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Abstract：
BACKGROUND: 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.
METHODS: 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).
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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).
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
背景：双侧肾上神经母细胞瘤（BSN）是一种罕见的表现。先前发表的文献很少显示BSN患者具有良好的模式和预后。这项研究的目的是评估与BSN埃及患者预后相关的临床和生物学特征。
方法：纳入的患者于2007年至2017年进行了回顾性诊断。组织活检，影像学和骨髓在演示时进行评估.临床,人口统计学,我们确定了生物学变量和风险组,并分析了它们与总体生存(OS)和无事件生存(EFS)的关系.
结果：在10年的研究期间，BSN患者（n=33）占医院神经母细胞瘤患者的2％，男性17人，女性16人。24例（72.7%）为婴儿，9例（27.3%）患者年龄在1岁以上（范围：1个月至3岁）。仅在一名患者中存在异时性疾病。在10例患者中发现扩增的MYCN。最初，大多数患者（n=25）有远处转移，6的阶段为3，而2的阶段为2。15人是高风险（HR），15中间体（IR），1个低风险（LR）和2个由于组织活检不充分而未确定。HR和IR患者的三年OS分别为40.5％和83.9％，而EFS分别为23.2％和56.6％。
结论：BSN治疗与单侧疾病相似。应考虑在较不广泛的一侧保留肾上腺组织的更保守的手术方法。生物学变量和疾病程度是最重要的预后决定因素之一。未来的研究有必要进一步解决BSN的生物学特征，并强调两个肾上腺侧之间大小差异的预后意义。
方法：纳入的患者于2007年至2017年进行了回顾性诊断。组织活检，影像学和骨髓在演示时进行评估.临床,人口统计学,我们确定了生物学变量和风险组,并分析了它们与总体生存(OS)和无事件生存(EFS)的关系.
结果：在10年的研究期间，BSN患者（n=33）占医院神经母细胞瘤患者的2％，男性17人，女性16人。24例（72.7%）为婴儿，9例（27.3%）患者年龄在1岁以上（范围：1个月至3岁）。仅在一名患者中存在异时性疾病。在10例患者中发现扩增的MYCN。最初，大多数患者（n=25）有远处转移，6的阶段为3，而2的阶段为2。15人是高风险（HR），15中间体（IR），1个低风险（LR）和2个由于组织活检不充分而未确定。HR和IR患者的三年OS分别为40.5％和83.9％，而EFS分别为23.2％和56.6％。
结论：BSN治疗与单侧疾病相似。应考虑在较不广泛的一侧保留肾上腺组织的更保守的手术方法。生物学变量和疾病程度是最重要的预后决定因素之一。未来的研究有必要进一步解决BSN的生物学特征，并强调两个肾上腺侧之间大小差异的预后意义。
