Delayed rupture of the celiac trunk and superior mesenteric artery associated with pancreatic leakage after resection of a giant retroperitoneal ganglioneuroblastoma in a child: a case report

Introduction

Neurogenic tumors commonly arise in the neck, mediastinum, adrenal region, or retroperitoneum (1). In children, giant retroperitoneal lesions frequently encase major abdominal vessels—including the aorta, celiac trunk, and superior mesenteric artery (SMA)—and may involve adjacent organs such as the pancreas and biliary system. Although surgical resection remains the treatment of choice, complete excision is technically demanding and is associated with substantial perioperative risk when critical vascular or pancreatic structures are involved (1,2).

Postoperative pancreatic leakage after resection of neurogenic tumors is rare but may lead to severe complications when major vessels are exposed. Activated pancreatic enzymes can erode adjacent arterial walls, resulting in pseudoaneurysm formation and rupture. While this mechanism is well documented in adult pancreatitis (3), it is seldomly reported in pediatric oncology. Here, we present a child with a giant retroperitoneal ganglioneuroblastoma who developed pancreatic leakage-associated rupture of both the celiac trunk and SMA, necessitating emergent vascular reconstruction. This case underscores the need for heightened vigilance and prompt vascular assessment in patients with postoperative pancreatic leakage. We present this article in accordance with the CARE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-2026-1-0101/rc).

Case presentation

A previously healthy 9-year-old boy presented with nausea and vomiting. Physical examination revealed a firm, non-tender abdominal mass occupying the upper abdomen, with limited mobility. The abdomen was soft, without guarding or rebound tenderness. Abdominal magnetic resonance imaging revealed a giant retroperitoneal mass measuring 19.8 cm × 17.0 cm × 25.0 cm, which displaced the inferior vena cava (IVC), encased the celiac trunk and SMA, and closely abutted the pancreatic body (Figure 1A). Tumor markers, including neuron-specific enolase (NSE), and urinary vanillylmandelic acid (VMA), were within normal limits. An image-guided core needle biopsy suggested ganglioneuroma. Despite the benign biopsy result, surgery was chosen due to the tumor’s large size, major vessel encasement, and risk of missing intermixed malignant components. Final histopathology confirmed intermixed ganglioneuroblastoma. All procedures performed in this study were in accordance with the Helsinki Declaration and its subsequent amendments. Ethical approval was obtained from the Ethics Committee of Baoding Hospital of Beijing Children’s Hospital (No. 2025-54). Written informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Figure 1 Radiologic and intraoperative findings of the patient. (A) Preoperative magnetic resonance imaging demonstrates a giant retroperitoneal mass. The wide arrow indicates the abdominal aorta, the narrow red arrow indicates the pancreas, and the narrow blue arrow indicates the SMA. (B) Computed tomography angiography shows a pseudoaneurysm arising from the superior mesenteric artery (narrow red arrow); the narrow blue arrow indicates the SMA. (C) Catheter-based angiography demonstrates focal arterial wall irregularity and luminal roughening of the superior mesenteric artery (narrow blue arrow), consistent with pseudoaneurysm formation. (D) Intraoperative photograph showing active bleeding at the origin of the celiac trunk (narrow blue arrow). (E) Intraoperative photograph demonstrating the rupture site of the superior mesenteric artery (narrow blue arrow). (F) Intraoperative photograph showing reconstruction of the superior mesenteric artery using an autologous great saphenous vein interposition graft (narrow blue arrow).

Exploratory laparotomy revealed a large retroperitoneal tumor causing marked displacement of the IVC and significant traction on the hepatic hilum and duodenum, with pronounced thinning of the bile duct wall. The tumor completely encased the celiac trunk and SMA and was densely adherent to the pancreatic capsule. Complete macroscopic tumor resection was achieved.

Postoperatively, the patient exhibited persistent elevation of drain fluid and serum amylase levels. The drain fluid amylase exceeded three times the serum level, meeting the diagnostic criteria for pancreatic leakage. The patient was managed with somatostatin therapy and adequate external drainage. On postoperative day (POD) 11, bile-stained drainage persisted, indicating a bile leak, and was managed conservatively. The initial bile drainage volume was 260 mL, and the drainage pattern remained stable in subsequent days without a significant increase. Serum bilirubin levels remained within normal limits. The patient did not develop severe postoperative infection.

