Gastroschisis in a fetus with a congenital neuroblastoma: Association or coincidence?
| Autor: | Valentina Oliveira Provenzi, Laura Michelon, Átila Masiero, Vanessa F. De Souza, Adyr Eduardo Virmond Faria, Paulo Ricardo Gazzola Zen, Rafael Fabiano Machado Rosa, Luciano Vieira Targa, Cristine Dietrich, Silvana Rodrigues Streit Pires |
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| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Embryology Fetus Pathology medicine.medical_specialty 030219 obstetrics & reproductive medicine Gastroschisis business.industry Abdominal wall defect Adrenal Gland Neoplasm General Medicine 030105 genetics & heredity medicine.disease Umbilical cord Abdominal wall 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure Antifibrinolytic agent Neuroblastoma Pediatrics Perinatology and Child Health medicine business Developmental Biology |
| Zdroj: | Birth Defects Research Part A: Clinical and Molecular Teratology. 106:208-212 |
| ISSN: | 1542-0752 |
| Popis: | Background Gastroschisis is the most common abdominal wall defect. It is characterized by herniation of the intestine and other abdominal organs through a defect in the abdominal wall. Neuroblastoma is the most common malignant tumor observed during the neonatal period. It is a neuroendocrine tumor derived from neural crest cells that develops into the adrenal gland. Case We report on the undescribed association between gastrochisis and congenital neuroblastoma, diagnosised during the prenatal period. The mother was a 20-year-old healthy pregnant woman in her second pregnancy. Obstetric ultrasound examination showed a fetus presenting an abdominal wall defect on the right side of the umbilical cord, compatible with gastroschisis, and a hyperechogenic and spherical solid lesion on the left adrenal gland. Fetal magnetic resonance imaging disclosed similar features associated to a heterogeneous aspect of the liver. The diagnosis of metastatic neuroblastoma was confirmed after birth through liver biopsy. At 2 days of life, the prothrombrin time was abnormal, and the patient needed vitamin K. Conclusion We cannot rule out the possibility that a clotting defect, commonly observed in disseminated malignancies such as a metastatic neuroblastoma may be associated with the etiology of the gastroschisis, as this defect may result from a thrombosis occurring around 3 to 4 weeks of gestation, a period when neuroblasts development occurs into the adrenal medulla. However, we cannot exclude the possibility that both events may have occurred simultaneously by chance. Birth Defects Research (Part A) 106:208–212, 2016. © 2016 Wiley Periodicals, Inc. |
| Databáze: | OpenAIRE |
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