pirarubicin and Ganglioneuroblastoma

The present study sought to reduce the incidence of treatment complications of low-risk neuroblastoma by using image-defined risk factors (IDRFs) to inform the timing of surgical resection.. Eligible patients included children (<18 years of age) with stage 1 or 2 disease, children (<365 days of age) with stage 3 disease, and infants with stage 4S disease. In IDRF-negative cases, treatment was completed with surgical resection alone. In IDRF-positive cases, the timing of surgery was determined based on the IDRFs after low-dose chemotherapy with 2-3 of the following four drugs: vincristine, cyclophosphamide, pirarubicin, and carboplatin. The outcome measures were overall survival, progression-free survival, and adverse events. This study was registered with the UMIN Clinical Trials Registry (number 000004355).. Of the 60 patients screened between 2010 and 2013, 58 eligible patients were enrolled; 32 were identified as IDRF negative at diagnosis while 26 were identified as IDRF positive and underwent induction chemotherapy. The 3-year overall and progression-free survival rates of the 58 patients were 100% and 82.8% (95% confidence interval: 70.3-90.3), respectively. Neutropenia was the most frequently reported grade 3 or 4 chemotherapy-related form of toxicity (41.7%). With regard to surgical complications, 2.5% of all patients developed pleural effusion and ascites as early complications, while only 2.5% developed renal atrophy as a long-term complication. No fatal toxicities were observed.. Using IDRFs to inform surgical decision making for the treatment of low-risk neuroblastoma improved prognosis and reduced the incidence of long-term complications.

Topics: Adolescent; Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Chemotherapy-Induced Febrile Neutropenia; Child; Child, Preschool; Clinical Decision-Making; Combined Modality Therapy; Cyclophosphamide; Doxorubicin; Female; Ganglioneuroblastoma; Humans; Infant; Infant, Newborn; Japan; Kaplan-Meier Estimate; Magnetic Resonance Imaging; Male; Neuroblastoma; Pleural Effusion; Postoperative Complications; Progression-Free Survival; Prospective Studies; Risk Factors; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed; Vascular Diseases; Vincristine

Obstructive jaundice is a rare symptom in the neuroblastic tumor. Seven cases of obstructive jaundice caused by neuroblastoma have been reported, and only three of these patients had the symptom at the onset of the disease. The authors report a case of ganglioneuroblastoma presenting with obstructive jaundice as a rare initial feature. After the histologic diagnosis with open biopsy, chemotherapy consisting of cisplatin, pirarubicin hydrochloride/doxorubicin, cyclophosphamide, and vincristine was given. The treatment resulted in reduction in the tumor size and relief of the obstructive jaundice. Complete resection of the tumor was possible after five courses of chemotherapy.

Topics: Antineoplastic Combined Chemotherapy Protocols; Biopsy; Child, Preschool; Cisplatin; Cyclophosphamide; Doxorubicin; Ganglioneuroblastoma; Humans; Jaundice, Obstructive; Male; Retroperitoneal Neoplasms; Vincristine