title: Long-term follow-up of the "wait and see" approach to localized perinatal adrenal neuroblastoma.
abstract: BACKGROUND
  Evidence-based guidelines for the management of localized perinatal adrenal neuroblastoma are not yet available. We describe our preliminary experience managing this tumor with a "wait and see" policy.
  
  
  METHODS
  A single-center prospective study (February 2002 to December 2009) was conducted with 12 consecutive patients in whom an adrenal mass was detected antenatally or within the first 3 months of life. Diagnostic workup included the following investigations: measurement of urine catecholamine metabolites, imaging studies (ultrasonography, magnetic resonance imaging, or computed tomography), metaiodobenzylguanidine scintigraphy, and/or core needle biopsy.
  
  
  RESULTS
  The male/female ratio was 1.4:1.0. Median tumor size at presentation was 29 mm (range 10-50 mm). Eight lesions were detected antenatally. Ten lesions were diagnosed as localized neuroblastoma. Of these ten lesions, four were excised because of parental preference (n = 2), tumor enlargement (n = 1) or tumor persistence (n = 1). The remaining six patients underwent watchful clinical observation, which showed progressive tumor shrinkage and complete regression within 10-39 months (median 12.5 months). The final two lesions were small predominantly cystic lesions without a clear-cut diagnosis. They were managed noninvasively. At an overall median follow-up of 109 months (range 30-122 months), all patients are alive and disease-free, although one patient progressed to stage 4 disease despite early excision of the primary tumor.
  
  
  CONCLUSIONS
  Spontaneous regression of localized perinatal adrenal neuroblastoma occurs often, and a "wait and see" strategy seems justified in these small infants. Patients with enlarging or stable lesions that have persisted for several months may benefit from surgery, although prompt excision may not prevent tumor progression.
authors.affiliation: Pediatric Surgery Unit, Department of Pediatrics, Sapienza University of Rome, 324 Viale Regina Elena, 00161 Rome, Italy. da.cozzi@uniroma1.it
authors.forename: Denis Andrew
authors.initials: DA
authors.lastname: Cozzi
authors.forename: Ermelinda
authors.initials: E
authors.lastname: Mele
authors.forename: Silvia
authors.initials: S
authors.lastname: Ceccanti
authors.forename: Fabio
authors.initials: F
authors.lastname: Natale
authors.forename: Anna
authors.initials: A
authors.lastname: Clerico
authors.forename: Amalia
authors.initials: A
authors.lastname: Schiavetti
authors.forename: Carlo
authors.initials: C
authors.lastname: Dominici
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000151:congenital / Q000175:diagnosis / Q000628:therapy* / Q000652:urine; D015415:Biomarkers / Q000652:urine; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009365:Neoplasm Regression, Spontaneous; D009447:Neuroblastoma / Q000151:congenital / Q000175:diagnosis / Q000628:therapy* / Q000652:urine; D011247:Pregnancy; D011446:Prospective Studies; D014057:Tomography, X-Ray Computed; D016216:Ultrasonography, Prenatal; D057832:Watchful Waiting
chemical_list: D015415:Biomarkers