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Objective: Primary CNS sarcomas are very rare tumours with no defined standard of care.
Patients and methods: This study was a retrospective review of seven patients diagnosed with a primary CNS sarcoma at neurosurgery department, Mansoura university hospital between 2006 and 2018. We reviewed the clinical, radiological, and pathological data of these patients. There were 2 female (28.6%) and 5 male (71.4%) with age ranged from 8 years to 73 years (mean age 25.4 years). Three patients (42.9%) had an intracranial sarcoma, and four (57.1%) had intraspinal tumours. All intracranial tumours located in supratentorial region.
Results: we have characteristic imaging findings inform of osteolytic bony erosion in 3 patients and marked enhancement of the tumour in 5 (71.4%) patients. We operated upon all patients to remove the tumour surgically with our aim is gross tumour resection. Tumour was totally resected in 5 patients (71.4%) and subtotal in another 2 patients (28.6%). Tumour has dural attachment in 5 cases (71.4%) and brain invasion was present in all intracranial 3 patients (42.9%). Postoperative radiotherapy was used in 5 patients and postoperative chemotherapy was used in all patients. We used immunohistochemical studies for all patients with the most consistent finding being strong Desmin positivity. The mean length of patients survival was 4.6 years (range from 3 month to 8 years).
Conclusions: Primary CNS sarcomas are very rare CNS tumours, total surgical resection and post-operative radio and chemotherapy provided encouraging outcomes.