Clinical Report 2008

Alan Rosenthal Research Fund In Hemangiopericytoma Memorial Sloan-Kettering Cancer Center 2008

Hemangiopericytoma is a rare mesenchymal tumor associated with blood vessel walls. It comprises less than 5 percent of all soft-tissue sarcomas. There are approximately 70 different histologic sub types of soft-tissue sarcomas.

Surgery remains the mainstay of treatment for hemangiopericytoma. The role of adjuvant chemotherapy remains controversial for patients with soft-tissue sarcoma in general and is less defined in patients with hemangiopericytoma. It remains difficult to predict the prognosis and clinical behavior of these tumors. In general, hemangiopericytomas tend to recur and metastasize. Common sites of involvement include bone, liver, lung and central nervous system.

Hemangiopericytomas are highly vascularized tumors and express platelet derived growth factor receptor (PDGFR) and vascular endothelial growth factor receptor (VEGFR). Treatment of multifocal or unresectable disease with agents inhibiting angiogenesis is an ongoing clinical strategy. Hence, the rationale for treatment with agents such as sunitinib, an inhibitor or receptors for VEGF and PDGR, fibroblast growth factor and stem cell factor. These tumors also express insulin growth factors (IGFs) and insulin growth factor receptors (IGF-R). IGFs stimulate tumor cells to proliferate and inhibition of this pathway is also being investigated as a therapeutic approach to treatment.

The rarity of sarcomas and the diverse histologic subtypes and biologic behavior contribute to the challenges of predicting behavior and prognosis. The Alan Rosenthal Research Fund will support clinical research projects in hemangiopericytoma that include the identification of critical genes and regulatory pathways required for the growth of soft-tissue sarcoma.

Memorial Sloan-Kettering has a long and distinguished history in sarcoma diagnosis and treatment with a vibrant research program that holds great promise for continued advances. We hope you share our sense of optimism about the opportunities for significant breakthroughs and will agree to provide support at this time of special opportunity.