Kerdudo C, Corradini N, Michon J, Leverger G
Service d'hématologie-oncologie pédiatrique, hôpital d'enfants Armand-Trousseau, Assistance Publique-Hôpitaux de Paris, 26, avenue du Docteur-A-Netter, 75012 Paris, France.
Arch Pediatr. 2004 Dec;11(12):1450-6. doi: 10.1016/j.arcped.2004.09.020.
4S neuroblastoma with bilateral adrenal involvement is defined by small primitive tumors (stage 1 or 2) with disseminated disease restricted to the liver, skin, and/or bone marrow. Children are less than one year old. These tumors are rare and of multicentric origin.
Our multicentric study analyzed four children less than four months old at diagnosis.
All had a favourable histology, with normal MYC-N copy number, and one case had a diploid tumor. The four patients had first supportive care at the beginning, but three cases received chemotherapy because of progressive disease, with liver radiotherapy in two cases because of massive hepatomegaly; three cases had surgery (unilateral adrenal resection in two cases and bilateral in one case) and one had only a biopsy. Surgery was the only treatment in one case. One patient relapsed 17 months after initial treatment and was treated with intensive chemotherapy and stem cell rescue. The outcome is favorable for the four patients, without evidence of recurrent disease.
Children with 4S neuroblastoma with bilateral adrenal tumors have a good prognosis. Treatment should be the less aggressive as possible. The group with favorable prognostic parameters should have supportive care if spontaneous regression occurs. But we have to treat with chemotherapy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, high MYC-N copy number).
双侧肾上腺受累的4S期神经母细胞瘤定义为小的原始肿瘤（1期或2期），播散性疾病局限于肝脏、皮肤和/或骨髓。患儿年龄小于1岁。这些肿瘤罕见且多中心起源。
我们的多中心研究分析了4例诊断时年龄小于4个月的患儿。
所有患儿组织学表现良好，MYC-N拷贝数正常，1例为二倍体肿瘤。4例患儿起初均接受支持治疗，但3例因疾病进展接受了化疗，2例因肝脏肿大接受了肝脏放疗；3例接受了手术（2例行单侧肾上腺切除术，1例行双侧肾上腺切除术），1例仅接受了活检。1例患儿手术是唯一的治疗方式。1例患儿初始治疗17个月后复发，接受了强化化疗和干细胞救援。4例患儿预后良好，无疾病复发迹象。
双侧肾上腺肿瘤的4S期神经母细胞瘤患儿预后良好。治疗应尽可能采取温和方式。具有良好预后参数的患儿若出现自发消退，应接受支持治疗。但对于肝脏肿大的新生儿和具有一个或多个不良预后因素（不良组织学、高MYC-N拷贝数）的患儿，我们必须采用化疗进行治疗。
