# Bilateral adrenal neuroblastoma: peculiar pattern of a rare pediatric presentation

**Authors:** Mohamed Fawzy, Gehad Ahmed, Yasser Youssef, Naglaa Elkinaai, Amal Refaat, Mai Amr Elahmadawy, Fadwa Said, Salma Elmenawi

PMC · DOI: 10.1007/s12672-024-00966-6 · Discover. Oncology · 2024-04-12

## TL;DR

This study examines the clinical and biological features of a rare pediatric cancer called bilateral suprarenal neuroblastoma in Egyptian patients and finds that prognosis depends on factors like disease stage and MYCN amplification.

## Contribution

The study provides insights into the clinical and biological characteristics of bilateral suprarenal neuroblastoma in an Egyptian cohort, emphasizing prognostic factors and treatment approaches.

## Key findings

- BSN patients had a 3-year overall survival of 40.5% for high-risk and 83.9% for intermediate-risk patients.
- MYCN amplification was found in 10 out of 33 patients.
- Biological variables and disease extent were key prognostic determinants.

## Abstract

Bilateral suprarenal neuroblastoma (BSN) is a rare presentation. Few previously published literature showed BSN patients to have favorable pattern and prognosis. This study aim was to evaluate clinical and biological features in relation to outcome of Egyptian patients with BSN.

Included patients were diagnosed from 2007 to 2017, retrospectively. Tissue biopsy, imaging and bone marrow were evaluated at presentation. Clinical, demographic, biological variables and risk group were determined and analyzed in relation to overall (OS) and event-free-survival (EFS).

BSN patients (n = 33) represented 2% of hospital patients with neuroblastoma during the 10-year study period, 17 were males and 16 were females. Twenty-four patients (72.7%) were infants, and 9 patients (27.3%) were above 1 year of age (range: 1 month to 3 years). Metachronous disease was present in only one patient. Amplified MYCN was found in 10 patients. Initially, most patients (n = 25) had distant metastasis, 6 had stage 3 versus 2 stage 2. Fifteen were high risk (HR), 15 intermediate (IR), 1 low risk (LR) and 2 were undetermined due to inadequate tissue biopsy. Three-year OS for HR and IR patients were 40.5% and 83.9% versus 23.2% and 56.6% EFS; respectively.

BSN treatment is similar to unilateral disease. A more conservative surgical approach with adrenal tissue preservation on less extensive side should be considered. Biological variables and extent of disease are amongst the most important prognostic determinants. Future studies are warranted to further address the biologic profiling of BSN and highlight prognostic significance of size difference between both adrenal sides.

## Linked entities

- **Genes:** MYCN (MYCN proto-oncogene, bHLH transcription factor) [NCBI Gene 4613]
- **Diseases:** neuroblastoma (MONDO:0005072)

## Full-text entities

- **Genes:** MYCN (MYCN proto-oncogene, bHLH transcription factor) [NCBI Gene 4613] {aka FGLDS1, MODED, MPAPA, MYCNsORF, MYCNsPEP, N-myc}
- **Diseases:** unilateral disease (MESH:D046088), Metachronous disease (MESH:D016609), metastasis (MESH:D009362), BSN (MESH:D009447)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11014822/full.md

## References

25 references — full list in the complete paper: https://tomesphere.com/paper/PMC11014822/full.md

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Source: https://tomesphere.com/paper/PMC11014822