The value of pre-operative embolisation in primary inferior vena cava paraganglioma

Area of Science:

• Vascular Surgery
• Pediatric Oncology
• Radiology

Background:

• Paragangliomas are rare neuroendocrine tumors that can arise from various locations, including the inferior vena cava (IVC).
• Primary IVC paragangliomas are exceptionally rare, posing diagnostic and therapeutic challenges, particularly in pediatric patients.

Observation:

• A 13-year-old boy presented with progressive abdominal distension and anemia, indicative of an underlying serious condition.
• Radiological imaging identified a hypervascular tumor within the IVC, necessitating urgent intervention.
• The patient experienced an acute lower gastrointestinal bleed post-investigation, highlighting the tumor's aggressive nature.

Findings:

• Histopathological examination confirmed a chromogranin-positive paraganglioma originating from the IVC.
• The tumor was successfully managed through a combination of pre-operative embolization to reduce vascularity and subsequent surgical resection.
• Surgical intervention included tumor resection, right nephrectomy, and partial duodenal resection, with the patient remaining stable post-operatively.

Implications:

• This case underscores the importance of considering rare tumors like IVC paraganglioma in pediatric patients with unexplained abdominal symptoms.
• Pre-operative embolization is a valuable strategy for managing hypervascular IVC tumors, facilitating safer surgical resection.
• Highlighting the radiological features and successful multidisciplinary management of this rare entity contributes to the literature and informs clinical practice.