Clinical characteristics and risk factors of 47 cases with ruptured neuroblastoma in children
Hong Qin,
Shen Yang,
Siyu Cai,
Qinghua Ren,
Wei Han,
Wei Yang,
Haiyan Cheng,
Xiaoli Ma,
Huanmin Wang
Affiliations
Hong Qin
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Shen Yang
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Siyu Cai
Center for Clinical Epidemiology & Evidence-Based Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Qinghua Ren
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Wei Han
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Wei Yang
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Haiyan Cheng
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Xiaoli Ma
Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Huanmin Wang
Department of Surgical Oncology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health
Abstract Background Neuroblastoma (NB) tumor rupture is a rare oncology emergency with a poor prognosis. We aimed to evaluate patient clinical characteristics and risk factors for ruptured NB. Methods A retrospective study of 47 patients with confirmed NB rupture between January 2009 and January 2019 at Beijing Children’s Hospital was conducted. To identify tumor rupture risk factors in high-risk NB patients, we included 93 consecutive non-ruptured high-risk NB patients from January 2017 to January 2019. Results The median age at presentation was 29 months (adrenal and retroperitoneum origin) for 47 ruptured NB patients. Spontaneous tumor rupture occurred in 22 cases; 18 cases occurred during or after the first chemotherapy cycle, and 7 occurred after core needle biopsy. Five patients died of tumor rupture, and 17 patients’ parents refused further antitumor therapy. Among the 25 remaining patients, 6 survived without disease, 5 received ongoing treatment and achieved stable disease, and 14 died. According to multivariate logistic regression analysis, a maximum primary tumor diameter > 13.20 cm and MYCN gene amplification were independent risk factors for tumor rupture within high-risk NB. Conclusions Tumor rupture is an uncommon, life-threatening event for NB patients; these patients are most likely to have poor outcomes due to tumor recurrence or rapid progression. Several treatment modalities, including symptomatic support therapy and chemotherapy, are important for saving lives and for developing NB risk-based treatment in the future.
