Cervical C2 to C4 schwannoma with intratumoral hemorrhage presenting as acute spastic quadriparesis: A rare case report
- Department of Neurosurgery, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India
- Department of Paediatrics, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India
- Department of Pathology, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India
C. S. Gandhoke
Department of Neurosurgery, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India
DOI:10.4103/sni.sni_171_18Copyright: © 2018 Surgical Neurology International This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
How to cite this article: C. S. Gandhoke, S. K. Syal, D Singh, V. Batra, Y. Nallacheruvu. Cervical C2 to C4 schwannoma with intratumoral hemorrhage presenting as acute spastic quadriparesis: A rare case report. 24-Jul-2018;9:142
How to cite this URL: C. S. Gandhoke, S. K. Syal, D Singh, V. Batra, Y. Nallacheruvu. Cervical C2 to C4 schwannoma with intratumoral hemorrhage presenting as acute spastic quadriparesis: A rare case report. 24-Jul-2018;9:142. Available from: http://surgicalneurologyint.com/?post_type=surgicalint_articles&p=8937
Background:Spinal schwannomas are slow growing, benign nerve sheath tumors. These may be asymptomatic or may present as backache with radicular pain, slowly progressive neurological deficits, but rarely with acute spastic quadriparesis attributed to intratumoral hemorrhage.
Case Description:A 38-year-old male presented with the chief complaint of neck pain radiating to the left upper extremity for the last 8 months. On admission, he exhibited diffuse hyper-reflexia but had no motor or sensory deficit. Magnetic resonance imaging showed a solid-cystic intradural extramedullary (IDEM) C2 to C4 mass severely compressing the spinal cord. The same day the patient acutely developed a spastic quadriparesis. Immediately, a partial C2, C3, and C4 laminectomy was performed for tumor excision; within 5 postoperative days, he fully regained neurological function. The final histopathology was consistent with a “schwannoma showing areas of congestion and hemorrhage.”
Conclusion:Spinal schwannomas rarely present with intratumoral hemorrhage and acute spastic quadriparesis. Immediate operative decompression may lead to excellent postoperative neurological recovery.
Keywords: Cervical, intratumoral, hemorrhage, quadriparesis, schwannoma
Spinal schwannomas are slow growing, benign nerve sheath tumors. These may be asymptomatic, present as backache/radicular pain, or slowly progressive neurological deficits. Patients rarely present with acute spastic quadriparesis attributed to acute intratumoral hemorrhage.
We report the case of a 38-year-old male who presented with neck pain radiating to the left upper extremity for the last 8 months. On admission, he exhibited isolated hyper-reflexia without a motor or sensory deficit. The cervical magnetic resonance imaging (MRI) showed on T1, a hypointense, and on T2, a heterogeneously hyperintense solid-cystic intradural extramedullary (IDEM) C2 to C4 mass severely compressing the spinal cord; that heterogeneously enhanced with contrast [
MRI cervical spine suggestive of (a) T1 hypointense (b) T2 heterogeneously hyperintense solid - cystic intradural extramedullary (IDEM) C2 to C4 mass severely compressing the spinal cord and pushing it anteriorly and to the right. (c) The lesion shows heterogeneous post contrast enhancement.
The hematoxylin and eosin sections showed numerous congested and dilated blood vessels, with both hypercellular Antoni A and hypocellular Antoni B areas composed of spindle cells. Immunohistochemistry was also positive for S100 protein, leading to the final histopathological diagnosis of “schwannoma showing areas of congestion and hemorrhage” [
(a) Hematoxylin and eosin 40x magnification: Low power view of the tumor showing numerous congested and dilated blood vessels. (b) Hematoxylin and eosin 200x magnification: Both hypocellular and hypercellular areas of schwanomma seen with the hypercellular areas showing the tumour to be composed of spindle cells. (c and d) S100 staining, 100x and 200x magnification respectively: S100 stain at low and high power respectively showing cytoplasmic positivity in tumour cells proving the neuronal origin of the cells
Schwannomas are typically round or oval, lobulated, and encapsulated tumors arising eccentrically from a nerve root sheath. They are most commonly intradural extramedullary (58%) in location, followed by extradural (27%), dumb-bell shaped (15% both intradural/extradural), and rarely have intramedullary (less than 1%) spinal involvement.[
On MRI, spinal schwannomas are typically hypointense on T1; on T2-weighted studies, they may be hyperintense and/or heterogeneous with focal areas of hyperintensity/hypointensity corresponding to cyst formation, hemorrhage, dense cellularity, and/or collagen deposition.[
Intratumoral hemorrhages in spinal schwannomas
Intratumoral hemorrhages are occasionally reported with cranial schwannomas, but rarely with spinal schwannomas. There are various theories regarding the etiology of these acute intratumoral bleeds. According to the vascular theory, hyalinized ectatic vessels inside the schwannoma may undergo spontaneous thrombosis resulting in distal necrosis and hemorrhage.[
Treatment of acute spinal intratumoral hemorrhages
Treatment options for acute intratumoral hemorrhages in spinal schwannomas include complete excision of the tumor and its capsule. Early diagnosis and emergent removal of these lesions may lead to partial/full resolution of the acute neurological deficits.[
Spinal schwannomas rarely present with intratumoral hemorrhage leading to acute spastic quadriparesis. In this setting, immediate operative intervention may lead to complete neurological recovery.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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