Rare Atypical Presentation of a Neuroblastoma of Posterior Mediastinum

• Main Authors: Vishal Singh, Lamkordor Tyngkan, Abdul Rashid Bhat
• Format: Article
• Language: English
• Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2021-05-01
• Series: International Journal of Recent Surgical and Medical Sciences
• Subjects: neuroblastoma; paraplegia; atypical presentation; rare
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1730243

Introduction Neuroblastoma is the third most common malignancy and one of the most common neurogenic tumors of posterior mediastinum in the pediatric age group. A posterior mediastinal tumor causing spinal cord compression is an important example of an oncologic emergency arising from a neurogenic tumor. We present a rare case of posterior mediastinum neuroblastoma, with exclusive lower limb weakness presenting at an unusually older age. Case Presentation A 6-year-old girl reported with upper back ache and sudden onset progressive bilateral lower limb weakness, with difficulty in urination for 10 days. The weakness had progressed to paraplegia on the day of presentation. On examination, the child was observed to have loss of tone and power in both lower limbs and bilateral extensor plantar reflex. Chest X-ray showed heterogeneous opacity involving most of the left lung. Contrast-enhanced MRI showed posterior mediastinal tumor, extending into spinal canal. Patient underwent D3 to D6 laminectomy with subtotal tumor excision and spinal decompression. Crush biopsy showed round cell tumor, and final histopathological examination revealed poorly differentiated neuroblastoma, which was strongly positive for neuron-specific enolase on immunohistochemistry. The surgery was followed by chemotherapy, and the patient remained symptom free on 2-year follow-up. Discussion The presenting symptoms in case of thoracic neuroblastoma may include airway obstruction and chronic cough. Sometimes, it may present with weakness, limping, paralysis, and bladder and bowel disturbances in case of spinal cord involvement. Posterior mediastinal neuroblastoma presenting with paraparesis is uncommon. In our case, the child presented with isolated lower limb flaccidity with no other classical symptoms. To the best of our knowledge and thorough study, only four cases of posterior mediastinum neuroblastoma presenting with lower limb paralysis has been published in the English literature to date. Conclusion Tumor usually presents with pressure symptoms, caused by the mass in the posterior mediastinum, but it’s almost exclusive occurrence in the pediatric population warrants a thorough investigation in patients presenting with not only a symptomatic mass but also exclusive lower limb neurological symptoms.