Clinical characteristics and risk factors of 47 cases with ruptured neuroblastoma in children

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 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 highrisk 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.

Hemorrhage During Induction Chemotherapy in Neuroblastoma: Additional Risk Factors in High-Risk Patients

“…Hemorrhage is an uncommon, life-threatening event in patients affected by neuroblastoma (7,12,13). In a recent paper, Qin et al reported 47 neuroblastoma patients with hemorrhage, either secondary to spontaneous tumor rupture or after chemotherapy or biopsy, on a total population of ∼1,800 patients, with an incidence of approximately 2.6% and poor outcome; treatment was withdrawn in 17 of these patients, while other 5 patients died as an immediate consequence of this complication (9). The mechanism underlying spontaneous hemorrhage in neuroblastoma has been debated; in neonates, an adrenal mass could be crushed between the liver and the spine during delivery, causing tumor rupture and subsequent hemorrhage (14), while in older children the presence of neuroblastoma could predispose to adrenal hemorrhage following minor trauma (15).…”

“…On rare instances, children with neuroblastoma may present with acute hemorrhage such as hemothorax and hemoperitoneum (7)(8)(9). Such cases pose a great challenge for the caring clinicians.…”

Endovascular approaches in pediatric interventional oncology

“…Similar to Wilm's tumor, presurgical embolization can be performed in large neuroblastomas to reduce intraoperative bleeding at resection, treat bleeding after biopsy and shrink the tumor to decrease intraabdomial pressure and improve respiratory status (Krauel et al 2009;Kyo Jin et al 2018;Pio et al 2017). A maximum primary tumor diameter > 13.20 cm and MYCN gene amplification were two independent risk factors for high-risk NB tumor rupture (Qin et al 2020).…”

Rapid Unperturbed‐Tissue Analysis for Intraoperative Cancer Diagnosis Using an Enzyme‐Activated NIR‐II Nanoprobe

“…Children neuroblastoma (NB), the most common extracranial solid tumor in childhood, accounting for 8–10 % of all childhood malignancies, is mainly treated on surgery [29–33] . However, it remains challenging to make a quick decision on the malignancy and benignancy of the tissues during surgical operation.…”

“…[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] Children neuroblastoma (NB), the most common extracranial solid tumor in childhood, accounting for 8-10 % of all childhood malignancies, is mainly treated on surgery. [29][30][31][32][33] However, it remains challenging to make a quick decision on the malignancy and benignancy of the tissues during surgical operation. MMP14, a cell-membrane-anchoring enzyme, has been reported to be overexpressed especially in neuroblastoma of children.…”