4S neuroblastoma with bilateral adrenal involvement is defined by small primit ive tumors (stage 1 or 2) with disseminated disease restricted to the liver, ski n, and/or bone marrow. Children are less than one year ol...4S neuroblastoma with bilateral adrenal involvement is defined by small primit ive tumors (stage 1 or 2) with disseminated disease restricted to the liver, ski n, and/or bone marrow. Children are less than one year old. These tumors are rar e and of multicentric origin. Patients and Methods. -Our multicentric study ana lyzed four children less than four months old at diagnosis. Results. -All had a favourable histology, with normal MYC-N copy number, and one case had a diploi d tumor. The four patients had first supportive care at the beginning, but three cases received chemotherapy because of progressive disease, with liver radiothe rapy in two cases because of massive hepatomegaly ; three cases had surgery (uni lateral adrenal resection in two cases and bilateral in one case) and one had on ly 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 eviden ce of recurrent disease. Conclusion. -Children with 4S neuroblastoma with bilat eral adrenal tumors have a good prognosis. Treatment should be the less agressiv e as possible. The group with favorable prognostic parameters should have suppor tive care if spontaneous regression occurs. But we have to treat with chemiother apy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, highMYC-N copy number).展开更多
目的:神经母细胞瘤是新生儿最常见的一种肿瘤。本研究回顾了被确诊患局限性神经母细胞瘤的新生儿的临床特征、治疗和结局。研究对象和方法:分析了1990-1999年间法国18个小儿肿瘤中心按照1990年和1994年拟定的神经母细胞瘤(NBL)治疗方案...目的:神经母细胞瘤是新生儿最常见的一种肿瘤。本研究回顾了被确诊患局限性神经母细胞瘤的新生儿的临床特征、治疗和结局。研究对象和方法:分析了1990-1999年间法国18个小儿肿瘤中心按照1990年和1994年拟定的神经母细胞瘤(NBL)治疗方案进行救治的52例患者的数据。结果:患儿年龄的中位数是12 d (年龄范围0-28 d),其中14例(27%)在产前检查时确诊。肿瘤发生部位有40例在腹部(其中20例在肾上腺附近),8例位于胸部,3例在盆腔。展开更多
文摘4S neuroblastoma with bilateral adrenal involvement is defined by small primit ive tumors (stage 1 or 2) with disseminated disease restricted to the liver, ski n, and/or bone marrow. Children are less than one year old. These tumors are rar e and of multicentric origin. Patients and Methods. -Our multicentric study ana lyzed four children less than four months old at diagnosis. Results. -All had a favourable histology, with normal MYC-N copy number, and one case had a diploi d tumor. The four patients had first supportive care at the beginning, but three cases received chemotherapy because of progressive disease, with liver radiothe rapy in two cases because of massive hepatomegaly ; three cases had surgery (uni lateral adrenal resection in two cases and bilateral in one case) and one had on ly 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 eviden ce of recurrent disease. Conclusion. -Children with 4S neuroblastoma with bilat eral adrenal tumors have a good prognosis. Treatment should be the less agressiv e as possible. The group with favorable prognostic parameters should have suppor tive care if spontaneous regression occurs. But we have to treat with chemiother apy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, highMYC-N copy number).
文摘目的:神经母细胞瘤是新生儿最常见的一种肿瘤。本研究回顾了被确诊患局限性神经母细胞瘤的新生儿的临床特征、治疗和结局。研究对象和方法:分析了1990-1999年间法国18个小儿肿瘤中心按照1990年和1994年拟定的神经母细胞瘤(NBL)治疗方案进行救治的52例患者的数据。结果:患儿年龄的中位数是12 d (年龄范围0-28 d),其中14例(27%)在产前检查时确诊。肿瘤发生部位有40例在腹部(其中20例在肾上腺附近),8例位于胸部,3例在盆腔。
