engleski

Intracardial Juvenile Xanthogranuloma in a Newborn

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG is usually shown in form of the cutaneous lessions. Visceral involment is rare but it can occur in several locations. Deep JXG differs from the cutaneous form histologically by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of the JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG can rarely occur only in a inter or intramuscular location. Its presentation in heart is so far described in only two cases, but they had typical skin lesions. We present an unusual case of JXG arising intracavitary without systemic disease or metabolic abnormalities. A newborn had inconspicuos early neonatal period. At the age of 10 days systolic murmur grade 2/6 over aortic valve with radiation towards the right clavicle, was noted. Routine laboratory findings, as well as, thoracic radiogram were normal. Electrocardiogram showed sinus rhythm, hyperdeviation of the electrical axis towards the right (+170o) and right ventricular hyperthrophy associated with minor repolarisation defects. Echocardiographic exam revealed normal segmental cardiac morphology, cavities and wall thickness. Further echocardiographic evaluation showed a spherical structure 9 mm in diameter connected to the septum by a short peduncle, below the aortic valve, obstructing the left ventricular outflow tract, as well as, bulging spherical pedunculated tumor mass 7 mm in diameter on the anterior of the right ventricle, and few smaller welldefined, spherical, intramural structures at the right ventricular apex and in apical region of the ventricular septum slightly shifted towards the left ventricle. Due to obstructive nature of tumors operation was performed, and tumor masses in right and left ventricle were excided. Histologic findings were a surprise, and hence were checked with a number of other pathologists who finally approved of the initial diagnosis. Diagnosis was also confirmed by immunomarkers on histiocytes. Immunohistochemistry was performed with the avidin-biotin-peroxidase (ABC) technique (Daco, Denmark). Immunohistochemical staining of histiocytes for CD 68 was positive. Postoperative recovery was normal. In further clinical follow-up (6 years) we have not find any abnormalities or tumor relapse.

intracardial juvenile xanthogranuloma; newborn

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