Subscribe to RSS
Rare Atypical Presentation of a Neuroblastoma of Posterior Mediastinum
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.
12 May 2021 (online)
© 2021. Medical and Surgical Update Society. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
- 1 Robert MK. Nelson Textbook of Pediatrics. 20th ed Elsevier 2015. 2461.
- 2 McLatchie GR, Young DG. Presenting features of thoracic neuroblastoma. Arch Dis Child 1980; 55 (12) 958-962
- 3 Yon DK, Ahn TK, Shin DE, Kim GI, Kim MK. Extragonadal germ cell tumor of the posterior mediastinum in a child complicated with spinal cord compression: a case report. BMC Pediatr 2018; 18 (01) 97
- 4 Lacayo NJ, Davis KL. Pediatric neuroblastoma. Medscape 2015. Accessed July 9, 2015 at http://emedicine.medscape.com/article/988284.
- 5 Shohet JM, Nuchtern JG. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Accessed March 9, 2017 at: http://www.uptodate.com/contents/epidemiology-pathogenesis-and-pathology-of-neuroblastoma. Last updated: August 19, 2016
- 6 Chauhan G, Bhatia P, Bhardwaj AK, Mittal S, Sharma PD. Widely disseminated neuroblastoma presenting as acute flaccid paralysis in a two year old child: a clinical dilemma. Int J Contemporary Med 2014; 2: 161-163
- 7 Yahya FS, Al-Shami HA. Posterior mediastinal neuroblastoma masked as flaccid paraparesis in a 3 year child. Neurosciences (Riyadh) 2019; 24 (04) 320-323
- 8 Castleberry RP. Neuroblastoma. Eur J Cancer 1997; 33 (09) 1430-1437, discussion 1437–1438
- 9 Rajparath R, Rangesh S, Senthamarai MV. Posterior mediastinal neuroblastoma in an infant presenting with paraparesis: a case report. Pediatric Review: Int J Pediatr Res 2016; 9: 715-717
- 10 Brodeur GM, Pritchard J, Berthold F. et al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J Clin Oncol 1993; 11 (08) 1466-1477
- 11 Latchaw RE, L’Heureux PR, Young G, Priest JR. Neuroblastoma presenting as central nervous system disease. AJNR Am J Neuroradiol 1982; 3 (06) 623-630