Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis

• Main Authors: Paul Singh, Cristiano Jodicke, Tara Swanson, Dev Maulik
• Format: Article
• Language: English
• Published: Thieme Medical Publishers, Inc. 2014-04-01
• Series: American Journal of Perinatology Reports
• Subjects: ganglioneuroma; hydrops fetalis; thoracic; neuroblastoma
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1371751

Abstract Introduction Most often, ganglioneuromas affect older pediatric and adult patients. They are typically slow growing tumors that remain clinically silent until they become large enough to cause symptoms by compression of adjacent structures. Case We report a case of a 22-year-old Hispanic gravida 2 para 1 female patient who was found to have massive hydrops fetalis at 20 completed gestational weeks. Fetal echocardiography revealed a narrowed distal ductal arch and proximal descending aorta. Cesarean delivery was undertaken at 29 completed gestational weeks for refractory labor and nonreassuring fetal status. The neonate expired at 47 minutes of life despite aggressive resuscitation. At autopsy, multiple thoracic masses were found adjacent to a compressed proximal descending aorta. Histological and immunohistochemical analysis confirmed the diagnosis of a ganglioneuroma, a rare type of neural crest tumor. Discussion A variety of intrathoracic masses have previously been reported to cause hydrops fetalis including teratomas, fibrosarcomas, and lymphangiomas. To our knowledge, this case is the first description of hydrops fetalis caused by ganglioneuromas. We propose that multiple thoracic ganglioneuromas led to biventricular distal outflow tract obstruction and hydrops fetalis.