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Volume 25, Number 2, 2020

Sacral solitary fibrous tumour: surgery and hadrontherapy, a combined treatment strategy

Cesare Zoia, Francesco Lombardi, Maria Rosaria Fiore, Andrea Montalbetti, Alberto Iannalfi, Mattia Sansone, Daniele Bongetta, Francesca Valvo, Mattia Del Maestro, Sabino Luzzi, Renato Juan Galzio

Summary:

Hemangiopericytoma (HPC), recently associated by the WHO classification with Solitary Fibrous Tumor, is a kind of soft tissue sarcoma. It originates in Zimmerman’s pericytes,1 i.e. spindle cells wrapping the capillaries and postcapillary venules that provide mechanical and functional support to the endotheliocytes. Because of its origins, HPC can occur wherever endothelial tissue is present, the most common sites being the lower extremities, retroperitoneum and head-and-neck region.2 It accounts for less than 1% of all vascular tumors and 5% of all sarcomatous tumors. Not only can this tumor have a locally aggressive behavior, especially inside the nervous system, it can also recur locally and metastasize through the bloodstream, mainly in the lungs and bone.3 HPC can affect equally both sexes, mainly in adults in the fourth through sixth decades of life. It can manifest itself in two forms: soft and osseous with a slight predominance among males. The cellular architecture is characterized by tumor cells arranged in sheets and fascicles with staghorn-shaped vessels and dense intercellular reticulin meshwork (particularly visible at the silver staining) without evidence of epithelial membrane antigen (in contrast to meningiomas). At immunohistochemical study the tumor is positive for Vimentin, CD34 but negative to Factor VIII, S-100 protein, Actin, CKP, EMA and Desmin3. The presence of necrosis, hemorrhage, anaplasia and a high mitotic activity (more than five mitoses per ten microscopic fields), are a poor prognostic factor.2 Surgical resection is the treatment of choice, with the use of chemotherapy reserved for metastatic or non-resectable lesions, albeit with no standardized regimens. Radiotheraphy, too, may play a role, as a sole management for lesions deemed not viable for surgery or on remnant/recurrencies.4

Signature: Rep Pract Oncol Radiother, 2020; 25(2) : 241-244


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Indexed in: EMBASE®, the Excerpta Medica database, the Elsevier BIOBASE (Current Awareness in Biological Sciences) and in the Index Copernicus.

http://www.sciencedirect.com/science/journal/15071367/19/2