title: Spinal adrenal cortical adenoma associated with Beckwith-Wiedemann syndrome: case report and review of the literature.
abstract: OBJECTIVE
  Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. To date, only eight spinal cases have been reported. This is the third reported case in pediatric population, the first one associated with genetic syndrome and the first benign to recur. We review the current literature on this topic.
  
  
  METHODS
  We present a 2-year-old boy affected by Beckwith-Wiedemann syndrome who developed a tumor at L4-L5 level. He underwent a gross total resection with MRI post-surgery demonstrating non-residual tumor. Histology disclosed an ectopic adrenal cortical adenoma with oncocytic features. Immunohistochemically was positive for inhibin-alpha, synaptophysin, and melan-A. It was negative for chromogranin A, GFAP, S-100, and other markers. One year later, he developed a recurrence at the same level being necessary a second surgery leaving a small sheet of residual tumor.
  
  
  CONCLUSIONS
  Spinal adrenocortical adenomas are exceptional, and its behavior could be related to other conditions such as BWS. Gross total resection can be curative but a tight follow-up is needed. Immunohistochemical studies that include inhibin-alpha, synaptophysin, and melan-A can be useful in differential diagnosis as ultrastructural study. The decision on how to treat these patients is difficult given the low number of cases.
authors.affiliation: Department of Pediatric Neurosurgery, University Hospital La Paz, Paseo de La Castellana 261, 28043, Madrid, Spain. javier.giner.garcia@gmail.com.
authors.forename: Javier
authors.initials: J
authors.lastname: Giner
authors.affiliation: Department of Pathology, University Hospital La Paz, Madrid, Spain.
authors.forename: Isabel
authors.initials: I
authors.lastname: Esteban
authors.affiliation: Department of Pediatric Neurosurgery, University Hospital La Paz, Paseo de La Castellana 261, 28043, Madrid, Spain.
authors.forename: Fernando
authors.initials: F
authors.lastname: Carceller
authors.affiliation: Department of Pediatric Neurosurgery, University Hospital La Paz, Paseo de La Castellana 261, 28043, Madrid, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Saceda
authors.affiliation: Department of Pathology, University Hospital La Paz, Madrid, Spain.
authors.forename: R M
authors.initials: RM
authors.lastname: Regojo
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D018246:Adrenocortical Adenoma / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D002675:Child, Preschool; D006801:Humans; D008297:Male; D013125:Spinal Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery
keywords: Adrenal cortical adenoma; Beckwith-Wiedemann syndrome; Inhibin-alpha; Melan-A; Synaptophysin