Dr. Koehan is very excited about this research project because, as of today, she said there are no publications of microarray analysis (a process that will attempt to help define regulatory genes involved) of hemanogiopericytoma. She also stated that none of this would be possible without the generous philanthropic support of the Alan Rosenthal Fund, and everyone at Memorial Sloan-Kettering Cancer Center could not agree with her more!

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Principal Investigator: ML Keohan, MD, Attending Physician, Melanoma/Sarcoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center

Title of Project: Molecular determinants of Hemangiopericytoma/Solitary Fibrous Tumor development and progression

Background: Hemangiopericytoma (HPC), a highly vascular tumor, described by Stout and Murray in 1942 as a distinct soft tissue tumor. The abundant proliferation of small blood vessels surrounded by a connective tissue sheath in a branching pattern serves as a morphologic marker of this entity. These tumors are now classified as a variant of solitary fibrous tumors (SFT), and represent less than 10% of all soft tissue sarcomas (STS). Predicting clinical behavior remains a challenge with little correlation between morphology and outcome. Due to the relative rarity, there is little attention devoted toward the study of this disease entity.

Objectives: The initial goal of this pilot project is to identify the molecular signatures influencing the development, growth and recurrence/metastatic potential of HPC/SFT.
We will retrieve, from the MSKCC sarcoma tumor bank, fresh frozen tissue of resected HPC/SFT tumor samples. Resected specimens of primary tumors from CNS, lung and liver will be identified. If samples of recurrent or metastatic disease are available these specimens will be retrieved as well. RNA will be prepared from available material for micro array analysis. Following the identification of candidate genes, further analysis of potential targets will be pursued. Potential future aims include the establishment of cell lines to study in vitro the effects of novel targeted inhibitors.Vascular endothelial growth factor (VEGF) and receptor tyrosine kinase VEGFR-1 (FLT 1), as well as Insulin growth factor receptor (IGF-1R) are present in HPC/SFT. The overexpression of growth factors and respective receptors may be a driving force in the development of theses tumors. While these growth factors and receptors have been implicated in a number of tumor agents, targeting these receptors have not, as of yet, demonstrated robust activity in treatment of sarcomas, which suggests that combination strategies may be required.