Embolization of a hemorrhaging abdominal plexiform neurofibroma

Area of Science:

• Oncology
• Vascular Surgery
• Genetics

Background:

• Neurofibromas, particularly plexiform neurofibromas, are benign tumors that can arise from nerve sheath cells.
• Plexiform neurofibromas are associated with neurofibromatosis type 1 (NF1) in about 50% of cases.
• These tumors possess invasive and angiogenic properties, predisposing them to complications like hemorrhage.

Observation:

• A 21-year-old woman presented with a rapidly growing abdominal mass, later identified as a cutaneous neurofibroma.
• The patient experienced progressive anemia and hemodynamic instability due to the mass.
• Initial embolization of a pseudoaneurysm and the right internal mammary artery did not fully resolve the bleeding.

Findings:

• A second embolization targeting the inferior epigastric artery was performed.
• Surgical intervention included mass resection, hematoma evacuation, and flap reconstruction.
• Pathology confirmed the diagnosis of plexiform neurofibroma, consistent with the suspected etiology of spontaneous bleeding.

Implications:

• This case underscores the potential for significant, spontaneous hemorrhage from cutaneous neurofibromas.
• Effective management requires a multidisciplinary approach, often involving interventional radiology and surgery.
• Understanding the angiogenic nature of neurofibroma Schwann cells is crucial for managing such rare but serious complications.