Abstract
INTRODUCTION: Tumor rupture with neuroblastoma is an uncommon but serious complication, particularly in high-risk cases involving MYCN amplification. When rupture occurs soon after induction chemotherapy is initiated, rapid deterioration and abdominal compartment syndrome (ACS) may develop. Early identification of high-risk patients and their readiness for surgical management are essential to optimizing outcomes. CASE PRESENTATION: A 4-year-old girl presented with a large left adrenal mass and elevated neuroblastoma markers. Imaging showed a heterogeneous 11 × 9.5 × 17-cm tumor encasing the renal hilum. Because of intratumoral hemorrhage, biopsy was deferred and induction chemotherapy was initiated. Seven days later, sudden abdominal distension and severe anemia developed. Contrast-enhanced CT confirmed intraperitoneal bleeding from the ruptured tumor. Transarterial embolization, including occlusion of the left renal artery, achieved temporary hemostasis; however, intra-abdominal pressure increased to 20 mmHg, thus meeting the ACS criteria. Emergency laparotomy revealed extensive hemorrhagic ascites and a ruptured tumor capsule. En bloc resection of the tumor and left nephrectomy were performed over 4 hours, and total blood loss of 2968 mL occurred. Histopathology confirmed MYCN-amplified neuroblastoma invading the adjacent renal parenchyma, which was classified as high risk by the International Neuroblastoma Risk Group. Postoperatively, the chylous ascites resolved by day 11, and multimodal therapy, including chemotherapy, autologous stem cell transplantation, proton beam radiotherapy, and anti-GD2 antibody therapy, was completed. Remission has been maintained for 2 years. CONCLUSIONS: Embolization alone may not prevent ACS in patients with MYCN-amplified neuroblastoma and chemotherapy-induced rupture. Prompt surgical resection can be life-saving when anatomically feasible. A pretreatment risk assessment, cautious initiation of chemotherapy, vigilant monitoring, and early surgical preparedness are critical for managing high-risk neuroblastoma.