Unusual course of an epidural rhabdomyosarcoma of the upper thoracic spine

Tsitsopoulos, Parmenion; Tsonidis, Christos; Nanasis, K. A.; Tsoleka, Kalliopi; Tavridis, G. N.

doi:10.1007/bf02187768

Cited by 9 publications

(7 citation statements)

References 2 publications

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“…Primary spinal epidural RMS is an extremely rare tumor and only few cases have been reported in the literature [1][2][3]. RMS occurs sporadically and no predisposing or risk factors have been recognized in the majority of cases [4], it is thought that RMS arises as a consequence of regulatory disruption of skeletal muscle progenitor cell growth and differentiation [5].…”

Section: Discussionmentioning

confidence: 99%

“…The limb weakness is usually spastic, but may be flaccid if the conus medullaris or the cauda equine is involved as in our patient. Other symptoms and signs include localized swelling, scoliosis, torticollis and sensory disturbance [2,3,6,11]. Radiologically, primary spinal RMS is usually hypointense on T1Wand hyperintense on T2W images and shows homogeneous or inhomogeneous enhancement after Gadolinium injection [2,3].…”

Section: Discussionmentioning

confidence: 99%

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Primary Spinal Epidural Rhabdomyosarcoma of the Upper Thoracic Spine

Bouali¹,

Bouhoula²,

Boubaker³

et al. 2014

J Spine

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Introduction: Rhabdomyosarcoma is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space. Method:We report a 20-year-old girl who presented with back pain, progressive paraparesis, and urinary retention.She had muscular weakness in her lower extremities and absent deep tendon reflex.An epidural dumbbell-shape mass at T3-T4 level was observed on MRI.The patient underwent T3-T5 hemilaminectomy.Result: Histopathological examination Immunohistochemical staining confirmed the diagnosis of alveolar Rhabdomyosarcoma. She received radiotherapy and chemotherapy. The patient died 3 months after. Conclusion:Primary spinal epidural RMS is an extremely rare and very aggressive tumor. The treatment should focus on extensive resection with intensive combination of radiotherapy and chemotherapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Primary Spinal Epidural Rhabdomyosarcoma of the Upper Thoracic Spine

Bouali¹,

Bouhoula²,

Boubaker³

et al. 2014

J Spine

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“…Primary spinal epidural rabdomyosarcoma is an extremely rare tumor and only few cases have been reported. 20,44 It is a highly aggressive and rapidly growing neoplasm of skeletal muscle origin that occasionally appears in the vertebral column and spinal epidural space. This is a disease of children and young adults.…”

Section: Rabdomyosarcomamentioning

confidence: 99%

“…Radiologically primary spinal rabdomyosarcoma is usually hypointense on T 1 weighted and hyperintense in T 2 weighted images and shows homogeneous or inhomogeneous enhancement on contrast. 44 This malignant tumor invades local structures and metastases to remote site by lymphatic and hematogenous spread. Treatment of spinal rabdomyosarcoma requires multi-disciplinary approaches that include surgery, chemotherapy and radiotherapy.…”

Section: Rabdomyosarcomamentioning

confidence: 99%

Extradural Spinal Tumors: Report of 36 Cases and Review of Literature

et al. 2016

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About 55% of spinal tumors are extradural arising from vertebral bodies, epidural and surrounding neural and soft tissues. More than 90% of extradural spinal tumors are metastatic lesions. Extradural spinal tumors are common cause of back pain, sensory motor deficit and sphincters dysfunction. The wide range of lesions and varied clinical profile, make management of spinal extradural tumors a challenging task for any neurosurgeon.Aim of this study is to analyze and discuss the results of extradural spinal tumors after surgical treatment and relevant literature will be reviewed.This is a retrospective study of 36 patients who were operated for spinal extradural tumors between May 1999 and December 2012 in our institute.Follow up period ranged from 3 years to 12 years. Functional neurological outcome was assessed by McCormick's grading. There were 20 male and 16 female and age ranged from 10 to 80 years. 30 patients presented with back pain and 15 had radicular pain. On presentation 18 patients had motor sensory deficit and 15 had sphincters dysfunction. Most common involved level of spines were thoracic followed by cervical and lumbar.Gross total excision of masses were carried out in all cases. Common pathologies were neurofibromas (16), Ewing's sarcoma (7), granulomas (3), metastatic lesions (2), angiolipoma (2), chondroma (2), aneurysmal bone cyst (1), plasmacytoma (1), rabdomyosarcoma (1) and neuroblastoma (1).Out of 14 patients who harbored malignant pathology 12 patients received radio and chemotherapy. Post operative wound infection occurred in 5 patients. Regarding post operative neurological status, 18 patients showed improvement, 6 patients remained same and 12 patients had deteriorated neurology. Tumor recurrence occurred in 15 patients; 12 patients with malignant and 3 patients with benign lesions on follow up period. There was no surgery related mortality, however, 11 patients died during 3 years follow up period due to adverse pathology they were having.Nepal Journal of Neuroscience 13:3-10, 2016

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“…These rare adult tumors predominantly arise in the head and neck region and on the extremities [3]. There have been reported cases of spinal RMS found in children, but only 2 cases have been reported in adults [4‐9]. Interestingly, neither occurred in the lumbar spine nor was found to be the adult spindle cell variant.…”

Section: Introductionmentioning

confidence: 99%