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Journal of Clinical Oncology, Vol 25, No 4 (February 1), 2007: pp. 447-449 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.07.8527
Gliosarcoma With Intramedullary Spinal Metastases: A Case Report and Review of the LiteratureWashington Cancer Institute at Washington Hospital Center, Washington, DC
Department of Pathology, Washington Hospital Center, Washington, DC
Washington Hospital Center/Center for Image Guided Neurosurgery, Washington, DC
Washington Cancer Institute at Washington Hospital Center, Washington, DC A 50-year-old man from El Salvador presented with a 3-week history of lower back pain, intermittent bitemporal headaches, and intermittent dizziness. Three days before his hospital admission, he developed weakness and numbness in the left lower extremity along with right foot numbness. A computed tomography (CT) scan of the head was performed at an outside hospital and showed multiple brain lesions on the right side. His past medical and social history were unremarkable. He last visited El Salvador 2 years ago. His family history was noteworthy because of a sister who died as a teenager from a brain tumor. No additional information regarding this diagnosis was available. A magnetic resonance image (MRI) with gadolinium contrast of the brain and spine was performed (Fig 1), which showed a 1.8 cm enhancing intramedullary lesion at the T9-T10 level with surrounding edema and six small intracranial masses in the right cerebral hemisphere, each with surrounding vasogenic edema. CT scans of the chest, abdomen, and pelvis revealed no additional findings. Because of worsening weakness, the patient underwent an emergent posterior thoracic laminectomy with resection of intradural tumor. Pathologic review showed the presence of malignant appearing spindle and epithelioid cells with visible mitotic figures and necrosis (Fig 2A). He also underwent biopsy and resection of a frontal lobe lesion for further diagnostic confirmation. Histologic examination of this specimen again revealed a biphasic neoplasm having both epithelioid and spindle cells (Fig 2B). The highly proliferative nature of tumor was again noted and supported by a markedly increased nuclear Ki-67 staining. The epithelioid component exhibited strongly positive staining of glial fibrillary acid protein (GFAP, Fig 2C). The spindle cells stained positive for smooth muscle actin and were surrounded by abundant reticulin fiber (Fig 2D). These findings strongly supported the diagnosis of gliosarcoma; a glioblastoma multiforme (GBM) variant recognized in the most recent classification by the WHO (2000). The spinal lesion represented a CSF drop metastasis from the primary cerebral gliosarcoma. In addition to aggressive inpatient rehabilitation, the patient received whole brain radiotherapy as well as local spinal irradiation with concurrent administration of temozolomide. Initially, he exhibited slow neurologic improvement. Subsequently, his performance status deteriorated and he was admitted to a home hospice program. He died about 5 months after his initial diagnosis.
Gliosarcoma is a very rare primary CNS neoplasm, which consists of both neuroectodermal and mesenchymal elements. Gliosarcoma represents approximately 2% of all cases of GBM.1 A glioblastoma with sarcomatous elements was originally described by Stroebe et al2 in 1895. In 1955, Feigin et al,3 reviewed several gliosarcoma cases and proposed that the endothelial hyperplasia of cerebral blood vessels within the high grade glial tumors may represent the malignant sarcomatous component. The characteristic histopathologic feature includes a biphasic tumor composed of malignant glial cells, which exhibit immunoreactivity for GFAP, and sarcomatoid cells that are invested by reticulin fibers.4 The predominant histologic picture of the sarcoma component is fibrous histiocytoma, however, descriptions of osteoid, chondroid, myxoid, lipoid, as well as smooth and skeletal muscle have been reported.5-10 Previously, controversy regarding the evolution of the mesenchymal portion of gliosarcomas existed. However, more recent studies support the monoclonol origin