Hemangiopericytoma and antiangiogenic therapy: clinical benefit of antiangiogenic therapy (sorafenib and sunitinib) in relapsed Malignant Haemangioperyctoma /Solitary Fibrous Tumour

Abstract

Soft tissue sarcomas are a highly heterogeneous group of tumors. Recently, different molecular abnormalities were identified and in some cases corresponding molecular targeted therapies led to significant clinical benefits. Malignant tumors of blood vessels are classified as sarcomas and are uncommon. They encompass angiosarcoma, hemangioendotheliomas, and hemangiopericytomas. The term hemangiopericytoma (HMP) was coined in 1942 by Stout and Murray for soft tissue tumours thought to originate from cell encircling blood vessels (pericytes)[1]. Over the years the concept of HMP has gradually evolved. It was namely shown that this term has been used for a wide variety of benign and malignant soft tissue tumours which share common morphologic features but correspond to different entities. It is now obvious that some conventional HMP correspond in fact to the spectrum of solitary fibrous tumour (SFT). SFT of the soft tissue (or extrapleural SFT) have an unpredictable behaviour, 10 to 15 % of them showing an aggressive evolution with either local recurrences and/or metastases. SFT located in the mediastinum, peritoneum, retroperitoneum and pelvis tend to be more aggressive than those in other locations. The most common sites of metastasis are the lung, bone, and liver. There is no standard treatment for patients with advanced disease with the exception of anthracyclins largely used in first chemotherapy line. Here, we describe a patient with a hemangioperictoma who achieved a partial response while on sorafenib therapy, and a second case with the same clinical outcome while on sunitinib. First patient was a 59 year-old female was referred to our center for a multi-relapsed hemangiopericytoma. The tumor was diagnosed in 1982 as a pre-sacral hemangiopericytoma and surgery was performed. The patient experienced multiple relapses: lung metastases in 1994 and pancreatic metastases in 1996 were completely resected. In 2000, she relapsed in the lung for the third time and first-line chemotherapy was administered with an anthracyclin-based regimen (cisplatin, epirubicin and ifosfamide). After 6 cycles, the CT-scan showed stable disease and surgery was performed. In 2003, due to further pulmonary progression, the patient was included in a phase II with trabectedin (Yondelis®) but therapy was discontinued because of hepatic toxicity. Invest New Drugs (2010) 28:199–202 DOI 10.1007/s10637-009-9249-1