Neuroblastome surrénalien bilatéral et syndrome de Pepper : à propos de quatre observations

Abstract

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. Patients and Methods. – Our multicentric study analyzed 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 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. Conclusion. – Children with 4S neuroblastoma with bilateral adrenal tumors have a good prognosis. Treatment should be the less agressive as possible. The group with favorable prognostic parameters should have supportive care if spontaneous regression occurs. But we have to treat with chemiotherapy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, high MYC-N copy number).