On POD 18, the patient suddenly developed massive hemorrhagic drainage, hematemesis, hematochezia, and rapid hemoglobin decline. Emergency upper gastrointestinal endoscopy revealed no active mucosal bleeding. Computed tomography angiography (CTA) (Figure 1B) and catheter angiography (Figure 1C) demonstrated a pseudoaneurysm arising from the SMA.

Emergency laparotomy revealed an extensive intraperitoneal hematoma. After evacuation of the hematoma and exposure of the retroperitoneal space, rupture of both the celiac trunk (Figure 1D) and SMA (Figure 1E) was confirmed. Proximal control of the abdominal aorta was obtained. Intraoperative assessment revealed well-developed collateral circulation to the liver and stomach via pancreaticoduodenal arcades, allowing safe celiac trunk ligation without hepatic artery reimplantation. SMA continuity was restored using a 15-cm autologous great saphenous vein graft to minimize infection risk and ensure long-term patency (Figure 1F). Intraoperative assessment demonstrated severe biliary fragility, precluding primary repair. Therefore, the common bile duct was transected, cholecystectomy was performed, and Roux-en-Y biliary-enteric reconstruction was completed. Abdominal drains were repositioned to ensure adequate postoperative drainage.

Under multidisciplinary management, the patient improved gradually. Drain fluid amylase levels decreased, and abdominal drains were sequentially removed. By POD 36, the patient tolerated a regular diet without gastrointestinal symptoms. During the early postoperative period, liver enzymes showed only mild transient elevation and gradually normalized with hepatoprotective therapy. Follow-up imaging demonstrated normal hepatic and splenic perfusion (Figure 2). At the latest follow-up, 12 months after surgery, liver function tests, including bilirubin and cholestatic markers, remained within normal ranges. No imaging or clinical evidence of biliary fibrosis or cirrhosis was observed. Additionally, no tumor recurrence or major complications occurred during the follow-up period.

Figure 2 Postoperative contrast-enhanced CT demonstrating the reconstructed SMA with a great saphenous vein bypass graft (arrow). CT, computed tomography; SMA, superior mesenteric artery.

Discussion

The management of tumors initially diagnosed as ganglioneuroma on biopsy remains controversial. Observation may be considered for small and asymptomatic lesions (4). However, biopsy sampling may fail to capture malignant components in large heterogeneous tumors. In the present case, the extremely large tumor size, extensive encasement of major abdominal vessels, and the potential risk of sampling error strongly favored surgical resection. The final pathological diagnosis of intermixed ganglioneuroblastoma further validated this treatment strategy.

Giant retroperitoneal neurogenic tumors pose substantial surgical challenges due to their close relationship with major abdominal vessels and adjacent pancreatic structures. Complete resection often requires extensive vascular skeletonization, which increases the risk of postoperative pancreatic injury and subsequent leakage. Although pancreatic leakage is uncommon after neurogenic tumor resection, its clinical consequences can be devastating when major vessels are exposed to activated pancreatic enzymes (2,5).

In the present case, intraoperative pancreatic capsule injury was observed during tumor dissection. Postoperatively, the diagnosis of pancreatic leakage was established based on persistently elevated drain fluid amylase levels exceeding three times the serum concentration. Despite adequate external drainage and somatostatin therapy, ongoing enzymatic exposure likely resulted in progressive local tissue damage. The subsequent bile leak, characterized by persistent bile-stained drainage with normal serum bilirubin levels, suggested localized biliary tract injury without systemic cholestasis. Although histopathological confirmation of arterial wall injury was unavailable, the close temporal relationship between pancreatic leakage and delayed arterial rupture supports a possible association between enzymatic injury and vascular wall destruction.

The mechanism of pancreatic leakage-associated vascular injury is well recognized in adult patients with severe pancreatitis. Activated pancreatic enzymes can induce proteolytic degradation of the arterial media, predisposing to pseudoaneurysm formation and rupture (3). Experimental studies further suggest that the admixture of pancreatic juice with intestinal fluid may amplify protease activation and exacerbate vascular damage (6). However, such catastrophic vascular events are exceedingly rare in pediatric oncology, and awareness among pediatric surgeons remains limited.

Clinically, the abrupt onset of gastrointestinal bleeding accompanied by massive hemorrhagic drainage represented a critical warning sign of vascular catastrophe. Conventional endoscopy may fail to identify the bleeding source in such scenarios, potentially delaying definitive diagnosis. In contrast, CTA and catheter-based angiography are essential for rapidly distinguishing mucosal bleeding from arterial rupture and should be promptly performed when unexplained hemorrhage occurs in patients with pancreatic leakage.

