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BACKGROUND: Primary intracranial rhabdomyosarcoma is an extraordinarily rare malignant tumor, with even fewer presenting with distant metastasis. To date, only five cases, including the one presented here, have been reported to present metastatic activity. OBSERVATIONS: A 12-year-old boy presented with a few days of headache, nausea, vomiting, but no neurological deficit. Brain computed tomography and magnetic resonance imaging demonstrated hydrocephalus and a cystic lesion with left parieto-occipital extension. After resection, pathology reported primary rhabdomyosarcoma, with positive desmin and myogenin on immunohistochemistry. The patient presented with pulmonary metastasis. The patient had an overall survival of 21 months after diagnosis with optimal treatment. LESSONS: Rhabdomyosarcoma is a malignant neoplasm arising from undifferentiated skeletal muscle cells, with morphological, immunohistochemical, ultrastructural, or molecular genetic evidence of primary skeletal muscle differentiation. It presents with a rapidly worsening clinical course and the final outcome is poor. Treatment is widely based on protocols that have been proven to be effective in extracranial versions of these tumors, although repeatedly ineffective. Primary brain rhabdomyosarcoma poses a diagnostic challenge because of its infrequent presentation, grade of undifferentiation and tumor heterogeneity. Immunohistochemical and genetic testing have proven to be useful tools for diagnosis.
RESUMO
Study Design: Prospective case series study. Purpose: Description of the outcome of stand-alone cervical cages for single and multilevel cervical degenerative spine disease. Overview of Literature: The aim of anterior cervical discectomy and fusion (ACDF) for cervical spine disease is to improve patient symptoms and spine stability and restore lordosis. Locking stand-alone cages were developed with the goal of minimizing soft tissue disruption anterior to the vertebrae and reducing the profile of the construct by avoiding an anterior plate, thereby maximizing ACDF benefits. Methods: This study comprises a case series of patients surgically treated between July 2015 and February 2018 who received single or multilevel ACDF with a zero-profile stand-alone cervical cage. Surgical and clinical preoperative evaluation and surgical outcomes were evaluated using pre- and postoperative Nurick, Visual Analog Scale (VAS), Neck Disability Index (NDI), Japanese Orthopedic Association (JOA) score for myelopathy scales, cervical Cobb angles, postoperative surgical complications, and fusion and subsidence rates. Results: Fifty-three patients underwent ACDF; the mean age of these patients was 58.8 years, and their preoperative VAS, NDI, and JOA scores were 8.1, 31.6, and 15.3, respectively. The preoperative Cobb angle was 30.7°. Forty-five percent of patients had onelevel, 54.7% had two-level, and 13.2% had three-level procedures. On preoperative magnetic resonance imaging, foraminal stenosis was present in 94.3% of patients, whereas medullar stenosis was present in 41.5%. The rate of complications was 5.7%: two patients had postoperative dysphagia (3.7%), and one patient had a surgical site hematoma. Mean postoperative follow-up time was 6.7 months; postoperative VAS, NDI, and JOA scores were 2.4, 15.9, and 15.8, respectively. Postoperative Cobb angle was 35.9°, fusion rate was 84.9%, and subsidence rate was 11.3%. Conclusions: ACDF with zero-profile stand-alone cervical devices is an excellent option for cervical degenerative disc disease of one, two, and three levels, with similar results reported when using ACDF with either cage or plate.
