic cardiomyopathy and myocardial hamartoma.

The histogenesis of the lesion remains obscure, and we review the interpretation made by different authors.

We present a histopathological study of a patient with special emphasis in the possible histogenesis of the process. We have also realized a complete literature review.

• Title
• Materials & Methods
• Results
• Discussion
• Pictures
• Bibliography