Bilateral Adrenal Neuroblastoma is Different

F. Pederiva, A. Andres, A. Sastre, J. Alves, L. Martinez, Juan A. Tovar

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background/Aims: Bilateral adrenal neuroblastoma is rare and can be due to multifocal primary or contralateral metastasis. Staging is confusing in these patients and treatment guidelines are difficult to set. The present study examines the clinical, biological and therapeutic features of bilateral adrenal neuroblastoma. Methods: We identified 4 cases primarily located in both adrenals out of 148 neuroblastomas treated between 1992 and 2006. We studied the clinicopathological findings and biological features, including MYCN amplification, and analyzed the treatment strategies and results. Results: All patients were younger than 6 months of age and all had multiple liver metastases. Three had subcutaneous nodules and massive liver enlargement. All underwent chemotherapy prior to operation. Two babies had large bilateral tumors without preservable glands and underwent bilateral adrenalectomy. Both had MYCN gene amplification and died of widespread (brain and bone) metastases some weeks later. In the remaining two patients adrenalectomy was performed on the side of the larger tumor with tumor enucleation on the other side to preserve hormonal function followed by 2 courses of mild chemotherapy in one patient. These tumors were not amplified. Both of these children are doing well. Conclusions: Bilateral adrenal neuroblastomas fit neither into stage 4 s nor into stage 4. Their clinical behavior is exceptional with a number of multicystic forms, variable MYCN amplification, widespread metastases and a high mortality. Bilateral adrenalectomy is sometimes unavoidable, but unilateral removal with contralateral enucleation, partial resection or observation are valid alternatives. Mortality is higher than in regular stage 4 s cases. This particular group of neuroblastomas required individually tailored therapeutic strategies based on the size, extent and prognostic markers.

Original languageEnglish
Pages (from-to)393-396
Number of pages4
JournalEuropean Journal of Pediatric Surgery
Volume17
Issue number6
DOIs
Publication statusPublished - Dec 2007

Fingerprint

Neuroblastoma
Adrenalectomy
Neoplasm Metastasis
Neoplasms
Drug Therapy
Mortality
Gene Amplification
Liver
Therapeutics
Observation
Guidelines
Bone and Bones
Brain

Keywords

  • Adrenal
  • Bilateral
  • Metastases
  • MYCN amplification
  • Neuroblastoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Pederiva, F., Andres, A., Sastre, A., Alves, J., Martinez, L., & Tovar, J. A. (2007). Bilateral Adrenal Neuroblastoma is Different. European Journal of Pediatric Surgery, 17(6), 393-396. https://doi.org/10.1055/s-2007-965811

Bilateral Adrenal Neuroblastoma is Different. / Pederiva, F.; Andres, A.; Sastre, A.; Alves, J.; Martinez, L.; Tovar, Juan A.

In: European Journal of Pediatric Surgery, Vol. 17, No. 6, 12.2007, p. 393-396.

Research output: Contribution to journalArticle

Pederiva, F, Andres, A, Sastre, A, Alves, J, Martinez, L & Tovar, JA 2007, 'Bilateral Adrenal Neuroblastoma is Different', European Journal of Pediatric Surgery, vol. 17, no. 6, pp. 393-396. https://doi.org/10.1055/s-2007-965811
Pederiva, F. ; Andres, A. ; Sastre, A. ; Alves, J. ; Martinez, L. ; Tovar, Juan A. / Bilateral Adrenal Neuroblastoma is Different. In: European Journal of Pediatric Surgery. 2007 ; Vol. 17, No. 6. pp. 393-396.
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