Surgical Management of Pediatric Neuroblastoma: A Complex Case Study

Introduction: Navigating Pediatric Oncological Challenges

Neuroblastoma remains one of the most common extracranial solid tumors in children, accounting for approximately 8-10% of all childhood cancers. A recent case at Burjeel Medical City highlights the complex surgical management required for these challenging cases, particularly when the tumor presents with unfavorable histology. Dr. Rajasekhar Cingapagu, Pediatric Surgeon at Burjeel Medical City, shares insights from this complex case that illustrates the importance of multidisciplinary collaboration and surgical expertise in pediatric oncology.

Case Presentation: From Initial Symptoms to Diagnosis

A 2-year-old boy presented to the Emergency Department at Burjeel Medical City with complaints of constipation, generalized abdominal pain, and fever. Physical examination revealed a concerning finding: an abdominal mass on the right side.

The diagnostic journey began with an abdominal ultrasound that showed a soft tissue hyperechoic lesion in the right suprarenal region with internal vascularity and areas of cystic changes. The lesion was closely abutting the right lobe of the liver and the superior pole of the right kidney, though the exact organ of origin could not be definitively determined from the ultrasound alone.

Given these findings, the patient was referred to the Pediatric Oncology department, and a Pediatric Surgical consultation was sought to develop a comprehensive management plan.

Advanced Imaging and Preoperative Assessment

A CT scan provided crucial additional information, revealing:

• A large round to oval heterogeneous soft-tissue density mass with solid and cystic components
• Infrequent areas of calcification arising from the mid and upper pole of the right kidney
• Approximate dimensions of 11 × 12 × 13 cm
• Patchy irregular enhancement on post-contrast imaging
• Significant mass effect, with the tumor pushing the aorta and inferior vena cava (IVC) leftward

The radiological assessment raised concerns about potential vascular involvement, particularly noting:

• A severely compressed IVC at the portal region
• Unclear visualization of a segment of the IVC, raising questions about possible thrombus
• Compression of the right renal vein by the mass, though normal color flow was preserved

Multidisciplinary Approach to Treatment Planning

The case was presented at a multidisciplinary team (MDT) meeting to determine the optimal management strategy. After careful review of the imaging studies, the team concluded that the tumor was resectable since the critical vascular structures—specifically the IVC and right renal vein—were compressed but not infiltrated by the tumor.

This collaborative decision-making led to a treatment plan involving surgical resection followed by chemotherapy as needed, based on the final pathological assessment.

The Surgical Challenge: Right Radical Nephrectomy

Following comprehensive preoperative preparation, including complete blood investigations, blood reservation, and detailed discussion with the parents, the surgical team proceeded with a right radical nephrectomy using a right transverse abdominal incision.

The intraoperative findings revealed a challenging surgical scenario:

• A large tumor measuring 16 cm, with firm consistency and areas of necrosis
• A tumor nodule at the renal hilum separate from the main tumor
• Multiple enlarged lymph nodes (infra-renal and paraaortic) measuring over 2 cm
• The tumor firmly adherent to the IVC, splaying the vessel just below the hepatic hilum
• Extension of the tumor across the midline just above the renal hilum
• No evidence of tumor thrombus in the renal vein or IVC

Technical Aspects of the Procedure

The surgical approach required meticulous dissection and included several critical steps:

1. Careful dissection to ligate the left renal vein and renal artery
2. Dissection of the ureter down to its lower end, which was then transfix ligated and cut
3. Delicate release of adhesions between the tumor and liver surface at the hepatic hilum, performed without injuring the liver
4. Comprehensive dissection of multiple lymph nodes in the para-aortic and infra-renal regions
5. Placement of a drain in the sub-hepatic space
6. Layered closure of the abdominal wound

Postoperative Course and Pathological Findings

The patient made a remarkably smooth postoperative recovery and was discharged home on the 7th postoperative day. The histopathology report confirmed the diagnosis of neuroblastoma with unfavorable histology, providing essential information for planning subsequent oncological management.

Clinical Significance and Learning Points

This case highlights several important aspects of managing large pediatric renal tumors:

Diagnostic Challenges

• The initial presentation with constipation and abdominal pain is nonspecific and highlights the importance of thorough physical examination in pediatric patients
• The differential diagnosis for an abdominal mass in a child includes Wilms’ tumor, neuroblastoma, and other less common entities that require careful radiological and pathological characterization

Surgical Considerations

• The proximity of the tumor to major vascular structures necessitates meticulous preoperative planning and experienced surgical technique
• The value of a multidisciplinary approach cannot be overstated—input from pediatric oncology, pediatric surgery, radiology, and pathology is essential for optimal outcomes
• Preservation of vital structures while achieving complete tumor resection requires balancing oncological principles with functional preservation

Prognostic Implications

• Unfavorable histology in neuroblastoma is associated with a more aggressive disease course and may influence decisions regarding adjuvant therapy
• Complete surgical resection remains a cornerstone of treatment, even in challenging cases with unfavorable pathological features

Conclusion

This case from Burjeel Medical City exemplifies the complex decision-making and technical expertise required in the management of pediatric solid tumors. Through careful preoperative assessment, multidisciplinary collaboration, and skilled surgical technique, even large, complex tumors can be successfully resected, providing children with the best opportunity for long-term survival.

The successful management of this challenging case reflects the commitment to excellence in pediatric surgical oncology at Burjeel Medical City and highlights the importance of specialized centers capable of providing comprehensive care for children with complex oncological conditions.

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