PEDIATRICS Vol. 97 No. 2 February 1996, pp. 258-261
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Neuroblastoma and Congenital Cardiovascular Malformations

LUCA ROSTI MD1, ANGELA E. LIN MD2, and ALESSANDRO FRIGIOLA MD3

1 Department of Pediatrics, Ospedale Regina Elena, Milan, Department of Pediatric Cardiology/Cardiac Surgery, Ospedale San Donato Milanese, Milan, Italy
2 Franciscan Children's Hospital, Department of Pediatrics, Boston University School of Medicine, Boston, MA
3 Department of Surgery, Ospedale San Donato Milanese, Milan, Italy

Congenital cardiovascular malformations (CCVM) are frequently associated with noncardiac malformations, often comprising a multiple malformation syndrome, but occurring infrequently in nonsyndromic systemic disease states such as neuroblastoma. As the most common malignancy in the first year of life, neuroblastoma presents as both clinically apparent and clinically unsuspected in situ tumors detected at autopsy. Neuroblastoma's association with CCVMs, especially conotruncal defects, suggests a possible common embryologic role for abnormal neural crest cell migration and development. We provide further documentation of this intriguing, albeit rare, association by reporting a boy with a prenatally detected complex CCVM (univentricular heart and dextro-transposition of the great arteries [d-TGA]) and congenital neuroblastoma.

Submitted on August 9, 1994
Accepted on March 6, 1995




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