title: Gastroschisis in a fetus with a congenital neuroblastoma: Association or coincidence?
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Clinical Genetics, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA) and Complexo Hospitalar Santa Casa de Porto Alegre (CHSCPA), RS, Brazil.|Clinical Genetics, Hospital Materno Infantil Presidente Vargas (HMIPV), RS, Brazil.|Graduate Program in Pathology, UFCSPA, RS, Brazil.|Fetal Medicine, HMIPV, RS, Brazil.
authors.forename: Rafael F M
authors.initials: RF
authors.lastname: Rosa
authors.affiliation: Graduation in Medicine, Universidade Luterana do Brasil (ULBRA), RS, Brazil.
authors.forename: Laura
authors.initials: L
authors.lastname: Michelon
authors.affiliation: Graduation in Medicine, Universidade Luterana do Brasil (ULBRA), RS, Brazil.
authors.forename: Átila
authors.initials: Á
authors.lastname: Masiero
authors.affiliation: Pediatric Surgery, HMIPV, RS, Brazil.
authors.forename: Adyr E V
authors.initials: AE
authors.lastname: Faria
authors.affiliation: Fetal Medicine, HMIPV, RS, Brazil.
authors.forename: Vanessa F
authors.initials: VF
authors.lastname: De Souza
authors.affiliation: Fetal Medicine, HMIPV, RS, Brazil.
authors.forename: Cristine
authors.initials: C
authors.lastname: Dietrich
authors.affiliation: Pediatric Radiology, HMIPV, RS, Brazil.
authors.forename: Luciano V
authors.initials: LV
authors.lastname: Targa
authors.affiliation: Pathology, Grupo Hospitalar Conceição (GHC), RS, Brazil.
authors.forename: Valentina O
authors.initials: VO
authors.lastname: Provenzi
authors.affiliation: Neonatology, HMIPV, RS, Brazil.
authors.forename: Silvana R S
authors.initials: SR
authors.lastname: Pires
authors.affiliation: Clinical Genetics, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA) and Complexo Hospitalar Santa Casa de Porto Alegre (CHSCPA), RS, Brazil.|Graduate Program in Pathology, UFCSPA, RS, Brazil.
authors.forename: Paulo R G
authors.initials: PR
authors.lastname: Zen
mesh_terms: D034861:Abdominal Wall / Q000002:abnormalities / Q000601:surgery; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D005260:Female; D005333:Fetus; D020139:Gastroschisis / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000556:secondary; D009447:Neuroblastoma / Q000175:diagnosis* / Q000556:secondary / Q000601:surgery; D011247:Pregnancy; D013927:Thrombosis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D016216:Ultrasonography, Prenatal; D014812:Vitamin K / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000933:Antifibrinolytic Agents; D014812:Vitamin K
keywords: coagulation; congenital metastatic neuroblastoma; gastroschisis