Romanian Neurosurgery (2019) XXXIII (3): pp. 287-294 DOI: 10.33962/roneuro-2019-048 www.journals.lapub.co.uk/index.php/roneurosurgery Primary central nervous system sarcomas. Clinical, radiological and pathological features in our institution Wael K. Zakaria, Ahmed N. Taha, Mohamed State Neurosurgery Department; Mansoura University, EGYPT ABSTRACT 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. INTRODUCTION Primary CNS sarcomas are rare tumours. There are many theories of the cell of origin of these tumour. Tumour may arise from pluripotential primitive mesenchymal cells which located in the dura, or originated from the leptomeningial cells or their extensions via the pia into the brain and the spinal cord along the stroma of the choroid plexus, the tela choroidea, or the periadventitial spaces and these considered as widely accepted theory. We can defined sarcomas that have originated from metastases of soft tissue or bone sarcomas to the CNS as Secondary CNS sarcomas. These tumours result from direct extension of sarcoma located at places neighbouring the CNS. Keywords Primary CNS, sarcoma Corresponding author: Wael k. Zakaria Neurosurgery Department; Mansoura University, Egypt drwaelmusa@yahoo.com Copyright and usage. This is an Open Access article, distributed under the terms of the Creative Commons Attribution Non–Commercial No Derivatives License (https://creativecommons .org/licenses/by-nc-nd/4.0/) which permits non- commercial re-use, distribution, and reproduction in any medium, provided the original work is unaltered and is properly cited. The written permission of the Romanian Society of Neurosurgery must be obtained for commercial re-use or in order to create a derivative work. ISSN online 2344-4959 © Romanian Society of Neurosurgery First published September 2019 by London Academic Publishing www.lapub.co.uk http://www.lapub.co.uk/ 288 Wael k. Zakaria, Ahmed N. Taha, Mohamed State PATIENTS AND METHODS We looking for all types of primary CNS sarcomas between 2006-2018 at Mansoura university hospital through retrospective analysis of our CNS tumor database which archived in our neurosurgery department in these period. The Research Ethical committee at Mansoura medical school give us permission to use these patients’ data. In this study, we defined primary CNS sarcomas as tumours that originated primary in the CNS from non-neuronal, non-glial, and non-reticular elements, and we not include any previously diagnosed benign tumour with sarcomatous transformation. The medical data of these patients included in our definition of a primary CNS sarcoma were then analysed for clinical, radiological and pathological features. All primary CNS sarcoma were investigated for any possibility of the presence of extra CNS sarcomas at the time of study. The work-up study were chest X-ray, chest CT, abdominal and pelvic CT, and abdominal ultrasound. All cases surgical specimens were examined in pathology department by light microscopy using eosin and hematoxylin stain, periodic acid-Schiff, and reticulin. We used immune histochemical studies in all cases. The range of follow up varied from 3 month to 8 years (mean 2.9 years). We calculated the length of survival from the date of diagnosis of the disease and the end points were the patient death day or the last follow-up visit. 289 Primary central nervous system sarcomas 290 Wael k. Zakaria, Ahmed N. Taha, Mohamed State PATIENT Age/ sex location CT M R I Extent of resection Radio- therapy Chemo- therapy Dural attachment Brain invasion survival 1 73/f D10 Enhanceme nt ++, bone destruction Total Yes Yes No No Alive 1 years then absent 2 8/f D11-12 Enhanceme nt +++, cystic Near total No Yes Yes No Alive 1years 6 months 3 8/m Frontal lobe Enhanceme nt +++, bone destruction Total Yes Yes Yes No Alive 8years 4 47/ m Occipital lobe Cystic, enhanceme nt++,bone destruction Total Yes Yes Yes Yes Alive 3month s 5 8/m L2-5 Enhanceme nt +++, bone destruction ,cystic Total No Yes No No Alive 7years 6 18/ m Bifrontal lobe Cystic, enhanceme nt++, Near total Yes Yes Yes Yes Alive 7month s 7 16/ m D4-6 Enhanceme nt +++, bone destruction Total Yes Yes Yes No Alive 2years RESULTS Clinical pictures We summarized the clinical pictures in this study in Table 1. There were 2 female (28.6%) and 5 male (71.4%) ranged in age from 8 years to 47 years (mean age 19.4 years). Three patients in this study (42.9%) were in the first decade of life. Three sarcoma patients (42,9%) were intracranial and 4 patients (57.1%) were intraspinal. All intracranial sarcomas in this series were supratentorial in location, two of them presented bilaterally either in frontal or occipital lobes. Three of intraspinal tumours (75%) were located in the dorsal area and the remaining presented in the lumbar region. The clinical presentation of these patients varied from symptoms and signs of ICP as vomiting, headache and papillodema and other presentation as focal neurological deficit such as dysphasia, fits, hemiparesis for intra cranial tumours and paraparesis for the intra spinal tumours. Radiological features We summarized the radiological data in Table 1. CT and MRI used for investigation (Fig. 1). CT was performed in all patients, tumour enhancement with contrast had been obtained. All intracranial tumours patients in this study showing osteolytic bony destruction. 5 patients (71.4%) showed marked humoral enhancement. Magnetic resonance imaging in selected cases revealed increased signal on T2 images on these lesions, and irregularly enhancement after contrast study (Fig. 2). Surgery All patients in these series underwent at least one surgical procedure with the aim of surgery is gross tumoural resection; only 2 cases underwent another surgical operation due to recurrence of the tumour. Surgical tumoural resection was total in 5 cases (71.4%) and subtotal in the remaining 2 patients (28.6%). We performed true cut needle biopsy in just a patient of intracranial tumour with extra cranial extension prior to surgical excision. Postoperative radiotherapy was used in 5 patients (71.4%) and postoperative chemotherapeutic agents were used in all patients (100%). 291 Primary central nervous system sarcomas TABLE 1 showed the operative details of the patients included in this series. At surgery, we found dural attachment of the tumour in 5 patients (71.4%) and parenchymal invasion of the tumour in 2 patients (28.6%). We found a well-defined demarcation plane of dissection around the lesion in four patients, two of them were intracranial sarcoma and the other were intra spinal. Pathological features In this series, we found similar histological characters of the different intracranial and extra cranial sarcomas. We did immunohistochemical studies for all cases (Table 2). The most consistent data in immunohistochemical studies were strong positive results of vimentin and general negative results for both neuronal and glial markers. All patients showed positive Desmin with more rhabdomyoblastic differentiation. All patients tumour examined by Electron microscopic studies. There are many feature as the spindle shape of the cells, lack of well-developed junctions, dilated rough endoplasmic reticulum, and absence of microvilli and neurosecretory granules. The final pathological diagnosis in these series was a ewing's sarcoma in 2 (28.6%) patients, a embryonal rhabdomyosarcoma in 2 (28.6%), an otherwise low grade chodrosarcoma (14.3%), synovial sarcoma and an undifferinated sarcoma in the remaining three patients alternatively. Patient No desmi n neurone specific enolase BDL-2 LCA,CD3,CD9 9 1 negativ e positive negativ e NA 2 negativ e negative positive NA 3 positiv e negative NA NA 4 negativ e negative NA negative 5 negativ e positive NA NA 6 NA NA NA NA 7 positiv e NA NA negative Survival We calculate the length of survival from the day of the diagnosis of the