Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2007, Vol 23, Num, 1     (Pages: 005-015)

Primary sarcomas of the central nervous system: UCSF experience (1985-2005)

Tarık TİHAN 1, Ann GRİFFİN 2, Sema ÖZUYSAL 3

1 University of California, San Francisco, Department of Pathology, Neuropathology Unit, SAN FRANSİSCO
2 University of California, San Francisco, Cancer Center Tumor Registry, SAN FRANSİSCO
3 Uludağ University, School of Medicine, Department of Pathology, SAN FRANSİSCO

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Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS) are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.

Keywords : Sarcoma, primary, central nervous system, hemangiopericytoma, chondrosarcoma