CC BY-NC-ND 4.0 · Indian Journal of Neurosurgery 2018; 07(03): 239-248
DOI: 10.1055/s-0037-1599788
Case Report
Neurological Surgeons' Society of India

Cervical Intramedullary Schwannoma with Syrinx: Case Report and Review of the Literature

Abdulaziz AlQarni
1   Department of Neurosurgery, King Abdulaziz Medical City, Saudi Arabia, Riyadh
,
A. AlArifi
1   Department of Neurosurgery, King Abdulaziz Medical City, Saudi Arabia, Riyadh
,
Ali H. Alassiri
1   Department of Neurosurgery, King Abdulaziz Medical City, Saudi Arabia, Riyadh
,
Amjed Kouli
1   Department of Neurosurgery, King Abdulaziz Medical City, Saudi Arabia, Riyadh
,
M. T. Abbas
1   Department of Neurosurgery, King Abdulaziz Medical City, Saudi Arabia, Riyadh
› Author Affiliations
Further Information

Publication History

Received: 31 July 2016

accepted: 18 October 2016

Publication Date:
23 October 2017 (online)

Abstract

Schwannoma is a nerve sheath tumor originating from the Schwann cell. It is benign in nature and it arises from anywhere where Schwann cells can be found. It is rarely found in the parenchyma of the spinal cord. Intramedullary schwannomas (or neurilemmomas) without evidence of neurofibromatosis are rare spinal cord tumors. Intramedullary schwannoma was first reported in 1932 by Penfield. Our patient presented with neck pain, gradually worsening, weakness in the right upper and lower limbs, numbness in both shoulders, and a decrease in the grasping strength of both hands over a 4-year period. A magnetic resonance imaging of the spine showed a heterogeneously enhancing mass in the cervical spinal cord extending from the C2 to T1 levels with associated hemorrhagic changes. Histologically, the tumor was found to be composed of bland spindle cells with blunt-ended and sometimes wavy nuclei admixed with hyalinized vasculature. Surrounding reactive spinal cord parenchyma with frequent Rosenthal fibers was also observed. Focal Verocay bodies were evident, and with immunohistochemistry, there was diffuse and strong positivity for S100, which is confirmatory for the diagnosis of schwannoma. We report a case of cervical intramedullary schwannoma presented with syringobulbia in a young adult.