disease (Table 1). We have data on survival for 6 patients and lack information in just one patient. The longest survival time in these study was eight years and the shortest survival time was three month (mean 3.2 years). The durations of patient survival in intracranial tumours ranged from three months to eight years (mean 2.9 years), while the intraspinal tumours ranged from 18 months to 7 years (mean 3.5 years). Three months to 8 years (mean 4 years) was the length of survival in the cases with gross surgical resection of the lesion; and the range of length of survival in cases with subtotal tumour resection was 18 months to 2 years (mean 1, 8 years). Adjuvant therapy We used Postoperative radiation in 5 patients; with a radiation dose ranged from 4,500–5,900 Gy. On the other hand we used Postoperative chemotherapy in all patients. The most used chemotherapeutic agents were ifosfamide, carboplatin, vincristine and cyclophosphamide. DISCUSSION The occurrence of a primary CNS sarcoma described firstly by Bailey in 1929.2 CNS sarcoma with all forms considered as rare tumours.⁶⁻¹⁴⁻³ºthe survival in sarcoma patients increased than in the past due to advances technique in chemotherapy. ⁶⁻²⁶⁻⁴⁵In different studies, we found variation in the incidence of primary CNS sarcomas which ranged from 0.1% to 4.3%.³⁻¹⁹⁻²⁷⁻³¹⁻³³⁻³⁶ This variation happened due to the term of a primary CNS sarcoma were inconsistent in different series. Previous reported studies included different forms such as giant cell sarcoma, circumscribed sarcoma of the posterior fossa, reticulum cell sarcoma, and hemangiopericytoma, and this gave a false result in high incidence of these tumour type.⁷⁻²º⁻²¹⁻²⁵⁻³⁵ In other series, they considered cases of primary glial, neuronal, neuroectodermal, and/or previously diagnoesd benign meningeal tumours with. sarcomatous features as sarcomas and this lead to falsely high incidence of these tumour type .²³⁻³¹finally, many studies not used immunohistochemistry that could be helpful to resolve diagnostic issues for many examined sarcomatous tumors.²³⁻³³⁻³⁶⁻⁴³ The origin of sarcoma have many theories, the most accepted theory assumed the origin of these 292 Wael k. Zakaria, Ahmed N. Taha, Mohamed State tumour to pluripotential primitive mesenchymal cells, the leptomeninges or their extension into the brain and the spinal cord via the pia along the. periadventitial spaces, the stroma of the choroid plexus and the tela choroidea.²⁻⁴⁻¹⁻²⁴⁻²⁵⁻³¹⁻³⁻³⁵⁻⁴⁵ The causes for occurrence of the primary CNS sarcoma is not known yet well accepted inciting agent of sarcoma in the literature is radiation therapy¹⁹⁻²³⁻²⁷⁻³⁴⁻⁵⁻³⁸⁻⁴⁴. There are another predisposing factor as trauma¹⁷⁻¹⁹⁻²⁷⁻²⁸⁻²⁹⁻³¹, genetic and familial factors¹⁹⁻³¹ and viruses such as RSV.⁸⁻²¹⁻²⁷⁻³¹ Primary CNS sarcomas may happen at any life decade, with high incidence in children ²³⁻³¹⁻⁴⁴. In these series sarcomas were exist in the age range 8– 73 years, with the youngest case was 8 years old. Primary intracranial sarcoma clinically presented as other intracranial tumours. However, it can present with intracranial hemorrhage due to their extreme vascularity¹⁻¹⁵⁻²⁷⁻³º. The clinical presentation of primary intraspinal sarcoma had the same clinical presentation of other intraspinal tumours. There are nonspecific image of primary CNS sarcomas on radiology as CT or MRI. In our series, tumour was large and with different heterogeneous density, cystic (hypodense), or solid and in one patient presented bilaterally in intracranial. Two of the 7 tumours had both solid and cystic components. MRI of sarcoma was available with an inhomogeneous signal. Solid parts of the tumour usually enhanced after administration of intravenous contrast. Enhancement of the tumour after contrast in the MRI has been a feature of sarcomas in the many series in literature ³⁴⁻³⁵. An important characteristic feature of sarcoma is the location near to the surface of