Although endovascular techniques have been reported to be effective in hemorrhage secondary to pancreatic leakage (7,8), their applicability in pediatric patients is limited. In this case, small vessel caliber, ongoing enzymatic contamination, and the need for durable mesenteric perfusion rendered endovascular intervention less suitable. Open surgical exploration allowed definitive control of bleeding, optimization of drainage, and durable vascular reconstruction. Intraoperative evaluation confirmed well-developed collateral circulation to the liver and stomach via the pancreaticoduodenal arcades, allowing safe ligation of the celiac trunk without hepatic artery reimplantation. The SMA was reconstructed using a 15-cm autologous great saphenous vein graft rather than a prosthetic conduit to reduce the risk of infection and ensure long-term patency. This approach provided reliable restoration of mesenteric blood flow and minimized postoperative complications.

During follow-up, liver function remained stable, and no evidence of biliary fibrosis or cirrhosis was observed, suggesting that hepatic perfusion was adequately maintained through collateral circulation after celiac trunk ligation. The favorable outcome also highlights the feasibility of autologous great saphenous vein grafting for reconstruction of the SMA in contaminated surgical fields associated with pancreatic leakage. Careful long-term surveillance remains essential to evaluate graft patency and hepatic perfusion in pediatric patients undergoing complex visceral arterial reconstruction.

This case highlights an underrecognized but potentially fatal postoperative complication in children undergoing resection of giant retroperitoneal tumors. Early recognition of warning signs and timely vascular imaging are essential to prevent catastrophic outcomes. This report is limited by its single-case nature and the absence of histopathological confirmation of arterial wall injury; therefore, a causal relationship between pancreatic leakage and arterial rupture cannot be definitively established. Nevertheless, the clinical course strongly suggests a clinically meaningful association that warrants heightened vigilance and further investigation.

Conclusions

Pancreatic leakage after resection of giant retroperitoneal neuroblastic tumors may be associated with delayed arterial rupture, particularly when major vessels are extensively skeletonized. Sudden massive hemorrhagic drainage or gastrointestinal bleeding in this setting should prompt immediate vascular imaging and timely surgical intervention to prevent fatal outcomes.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-2026-1-0101/rc

Peer Review File: Available at https://tp.amegroups.com/article/view/10.21037/tp-2026-1-0101/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-2026-1-0101/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the Helsinki Declaration and its subsequent amendments. Ethical approval was obtained from the Ethics Committee of Baoding Hospital of Beijing Children’s Hospital (No. 2025-54). Written informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

1. Braungart S, Losty PD. Diagnosis and management of neuroblastoma in children. World J Pediatr Surg 2026;9:e001127. [Crossref] [PubMed]
2. Yoneda A. Role of surgery in neuroblastoma. Pediatr Surg Int 2023;39:177. [Crossref] [PubMed]
3. Evans RP, Mourad MM, Pall G, et al. Pancreatitis: Preventing catastrophic haemorrhage. World J Gastroenterol 2017;23:5460-8. [Crossref] [PubMed]
4. Sánchez-Galán A, Barrena S, Vilanova-Sánchez A, et al. Ganglioneuroma: to operate or not to operate. Eur J Pediatr Surg 2014;24:25-30. [Crossref] [PubMed]
5. He M, Cai JB, Wu X, et al. Perioperative complication incidence and risk factors for retroperitoneal neuroblastoma in children: analysis of 571 patients. World J Pediatr 2024;20:250-8. [Crossref] [PubMed]
6. Seki Y, Ishizawa T, Mitsuhashi K, et al. Mechanisms of histopathologic vascular damage caused by pancreatic juice leakage: Implications for preventing hemorrhagic complications. Surgery 2025;186:109591. [Crossref] [PubMed]
7. Kapranov MS, Kiselev AD, Brukh SL, et al. Possibilities of Endovascular Hemostasis in Treatment of Pancreatic Bleeding. Arch Razi Inst 2022;77:375-81. [PubMed]
8. Watanabe Y, Nakazawa K, Takase K, et al. Outcomes of Arterial Embolization vs Covered Stents for Delayed Massive Hemorrhage After Pancreatic or Biliary Surgery. J Gastrointest Surg 2022;26:1187-97. [Crossref] [PubMed]