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
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).