the brain or the meninges, and it existed in four patients in our studies `. CNS sarcoma have high incidence of leptomeningeal spread ¹¹⁻¹³⁻⁴⁵. There are many differential diagnosis of primary CNS sarcoma on radiology as CT and MRI includes glioma, medulloblastoma in posterior fossa, cranial and spinal meningioma, and ependymoma ¹⁹⁻²⁷⁻³²⁻³⁶. In current study, our paients tumor are located in supratentorial region with the most common sites of origin is the frontal lobes which is not match with other studies in literature ¹³⁻²²⁻²³⁻³²⁻³⁶⁻⁴⁵. None of our patients presented with their tumour in intraventricular region, although in many series reported intraventricular sarcomas which originated from the choroid plexus²³⁻²⁶⁻²⁸⁻⁴º⁻⁴¹⁴⁵. A common feature of primary CNS sarcoma is dural involvement⁵⁻¹⁷⁻³⁶⁻⁴⁴⁻⁴⁵⁻⁴⁷. We have dural involvement of the tumour in 3 patients which considered as a half of the cases in our study. We did not report any malignant glial tumour within or adjacent to the primary sarcoma on our series. Although some series document that reactive glioma can be happened in a primary CNS sarcoma ¹⁶⁻²³⁻²⁹⁻⁴⁶. Primary sarcomas of CNS has the ability to send metastasis outside the central nervous system, as the liver, bone, and lung with a poor prognosis³⁻¹²⁻¹³⁻². It is difficult to determine the incidence of the different histological subtypes of these tumour. This is due rare incidence of the lesion, and the deficient criteria for including specific subtypes in the patients who documented in study ³⁻⁴º. We have frequent changes happened in the classification system for primary CNS sarcomas due to appearance of new histological subtypes such as malignant fibrous histiocytoma, and in the literature its considered as most common soft tissue sarcoma⁴⁻⁵⁻¹⁶⁻²⁷⁻³⁸ . Undifferentiated sarcoma considered as the most common pathological type in our study, and this not matched with others series ⁷⁻⁴⁵. Other series reported fibrosarcomas and MFH as the most pathological tumoural subtype ¹⁻²⁻⁹⁻³⁶. Primary CNS sarcomas characteristic with a bad prognosis, although the long duration of postoperative survival is well reported in many series in literature¹³⁻³¹⁻³²⁻³⁴⁻⁴º⁻⁴⁵. Many studies in the literature reported that gross surgical resection is considered as the best treatment of choice. We can use postoperative radiation or chemotherapy for prolonged the patient survival ¹⁻³⁻¹¹⁻¹³⁻¹⁷⁻¹⁶⁻³⁶⁻⁴º⁻⁴⁵⁻⁴⁶. In Our study, we found that maximal surgical resection of the tumour followed by post-operative radiotherapy help the patient to receive his best choice of management. Although, There are many side effects of radiation on young patient category. The aim of postoperative chemotherapy could not be exactly reported in our study. CONCLUSION The primary central nervous system sarcoma is a rare tumour that mainly originated in the 293 Primary central nervous system sarcomas supratentorial region with ability to be exist in the intraspinal region. CSF dissemination and dural attachment of the tumour are frequently exist. Metastases outside the CNS associated with bad prognosis. For long term survival we advised gross surgical resection followed by postoperative radiotherapy. Chemotherapy regimens may be considered as one of the factor that prolong the survival if they are tolerated by the patient. Future studies should be focused to understand the histopathological subtypes of primary CNS sarcoma to use different chemotherapeutic agents which be helpful to give patients long survival and best choice of treatment. REFERENCES 1. Anderson WR, Cameron JD, Tsai SH (1980) Primary intracranial leiomyosarcoma. Case report with ultrastructural study. J Neurosurg 53:401– 405PubMedGoogle Scholar 2. Arumugasamy N (1969) Some neuropathological aspects of intracranial sarcomas. Med J Malaya 23:169– 173PubMedGoogle Scholar 3. Asai A, Yamada H, Murata S, Matsuno A, Tsutsumi K, Takemura T, Matsutani M, Takakura K (1988) Primary leiomyosarcoma of the dura mater. Case report. J Neurosurg 68:308–311PubMedGoogle Scholar 4. Bahr AL, Gayler BW (1977) Cranial chondrosarcomas. Report of four cases and review of the literature. Radiology 124:151–156PubMedGoogle Scholar 5. Berry ADD, Reintjes SL, Kepes JJ (1988) Intracranial malignant fibrous histiocytoma with abscess-like tumor necrosis. Case report. J Neurosurg 69:780– 784PubMedGoogle Scholar 6. Bryant BM, Wiltshaw E (1980) Central nervous system involvement in sarcoma: a presentation of 12 cases, a review of the literature, and a discussion of possible changing patterns with the use of chemotherapy, placing special emphasis on embryonal tumours. Eur J Cancer 16:1503–1507PubMedGoogle Scholar 7. Cassady JR, Wilner HL (1967) The angiographic appearance of intracranial sarcomas. Radiology 88:258– 263PubMedGoogle Scholar 8. Charman HP, Lowenstein DH, Cho KG, DeArmond SJ, Wilson CB (1988) Primary cerebral angiosarcoma. Case report. J Neurosurg 68:806–810PubMedGoogle Scholar 9. Christensen E, Lara D (1953) Intracranial sarcomas. J Neuropathol Exp Neurol 12:41–56PubMedGoogle Scholar 10. Cybulski GR, Russell EJ, D’Angelo CM, Bailey OT (1985) Falcine chondrosarcoma: case report and literature review. Neurosurgery 16:412–415PubMedGoogle Scholar 11. Dropcho EJ, Allen JC (1987) Primary intracranial rhabdomyosarcoma: case report and review of the literature. J Neurooncol 5:139–150PubMedGoogle Scholar 12. El-Gindi S, Abd-el-Hafeez M, Salama M (1974) Extracranial skeletal metastases from an intracranial meningeal chondrosarcoma. Case report. J Neurosurg 40:651–653PubMedGoogle Scholar 13. Gaspar LE, Mackenzie IR, Gilbert JJ, Kaufmann JC, Fisher BF, Macdonald DR, Cairncross JG (1993) Primary cerebral fibrosarcomas. Clinicopathologic study and review of the literature. Cancer 72:3277–3281PubMedGoogle Scholar 14. Gercovich FG, Luna MA, Gottlieb JA (1975) Increased incidence of cerebral metastases in sarcoma patients with prolonged survival from chemotherapy. Report of cases of leiomyosarcoma and chondrosarcoma. Cancer 36:1843–1851PubMedGoogle Scholar 15. Heros RC, Martinez AJ, Ahn HS (1980) Intracranial mesenchymal chondrosarcoma. Surg Neurol 14:311– 317PubMedGoogle Scholar 16. Hirato J, Nakazato Y, Sasaki A, Yokota M, Nojiri K, Toyoda O, Nakajima H (1994) Intracranial malignant fibrous histiocytoma: characterization of GFAP-positive cells in the tumor. Clin Neuropathol 13:315–322PubMedGoogle Scholar 17. Ho YS, Wei CH, Tsai MD, Wai YY (1992) Intracerebral malignant fibrous histiocytoma: case report and review of the literature. Neurosurgery 31:567– 571PubMedGoogle Scholar 18. Hockley AD, Joffman HJ, Hendrick EB (1977) Occipital mesenchymal tumors of infancy. Report of three cases. J Neurosurg 46:239–244PubMedGoogle Scholar 19. Ironside JW (1991) Classification of primary intracranial sarcoma and other central nervous system neoplasms. Histopathology 18:483–486PubMedGoogle Scholar 20. Kernohan JW, Uihlein A (1962) Sarcomas of the brain. Thomas, SpringfieldGoogle Scholar 21. Kishikawa T, Numaguchi Y, Fukui M, Komaki S, Ikeda J, Kitamura K, Matsuura K (1981) Primary intracranial sarcomas: radiological diagnosis with emphasis on arteriography. Neuroradiology 21:25–31PubMedGoogle Scholar 22. Kubota T, Hayashi M, Yamamoto S (1982) Primary intracranial mesenchymal chondrosarcoma: case report with review of the literature. Neurosurgery 10:105– 110PubMedGoogle Scholar 23. Lalitha VS, Rubinstein LJ (1979) Reactive glioma in intracranial sarcoma: a form of mixed sarcoma and glioma (“sarcoglioma”): report of eight cases. Cancer 43:246–257PubMedGoogle Scholar 24. Lam RMY, Malik GM, Chason JL (1981) Osteosarcoma of meninges: clinical, light, and ultrastructural observations of a case. Am J Surg Pathol 5:203–208PubMedGoogle Scholar 25. Legier JF, Wells Jr HA (1967) Primary cerebellar rhabdomyosarcoma. Case report. J Neurosurg 26:436– 438PubMedGoogle Scholar 26. Louis DN, Richardson