Excellent treatment outcomes in children younger than 18 months with stage 4 MYCN nonamplified neuroblastoma
- Author:
Chiwoo KIM
1
;
Young Bae CHOI
;
Ji Won LEE
;
Keon Hee YOO
;
Ki Woong SUNG
;
Hong Hoe KOO
Author Information
- Publication Type:Original Article
- Keywords: Neuroblastoma; Infant; Neoplasm metastasis; N-myc proto-oncogene protein
- MeSH: Abdomen; Bone Marrow; Carboplatin; Child; Cisplatin; Consensus; Cyclophosphamide; Doxorubicin; Drug Therapy; Etoposide; Follow-Up Studies; Humans; Ice; Ifosfamide; Infant; Isotretinoin; Liver; Lymph Nodes; Neoplasm Metastasis; Neuroblastoma; Prognosis; Radiotherapy; Recurrence; Retrospective Studies; Skin
- From:Korean Journal of Pediatrics 2018;61(2):53-58
- CountryRepublic of Korea
- Language:English
- Abstract: PURPOSE: Although the prognosis is generally good in patients with intermediate-risk neuroblastoma, no consensus has been reached on the ideal treatment regimen. This study analyzed treatment outcomes and toxicities in patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma. METHODS: We retrospectively analyzed 20 patients younger than 18 months newly diagnosed with stage 4 MYCN nonamplified neuroblastoma between January 2009 and December 2015. Patients received 9 cycles of chemotherapy and surgery, with or without local radiotherapy, followed by 12 cycles of differentiation therapy with 13-cis-retinoic acid. Chemotherapy consisted of alternating cycles of cisplatin, etoposide, doxorubicin, and cyclophosphamide (CEDC) and ifosfamide, carboplatin, and etoposide (ICE) regimens. RESULTS: The most common primary tumor site was the abdomen (85%), and the most common metastatic sites were the lymph nodes (65%), followed by the bones (60%), liver (55%), skin (45%), and bone marrow (25%). At the end of induction therapy, 14 patients (70%) achieved complete response, with 1 achieving very good partial response, 4 achieving partial response, and 1 showing mixed response. Nine patients (45%) received local radiotherapy. At a median follow-up of 47 months (range, 17–91 months), none of these patients experienced relapse, progression, or secondary malignancy, or died. Three years after chemotherapy completion, none of the patients had experienced grade ≥3 late adverse effects. CONCLUSION: Patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma showed excellent outcomes, without significant late adverse effects, when treated with alternating cycles of CEDC and ICE, followed by surgery and differentiation therapy.
