title: Atypical cardiac myxoma in the course of Carney complex in the child. Case Report.
abstract: Cardiac tumors in infants and children are rare. Myxomas are the second (after rhabdomyomas) most common primary cardiac tumors in pediatric patients. Cardiac, cutaneous and mucous myxomas are likewise the second most frequent manifestation of the Carney complex, an autosomal dominant multi neoplasia syndrome, which consists of myxomas in different locations, spotty skin pigmentation and endocrine overactivity. We present a case of 13-years-old boy send to our department from a district hospital because of the large tumor in the right atrium discovered in the echo study. On admission he presented discrete signs of Cushing's syndrome and scarse pigmented nevi on the face and trunc. The detailed echo examination showed the large right atrial tumor with features of myxoma, protruding across the tricuspid valve into the right ventricle during diastolic period. Atypical location of cardiac myxoma as well as the signs of Cushing's syndrome suggested Carney's complex. Detailed endocrine studies confirmed the hypothesis. Thus two-step bilateral adrenalectomy was planned. The histopathologic study confirmed primary pigmented nodular adrenocortical disease.
authors: Department of Pediatric Cardiology, Polish-American Institute of Pediatrics (PAIP), Jagiellonian University, Medical College, Krakow, Poland.
authors: Piotr
authors: P
authors: Weryński
authors: Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, PAIP, Jagiellonian University Medical College, Krakow, Poland.
authors: Dominika
authors: D
authors: Januś
authors: Department of Pediatric Cardiosurgery, PAIP, Jagiellonian University, Medical College, Krakow, Poland.
authors: Mirosława
authors: M
authors: Dudyńska
authors: Department of Clinical Immunology and Transplantation, Polish-American Institute of Paediatrics, Jagiellonian University, Medical College, Cracow, Poland.
authors: Grażyna
authors: G
authors: Drabik
authors: Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, PAIP, Jagiellonian University Medical College, Krakow, Poland.
authors: Jerzy B
authors: JB
authors: Starzyk
authors: Department of Pediatric Cardiosurgery, PAIP, Jagiellonian University, Medical College, Krakow, Poland.
authors: Janusz
authors: J
authors: Skalski
authors: Department of Pediatric Cardiology, Polish-American Institute of Pediatrics (PAIP), Jagiellonian University, Medical College, Krakow, Poland.
authors: Andrzej
authors: A
authors: Rudziński
mesh_terms: D000293:Adolescent; D056733:Carney Complex / Q000175:diagnosis*; D003480:Cushing Syndrome / Q000175:diagnosis*; D006801:Humans; D008297:Male