Jr EP, Dickersin GR, Petrucci DA, Rosenberg AE, Ojemann RG (1989) Primary intracranial leiomyosarcoma. Case report. J Neurosurg 71:279– 282PubMedGoogle Scholar 27. Martinez-Salazar A, Supler M, Rojiani AM (1997) Primary intracerebral malignant fibrous histiocytoma: 294 Wael k. Zakaria, Ahmed N. Taha, Mohamed State immunohistochemical findings and etiopathogenetic considerations. Mod Pathol 10:149–154PubMedGoogle Scholar 28. Matsukado Y, Yokota A, Marubayashi T (1975) Rhabdomyosarcoma of the brain. J Neurosurg 43:215– 221PubMedGoogle Scholar 29. Mayo CM, Barron KD (1966) Concurrent glioma and primary intracranial sarcoma. A report of two cases and a review of the literature. Neurology 16:662– 672PubMedGoogle Scholar 30. McDonald P, Guha A, Provias J (1997) Primary intracranial fibrosarcoma with intratumoral hemorrhage: neuropathological diagnosis with review of the literature. J Neurooncol 35:133–139PubMedGoogle Scholar 31. Mena H, Garcia JH (1978) Primary brain sarcomas: light and electron microscopic features. Cancer 42:1289– 1307Google Scholar 32. Mena H, Ribas J, Enzinger FM, Parisi JE (1991) Primary angiosarcoma of the central nervous system: study of eight cases and review of the literature. J Neurosurg 75:73–76PubMedGoogle Scholar 33. Min KW, Gyorkey F, Halpert B (1975) Primary rhabdomyosarcoma of the cerebrum. Cancer 35:1405– 1411PubMedGoogle Scholar 34. Niwa J, Hashi K, Minase T (1996) Radiation induced intracranial leiomyosarcoma: its histopathological features. Acta Neurochir 138:1470–1471Google Scholar 35. Onofrio BM, Kernohan JW, Uihhelein A (1962) Primary meningeal sarcomatosis: a review of the literature and report of 12 cases. Cancer 15:1197–1208PubMedGoogle Scholar 36. Paulus W, Slowik F, Jellinger K (1991) Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18:395–402PubMedGoogle Scholar 37. Raskind R, Grant S (1966) Primary mesenchymal chondrosarcoma of the cerebrum. Report of a case. J Neurosurg 24:676–678PubMedGoogle Scholar 38. Roosen N, Cras P, Paquier P, Martin JJ (1989) Primary thalamic malignant fibrous histiocytoma of the dominant hemisphere causing severe neuropsychological symptoms. Clin Neuropathol 8:16– 21PubMedGoogle Scholar 39. Roy S, Bhatia R, Nanda NR (1980) Primary rhabdomyosarcoma of the cerebellum. J Pathol 132:235–241PubMedGoogle Scholar 40. Rueda-Franco F, Lopez-Corella E (1995) Sarcomas in the central nervous system of children. 1982 [classical article]. Pediatr Neurosurg 22:49–55PubMedGoogle Scholar 41. Scott RM, Dickersin R, Wolpert SM, Twitchell T (1976) Myxochondrosarcoma of the fourth ventricle. Case report. J Neurosurg 44:386–389PubMedGoogle Scholar 42. Shuangshoti S, Piyaratn P, Viriyapanich PL (1968) Primary rhabdomyosarcoma of cerebellum—necropsy report. Cancer 22:367–371PubMedGoogle Scholar 43. Smith MT, Armbrustmacher VW, Violett TW (1981) Diffuse meningeal rhabdomyosarcoma. Cancer 47:2081–2086PubMedGoogle Scholar 44. Stone JL, Zavala G, Bailey OT (1979) Mixed malignant mesenchymal tumor of the cerebellar vermis. Cancer 44:2165–2172PubMedGoogle Scholar 45. Tomita T, Gonzalez-Crussi F (1984) Intracranial primary nonlymphomatous sarcoma in children: experience with eight cases and review of the literature. Neurosurgery 14:529–540PubMedGoogle Scholar 46. Vecchio F, Giordano R, Zanche LD, Caro RD, Maggioni F, Zanchin G (1988) Intracranial sarcoma with reactive glioma: a clinicopathological case report. Eur Neurol 28:301–305PubMedGoogle Scholar 47. Waga S, Matsushima M, Ando K, Morii S (1972) Intracranial chondrosarcoma with extracranial metastases. Case report. J Neurosurg 36:790– 794PubMedGoogle Scholar 48. Younis GA, Sawaya R, DeMonte F, Hess KR, Albrecht S, Bruner JM (1995) Aggressive meningeal tumours: review of a series. J Neurosurg 82:17–27PubMedGoogle Scholar 49. Zwartverwer FL, Kaplan AM, Hart MC, Hertel GA, Spataro J (1978) Meningeal sarcoma of the spinal cord in a newborn. Arch Neurol 35:844–846PubMedGoogle Scholar.