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Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis
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 p...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Thieme Medical Publishers
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4078144/ https://www.ncbi.nlm.nih.gov/pubmed/25032061 http://dx.doi.org/10.1055/s-0034-1371751 |
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author | Singh, Paul Jodicke, Cristiano Swanson, Tara Maulik, Dev |
author_facet | Singh, Paul Jodicke, Cristiano Swanson, Tara Maulik, Dev |
author_sort | Singh, Paul |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4078144 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Thieme Medical Publishers |
record_format | MEDLINE/PubMed |
spelling | pubmed-40781442014-07-16 Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis Singh, Paul Jodicke, Cristiano Swanson, Tara Maulik, Dev AJP Rep Article 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. Thieme Medical Publishers 2014-04-15 2014-05 /pmc/articles/PMC4078144/ /pubmed/25032061 http://dx.doi.org/10.1055/s-0034-1371751 Text en © Thieme Medical Publishers |
spellingShingle | Article Singh, Paul Jodicke, Cristiano Swanson, Tara Maulik, Dev Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title | Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title_full | Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title_fullStr | Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title_full_unstemmed | Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title_short | Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis |
title_sort | thoracic ganglioneuromas resulting in nonimmune hydrops fetalis |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4078144/ https://www.ncbi.nlm.nih.gov/pubmed/25032061 http://dx.doi.org/10.1055/s-0034-1371751 |
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