Pederiva F, Andres A, Sastre A, Alves J, Martinez L, Tovar J A
Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de la Castellana 261, Madrid, Spain.
Eur J Pediatr Surg. 2007 Dec;17(6):393-6. doi: 10.1055/s-2007-965811.
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.
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.
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.
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.
背景/目的：双侧肾上腺神经母细胞瘤较为罕见，可能是多灶性原发或对侧转移所致。这些患者的分期存在混淆，难以制定治疗指南。本研究探讨双侧肾上腺神经母细胞瘤的临床、生物学和治疗特征。
我们在1992年至2006年期间治疗的148例神经母细胞瘤中，确定了4例主要位于双侧肾上腺的病例。我们研究了临床病理结果和生物学特征，包括MYCN扩增，并分析了治疗策略和结果。
所有患者年龄均小于6个月，均有多发肝转移。3例有皮下结节和肝脏肿大。所有患者术前均接受化疗。2例婴儿双侧肿瘤巨大，腺体无法保留，接受了双侧肾上腺切除术。两者均有MYCN基因扩增，数周后死于广泛（脑和骨）转移。其余2例患者在较大肿瘤一侧行肾上腺切除术，另一侧行肿瘤剜除术以保留激素功能，其中1例患者随后接受了2个疗程的轻度化疗。这些肿瘤未扩增。这两名儿童情况良好。
双侧肾上腺神经母细胞瘤既不符合4s期也不符合4期。它们的临床行为特殊，有多种多囊性形式、MYCN扩增情况不一，并伴有广泛转移和高死亡率。双侧肾上腺切除术有时不可避免，但单侧切除加对侧剜除、部分切除或观察也是有效的选择。死亡率高于常规4s期病例。这一特殊类型的神经母细胞瘤需要根据肿瘤大小、范围和预后标志物制定个体化的治疗策略。