 
  • References

  • 1 Binatli O, Erşahin Y, Korkmaz O, Bayol U. Intramedullary schwannoma of the spinal cord. A case report and review of the literature. J Neurosurg Sci 1999; 43 (02) 163-167 discussion 167–168
  • 2 Ohtonari T, Nishihara N, Ota T, Ota S, Koyama T. Intramedullary schwannoma of the conus medullaris complicated by dense adhesion to neural tissue. Neurol Med Chir (Tokyo) 2009; 49 (11) 536-538
  • 3 Ross DA, Edwards MS, Wilson CB. Intramedullary neurilemomas of the spinal cord: report of two cases and review of the literature. Neurosurgery 1986; 19 (03) 458-464
  • 4 Kodama Y, Terae S, Hida K, Chu BC, Kaneko K, Miyasaka K. Intramedullary schwannoma of the spinal cord: report of two cases. Neuroradiology 2001; 43 (07) 567-571
  • 5 Colosimo C, Cerase A, Denaro L, Maira G, Greco R. Magnetic resonance imaging of intramedullary spinal cord schwannomas. Report of two cases and review of the literature. J Neuro-surg 2003; 99 (01) Suppl 114-117
  • 6 Conti P, Pansini G, Mouchaty H, Capuano C, Conti R. Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature. Surg Neurol 2004; 61 (01) 34-43 discussion 44
  • 7 Riffaud L, Morandi X, Massengo S, Carsin-Nicol B, Heresbach N, Guegan Y. MRI of intramedullary spinal schwannomas: case report and review of the literature. Neuroradiology 2000; 42 (04) 275-279
  • 8 Ho T, Tai KS, Fan YW, Leong LL. Intramedullary spinal schwannoma: case report and review of preoperative magnetic resonance imaging features. Asian J Surg 2006; 29 (04) 306-308
  • 9 Nadkarni TD, Rekate HL. Pediatric intramedullary spinal cord tumors. Critical review of the literature. Childs Nerv Syst 1999; 15 (01) 17-28
  • 10 Nicácio JM, Rodrigues JC, Galles MH, Faquini IV, de Brito PereiraCA, Ganau M. Cervical intramedullary schwannoma: a case report and review of the literature. Rare Tumors 2009; 1 (02) e44
  • 11 Kim NR, Suh YL, Shin HJ. Thoracic pediatric intramedullary schwannoma: report of a case. Pediatr Neurosurg 2009; 45 (05) 396-401
  • 12 Cantore G, Ciappetta P, Delfini R, Vagnozzi R, Nolletti A. Intramedullary spinal neurinomas. Report of two cases. J Neuro-surg 1982; 57 (01) 143-147
  • 13 Kyoshima K, Horiuchi T, Zenisaka H, Nakazato F. Thoracic dumbbell intra- and extramedullary schwannoma. J Clin Neurosci 2005; 12 (04) 481-484
  • 14 Cambier J, Masson M, Hurth M, Poirier J, Dehen H. Unilateral posterior-column syndrome due to intramedullary neurinoma. Imitative homolateral synkinesias [in French] Rev Neurol (Paris) 1974; 130 (05) (06) 189-199
  • 15 Wood WG, Rothman LM, Nussbaum BE. Intramedullary neurilemoma of the cervical spinal cord. Case report. J Neurosurg 1975; 42 (04) 465-468
  • 16 Morimoto T, Sasaki T, Mochizuki T, Takakura K, Sato A. Thoracic intramedullary neurinoma with multiple intracranial meningiomas; case report [in Japanese]. No Shinkei Geka 1992; 20 (04) 423-427
  • 17 Bhayani R, Goel A. Multiple intramedullary schwannomas–case report. Neurol Med Chir (Tokyo) 1996; 36 (07) 466-468
  • 18 Lee SE, Chung CK, Kim HJ. Intramedullary schwannomas: long-term outcomes of ten operated cases. J Neurooncol 2013; 113 (01) 75-81
  • 19 Yang T, Wu L, Deng X, Yang C, Xu Y. Clinical features and surgical outcomes of intramedullary schwannomas. Acta Neurochir (Wien) 2014; 156 (09) 1789-1797
  • 20 Rasmussen TB, Kernohan JW, Adson AW. Pathologic classification, with surgical consideration, of intraspinal tumors. Ann Surg 1940; 111 (04) 513-530
  • 21 Gorman PH, Rigamonti D, Joslyn JN. Intramedullary and extramedullary schwannoma of the cervical spinal cord–case report. Surg Neurol 1989; 32 (06) 459-462
  • 22 Sharma SC, Ray RC, Banerjee AK. Intramedullary spinal schwannoma. Indian Pediatr 1989; 26 (03) 290-292
  • 23 Mukerji G, Sherekar S, Yadav YR, Chandrakar SK, Raina VK. Pediatric intramedullary schwannoma without neurofibromatosis. Neurol India 2007; 55 (01) 54-56
  • 24 Eljebbouri B, Gazzaz M, Akhaddar A, Elmostarchid B, Boucetta M. Pediatric intramedullary schwannoma without neurofibromatosis: case report. Acta Med Iran 2013; 51 (10) 727-729
  • 25 Herregodts P, Vloeberghs M, Schmedding E, Goossens A, Stadnik T, D'Haens J. Solitary dorsal intramedullary schwannoma. Case report. J Neurosurg 1991; 74 (05) 816-820
  • 26 Darwish BS, Balakrishnan V, Maitra R. Intramedullary ancient schwannoma of the cervical spinal cord: case report and review of literature. J Clin Neurosci 2002; 9 (03) 321-323
  • 27 Kahilogullari G, Aydin Z, Ayten M, Attar A, Erdem A. Schwannoma of the conus medullaris. J Clin Neurosci 2005; 12 (01) 80-81
  • 28 Kim SD, Nakagawa H, Mizuno J, Inoue T. Thoracic subpial intramedullary schwannoma involving a ventral nerve root: a case report and review of the literature. Surg Neurol 2005; 63 (04) 389-393 discussion 393
  • 29 Shenoy SN, Raja A. Cystic cervical intramedullary schwannoma with syringomyelia. Neurol India 2005; 53 (02) 224-225
  • 30 Ozawa N, Tashiro T, Okamura T, Koyama K, Ohata K, Inoue Y. Subpial schwannoma of the cervical spinal cord mimicking an intramedullary tumor. Radiat Med 2006; 24 (10) 690-694
  • 31 Hida K, Yano S, Iwasaki Y. Staged operation for huge cervical intramedullary schwannoma: report of two cases. Neurosurgery 2008; 62 (05) (Suppl. 02) ONS456-ONS460 discussion ONS460
  • 32 Hayashi F, Sakai T, Sairyo K. et al. Intramedullary schwannoma with calcification of the epiconus. Spine J 2009; 9 (05) e19-e23
  • 33 Lyle CA, Malicki D, Senac MO, Levy ML, Crawford JR. Congenital giant intramedullary spinal cord schwannoma. Neurology 2010; 75 (19) 1752
  • 34 Ryu KS, Lee KY, Lee HJ, Park CK. Thoracic intramedullary schwannoma accompanying by extramedullary beads-like daughter schwanommas. J Korean Neurosurg Soc 2011; 49 (05) 302-304
  • 35 Vij M, Jaiswal S, Jaiswal AK, Behari S. Coexisting intramedullary schwannoma with intramedullary cysticercus: report of an unusual collision. Indian J Pathol Microbiol 2011; 54 (04) 866-867
  • 36 Li J, Ke Y, Huang M, Li Z, Wu Y. Microexcision of intramedullary schwannoma at the thoracic vertebra. Exp Ther Med 2013; 5 (03) 845-847
  • 37 Karatay M, Koktekir E, Erdem Y, Celik H, Sertbas I, Bayar MA. Intramedullary schwannoma of conus medullaris with syringomyelia. Asian J Surg 2014 (e-pub ahead of print). Doi: 10.1016/j.asjsur.2014.04.004
  • 38 Wu L, Yao N, Chen D, Deng X, Xu Y. Preoperative diagnosis of intramedullary spinal schwannomas. Neurol Med Chir (Tokyo) 2011; 51 (09) 630-634
  • 39 Balériaux DL. Spinal cord tumors. Eur Radiol 1999; 9 (07) 1252-1258
  • 40 Nemoto Y, Inoue Y, Tashiro T. et al. Intramedullary spinal cord tumors: significance of associated hemorrhage at MR imaging. Radiology 1992; 182 (03) 793-796
  • 41 Miyazawa N, Hida K, Iwasaki Y, Koyanagi I, Abe H. MRI at 1.5 T of intramedullary ependymoma and classification of pattern of contrast enhancement. Neuroradiology 2000; 42 (11) 828-832
  • 42 Chu BC, Terae S, Hida K, Furukawa M, Abe S, Miyasaka K. MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol 2001; 22 (01) 206-217
  • 43 Duong H, Tampieri D, Melançon D, Salazar A, Robert F, Alwatban J. Intramedullary schwannoma. Can Assoc Radiol J 1995; 46 (03) 179-182