Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Mohit Kukreja^1, , V G Telang² and Chetan Anchan³

¹Orthopedic Research Fellow, Desert Orthopedic Centre, Las Vegas, NV, USA

²Dhanavantari Hospital & Research Centre, Mumbai

³Bombay Hospital & Research Centre, Mumbai

Cite this paper

Mohit Kukreja, V G Telang and Chetan Anchan. Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report. American Journal of Medical Case Reports. 2017; 5(8):221-225. doi: 10.12691/AJMCR-5-8-5

Abstract

Introduction: The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor). The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases.EWS is the second most common primary bone malignancy in childhood. In contrast to long bone involvement, delays in spinal EWS diagnosis may occur because symptoms may not be present until neurological deficits occur. To date, although there have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Case Presentation: A 14 year old boy comes with a 12 weeks h/o urinary incontinence and constipation. Over the next 4 months, the patient had new-onset intermittent abdominal pain with worsening urinary symptoms.MRI confirmed a diagnosis of Koch's spine. However, the histopathology reported it as an “Ewings sarcoma/Primitive Neuroectodermal Tumor”. Discussion: Ewing’s tumor of sacrum is rare, but should be suspected in low backache in children. Cauda equina syndrome can be a valid presentation for EWS spine. MRI can identify cases early and enables early treatment though it is not specific. Histopathological diagnosis is a must before any definitive management.EWS spine can mimick Tuberculosis both clinico-radiologically, only to be confirmed with a tissue biopsy. Conclusion: Ewing’s sarcoma of the lumbosacral spine can have an atypical presentation and there should be a high degree of suspicion to diagnose it early.

Keywords

Ewing’s, cauda-equina, lumbosacral

Copyright

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References

[1]	J. A. Ellis, R. J. Rothrock, G. Moise et al., “Primitive neuroectodermal tumors of the spine: a comprehensive review with illustrative clinical cases,” Neurosurgical Focus, vol. 30, no. 1, pp. 1-13, 2011.
[2]	L. Venkateswaran, C. Rodriguez-Galindo, T. E. Merchant, C. A. Poquette, B. N. Rao, and A. S. Pappo, “Primary Ewing tumor of the vertebrae: clinical characteristics, prognostic factors, and outcome,” Medical and Pediatric Oncology, vol. 37, no. 1, pp. 30-35, 2001.
[3]	C. Rodriguez-Galindo, S. L. Spunt, and A. S. Pappo, “Treatment of ewing sarcoma family of tumors: current status and outlook for the future,” Medical and Pediatric Oncology, vol. 40, no. 5, pp. 276-287, 2003.
[4]	K. Fizazi, N. Dohollou, J. Y. Blay et al., “Ewing’s family of tumors in adults: multivariate analysis of survival and longterm results of multimodality therapy in 182 patients,” Journal of Clinical Oncology, vol. 16, no. 12, pp. 3736-3743, 1998.
[5]	S. J. Cotterill, S. Ahrens, M. Paulussen et al., “Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma StudyGroup,” Journal of Clinical Oncology, vol. 18, no. 17, pp. 3108-3114, 2000.
[6]	G. Bacci, S. Ferrari, F. Bertoni et al., “Prognostic factors in nonmetastatic Ewing’s sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Istituto Ortopedico Rizzoli,” Journal of Clinical Oncology, vol. 18, no. 1, pp. 4-11, 2000.
[7]	H. Ilaslan, M. Sundaram, K. K. Unni, and M. B. Dekutoski, “Primary Ewing’s sarcoma of the vertebral column,” Skeletal Radiology, vol. 33, no. 9, pp. 506-513, 2004.
[8]	A. A. Gupta, A. Pappo, N. Saunders et al., “Clinical outcome of children and adults with localized ewing sarcoma: impact of chemotherapy dose and timing of local therapy,” Cancer, vol.116, no. 13, pp. 3189-3194, 2010.
[9]	G. Gatta, J. M. Van Der Zwan, P. G. Casali et al., “Rare cancers are not so rare: the rare cancer burden in Europe,” European Journal of Cancer, vol. 47, no. 17, pp. 2493-2511, 2011.
[10]	K.R. Weiss, D.J. Biau, and R. Bhumbra, “Axial skeletal location predicts poor outcome in Ewing,” Sarcoma, vol. 2011, Article ID 395180, 5 pages, 2011.
[11]	H. W. Hense, S. Ahrens, M. Paulussen, M. Lehnert, and H. Jürgens, “Factors associated with tumor volume and primary metastases in Ewing tumors: results from the (EI) CESS studies,” Annals of Oncology, vol. 10, no. 9, pp. 1073-1077, 1999.
[12]	M. J. A. Sharafuddin, F. S. Haddad, P. W. Hitchon, S. F. Haddad, and G. Y. El-Khoury, “Treatment options in primary Ewing’s sarcoma of the spine: report of seven cases and review of the literature,” Neurosurgery, vol. 30, no. 4, pp. 610-619, 1992.
[13]	Dhatt S, Dhillon MS, Tripathy SK, Goyal T, Jagadeesh V. Peripheral primitive neuroectodermal tumor causing cauda equina syndrome with destruction of L5 vertebra. Indian J Orthop, 44, pp. 339-41, 2010.
[14]	Jithendra K S,rachna S ,karak AK ,Thavaraj V, Kabra SK. Primitive Neuroectodermal Tumor presenting as Cauda equine syndrome. Indian J Med and Paediatr Oncol, 28, pp. 34-37, 2007.
[15]	Krishnakumar R, Renjitkumar J. Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine. Neurol India, 59, pp. 931-3, 2011.
[16]	Llauger J, Palmer J, Amores S, Bague S, Camins A. Primary Tumors of Sacrum-Diagnostic Imaging. American Roentgen Ray Society. 174, pp. 417-24, 2000.
[17]	Gagnier, Joel J., et al. “The CARE guidelines: consensus-based clinical case reporting guideline development.” Journal of medical case reports, 7.1, pp. 23, 2013.