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World Journal of Surgery

, Volume 37, Issue 2, pp 459–465 | Cite as

Long-term Follow-up of the “Wait and See” Approach to Localized Perinatal Adrenal Neuroblastoma

  • Denis Andrew CozziEmail author
  • Ermelinda Mele
  • Silvia Ceccanti
  • Fabio Natale
  • Anna Clerico
  • Amalia Schiavetti
  • Carlo Dominici
Article

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.

Keywords

Neuroblastoma Adrenal Mass Spontaneous Regression Adrenal Hemorrhage MYCN Amplification 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgment

The authors are very much obliged to Prof. Francesco Cozzi of Rome, Italy, for critical reading of the manuscript.

Conflict of interest

This work had no specific funding. All of the authors state that there are no direct or indirect commercial financial incentives associated with publishing the present work.

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Copyright information

© Société Internationale de Chirurgie 2012

Authors and Affiliations

  • Denis Andrew Cozzi
    • 1
    Email author
  • Ermelinda Mele
    • 1
  • Silvia Ceccanti
    • 1
  • Fabio Natale
    • 2
  • Anna Clerico
    • 3
  • Amalia Schiavetti
    • 3
  • Carlo Dominici
    • 3
    • 4
  1. 1.Pediatric Surgery Unit, Department of PediatricsSapienza University of RomeRomeItaly
  2. 2.Neonatal Intensive Care Unit, Department of PediatricsSapienza University of RomeRomeItaly
  3. 3.Pediatric Oncology Unit, Department of PediatricsSapienza University of RomeRomeItaly
  4. 4.School of Reproductive and Developmental MedicineLiverpool UniversityLiverpoolUK

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