Primary spinal epidural rhabdomyosarcoma: a case report and review of the literature.
BACKGROUND
Primary intraspinal sarcomas are very rare in children. Rhabdomyosarcoma (RMS) is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space.


METHODS
We report a 13-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 T11-T12 level was observed on MRI. The patient underwent T12 hemilaminectomy, partial T11 hemilaminectomy, and right facetectomy. A large, firm, dark-red vascular epidural tumor was found compressing the cord. The tumor which extended to intracanal and foraminal parts was removed completely.


CONCLUSIONS
Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.
Department of Neurosurgery, Arad Hospital, Somayeh St., between Dr. Shariati & Bahar Ave., Tehran 1445613131, Iran. mrkhalatbari@hotmail.com
Mahmoud Reza
MR
Khalatbari
Hassan
H
Jalaeikhoo
Mehrdokht
M
Hamidi
Yashar
Y
Moharamzad
D000293:Adolescent; D015174:Epidural Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D004824:Epidural Space / Q000473:pathology*; D005147:Facial Bones / Q000601:surgery; D005260:Female; D006801:Humans; D007796:Laminectomy / Q000379:methods*; D008279:Magnetic Resonance Imaging; D018008:Myogenin / Q000378:metabolism; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000601:surgery*; D014057:Tomography, X-Ray Computed
D018008:Myogenin