of gliosarcomas.11-12 Reis et al11 demonstrated that gliosarcomas exhibit genetic anomalies previously noted in GBM, such as p53 mutations, phosphatase and tensin homolog gene deleted on chromosome 10 (PTEN) mutations, and homozygosity for the p16 deletion. Gliosarcomas, however, were not noted to overexpress epidermal growth factor receptor (EGFR), which is commonly seen with GBM. In addition, the presence of identical alterations in both the gliomatous and sarcomatous portions supports this notion. As with GBM, cerebral gliosarcomas rarely metastasize and are usually unifocal. To our knowledge, this is the third reported case of multifocal gliosarcoma with intramedullary spinal metastases.13-14 Gliosarcoma is considered a GBM variant and is generally treated as such. The two largest series comparing the outcomes and prognosis of patients with gliosarcomas with GBM found no significant differences in time to progression, median overall survival, or actuarial survival when treated with radiotherapy and a nitrosurea-based chemotherapy regimen.4,15 Galanis et al15 reported a median time to progression and overall survival for 18 patients with gliosarcoma of 28.0 weeks and 35.1 weeks, respectively, compared with 16.7 weeks and 34.4 weeks for 18 patients with GBM after multiple patient characteristics were matched between the two groups. These reviews did not include patients treated with temozolomide. Although a rare occurrence, intramedullary spinal metastases should be sought in patients with gliosarcoma who present with back pain or demonstrate lower motor neuron weakness. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. Meis JM, Martz KL, Nelson JS: Mixed glioblastoma multiforme and sarcoma: A clinicopathologic study of 6 radiation therapy oncology group cases. Cancer 67:2342-2349, 1991[CrossRef][Medline] 2. Stroebe H: Uber Entstehung und Bau der Gehirngliome [in German]. Beitr Pathol Anat Allg Pathol 18:405-486, 1895 3. Feigin IM, Gross SW: Sarcoma arising in glioblastoma of the brain. Am J Pathol 31:6333-6353, 1955 4. Meis JM, Ho KL, Nelson JS: Gliosarcoma: A histologic and immunohistochemical reaffirmation. Mod Pathol 3:19-24, 1990[Medline] 5. Grant JW, Steart PZ, Aguzzi A, et al: Gliosarcoma: An immunohistochemical study. Acta Neuropathol 79:305-309, 1989[CrossRef][Medline] 6. Hayashi K, Ohara N, Jeon HJ, et al: Gliosarcoma with features of chondroblastic osteosarcoma. Cancer 67:2342-2349, 1991[CrossRef][Medline] 7. Panayiotides JG, Arapantoni-Dadioti P, Lekka-Katsoulic J, et al: Gliosarcoma with features of chondroblastic osteosarcoma. Cancer 72:850-855, 1993[CrossRef][Medline] 8. Vlodavsky E, Konstantinesku M, Soustiel JF: Gliosarcoma with liposarcomatous differentiation: The new member of the lipid-containing brain tumors family. Arch Pathol Lab Med 130:381-384, 2006[Medline] 9. Marucci G, Hadjmohammadi N, Cenni P, et al: Malignant glial tumor with skeletal muscle differentiation: Description of a case. Pathologica 92:198-203, 2000[Medline] 10. Perry JR, Ang LC, Bilbao JM, et al: Clinicopathologic features of primary and postirradiation cerebral gliosarcoma. Cancer 75:2910-2918, 1995[CrossRef][Medline] 11. Reis RM, Konu-Lebleblicinglu D, Lopes JM, et al: Genetic profile of gliosarcomas. Am J Path 156:425-432, 2000 12. Ozolek JA, Finkelstein SD, Couce ME: Gliosarcoma with epithelial differentiation: Immunohistochemical and molecular characterization: A case report and review of the literature. Mod Pathol 17:739-745, 2004[CrossRef][Medline] 13. Witwer BP, Salamat MS, Resnick DK: Gliosarcoma metastatic to the cervical spinal cord: Case report and review of the literature. Surg Neurol 54:373-378, 2000[CrossRef][Medline] 14. Pakos EE, Goussia AC, Zina VP, et al: Multi-focal gliosarcoma: A case report and review of the literature. J Neurooncol 74:301-304, 2005 15. Galanis E, Buckner JC, Dinapoli RP, et al: Clinical outcome of gliosarcoma compared with glioblastoma multiforme: North Central Cancer Treatment Group results. J Neurosurg 89:425-430, 1998[Medline]
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Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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