Multimodal Management of an Aggressive Vertebral Hemangioma in a Child with Neurological Deficit: A Case Report with 5-Year Follow-Up

Introduction

Vertebral hemangiomas (AVH) are common benign vascular lesions (10–12%) ^[1] accounting for 2–3% of all spinal tumors ^[2], and are often asymptomatic and incidentally detected. However, a small subset, termed aggressive AVH, exhibits locally invasive behavior characterized by cortical destruction, epidural extension, and neurological compromise ^[1,3]. These lesions account for approximately 1% of all AVH and may present with progressive myelopathy or radiculopathy ^[4].

Management remains controversial, and treatment options include radiotherapy, embolization, vertebroplasty, and surgical decompression and resection, often combined ^[5,6,7]. Pediatric cases are rare, and optimal strategies have not been well established. We present a case of aggressive vertebral hemangioma in a 12-year-old girl with progressive neurological deterioration, successfully managed with a multimodal approach and long-term follow-up.

Case Report

A 12-year-old girl presented with right-side dominant lower limb sensory loss. One month later, she developed progressive bilateral lower limb weakness, numbness, and gait imbalance. She also reported night pains; there were no bowel or bladder complaints.

Neurological examination revealed a high-stepping, ataxic gait. Motor power was 3/5 at the hips and knees and 2/5 at the ankles (bilateral). Sensory examination revealed reduced sensation in the bilateral L4, L5, and S1 dermatomes. Deep tendon reflexes were brisk, with bilateral ankle and patellar clonus and extensor plantar responses (Nurick grade-4). Based on the biopsy findings and clinical progression, the lesion was characterized as an aggressive vertebral hemangioma (Enneking stage 3) with evolving conus compression. The patient underwent radiological investigations. (Fig. 1a and b) Anteroposterior and lateral thoracolumbar radiographs demonstrated a D12 vertebral body lesion with coarse trabeculations, (Fig. 1c and d) Pre-operative computed tomography showed Corduroy and Polka dot appearances in sagittal and axial sections. (Fig. 2a, b, c, d,e,f) Pre-operative magnetic resonance imaging demonstrated an aggressive D12 vertebral hemangioma with vertebral body involvement and epidural extension, causing significant spinal cord compression.

Discussion

AVH are rare entities with the potential for significant neurological compromise. Management of these tumors requires careful planning because of their high vascularity and risk of intraoperative bleeding ^[2,5]. Pre-operative embolization plays a critical role in reducing intraoperative blood loss and facilitating safe resection ^[7,8]. Surgical decompression remains the mainstay treatment for patients with neurological deficits, with or without stabilization, depending on structural involvement ^[4]. Vertebroplasty provides the additional benefits of mechanical stabilization and tumor devascularization ^[9] and also significantly reduces the need for radical surgical resection by achieving local tumor control ^[10]. Main indication for surgical stabilization in our case is to maintain spinal stability and to prevent post-operative deformity in patient with extensive pan vertebral involvement, requiring extensive decompression, rather than solely the lesion being a stable aggressive vertebral hemangioma ^[2,6]. Adjuvant radiotherapy has been shown to reduce recurrence rates and improve local control, particularly in subtotal resections ^[6,10]. Post-operative radiotherapy (40 Gy) with in the therapeutic range, was mainly indicated here because of locally aggressive nature of the lesion and anticipated residual intraosseous remnants even following extensive decompression to achieve complete disease free environment ^[3,9]. A multimodal approach combining embolization, surgery, and radiotherapy is increasingly advocated in the literature and is associated with favorable outcomes ^[10]. Pediatric aggressive vertebral hemangioma is extremely rare and this case adds to the limited available literature demonstrating successful long-term outcomes and that a carefully planned multimodal approach may achieve satisfactory disease control while avoiding the morbidity associated with radical surgical excision in aggressive vertebral hemangioma. Recurrence should not be considered inevitable but residual disease foci and incomplete treatment strategy can result in late recurrence ^[2]. Our Patient with 5 year asymptomatic and disease free follow-up is the result of implementing appropriate treatment strategy in such exceptionally rare presentation of Pediatric aggressive vertebral hemangioma.

Conclusion

AVH in pediatric patients can cause rapid neurological deterioration. Early diagnosis and a multimodal treatment strategy involving pre-operative embolization, surgical decompression, vertebral stabilization, and adjuvant radiotherapy can result in excellent neurological recovery and long-term disease control without the need for morbidly aggressive resection.

Clinical Message

Pediatric aggressive vertebral hemangiomas are exceptionally uncommon, and the literature has only a few cases reported. They are prone for causing rapid neurological deterioration, necessitates need for a multidisciplinary multimodal strategy incorporating embolization, vertebroplasty, decompression, stabilization, and post-operative adjuvant radiotherapy, avoiding the morbidity associated with en bloc vertebral resection and can achieve excellent neurological recovery and durable long-term disease control without radical resection.

References

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About the Authors

• 

  Guganesh Palanisamy

• 

  Abhay Ghorpade

• 

  Swapnil Kumar

• 

  Saikumar Waghmore

• 

  Ashutosh Sabnis Balkrishna