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Solitary fibrous tumor

Elizabeth G Demicco, MD, PhD
Christian Meyer, MD, PhD
Section Editors
Robert Maki, MD, PhD
Thomas F DeLaney, MD
James R Jett, MD
Joseph S Friedberg, MD
Russell S Berman, MD
Raphael E Pollock, MD
Deputy Editors
Diane MF Savarese, MD
Kathryn A Collins, MD, PhD, FACS


Solitary fibrous tumor (SFT) comprises a histologic spectrum of rarely metastasizing fibroblastic mesenchymal neoplasms that includes hemangiopericytoma [1,2]. Although they are commonly thought of as intrathoracic tumors, 50 to 70 percent of SFTs arise outside the thorax, including the central nervous system (CNS). The clinical manifestations, diagnosis, management, and prognosis of SFT at sites other than the CNS are reviewed here. SFTs/hemangiopericytomas arising within the CNS are discussed elsewhere. (See "Uncommon brain tumors", section on 'Solitary fibrous tumor/hemangiopericytoma'.)


SFT was first described in the pleura in 1931 [3] and historically recognized by several names, including benign mesothelioma, localized mesothelioma, solitary fibrous mesothelioma, pleural fibroma, submesothelial fibroma, subserosal fibroma, and localized fibrous tumor. SFT is now recognized to occur anywhere in the body, including soft tissue and viscera, albeit with a peculiar predilection for body cavity sites, including pleura, peritoneum, and meninges.

Hemangiopericytomas were first described in 1942 and initially thought to be a vascular neoplasm related to smooth muscle perivascular cells known as pericytes [4]. However, the diagnosis was largely descriptive, based upon a nonspecific, albeit characteristic, staghorn vascular pattern, and the term became a "wastebasket" diagnosis, which included a variety of unrelated benign and malignant entities. With the advent of diagnostic immunohistochemistry (IHC) and cytogenetic analysis, pathologists were able to exclude histologic mimics, and hemangiopericytoma became more accepted as a distinct entity, although largely a diagnosis of exclusion, until the recognition of phenotypic and behavioral overlap with SFT led pathologists to consider the two as one tumor type.

The discovery of a shared, recurrent, and thus far unique gene fusion in SFT and tumors histologically identified as hemangiopericytoma has confirmed the identical nature of these tumors. At present, the term SFT is preferred [5,6] and use of the term "hemangiopericytoma" is discouraged in clinical practice. However, some neuropathologists still prefer the term hemangiopericytoma in reference to meningeal tumors to emphasize their aggressive behavior compared with other SFT. (See "Uncommon brain tumors", section on 'Solitary fibrous tumor/hemangiopericytoma'.)

Sinonasal glomangiopericytoma, sometimes also called "sinonasal hemangiopericytoma," is a clinically, morphologically, and biologically distinct entity that differs from SFT and should not be confused with it [7].

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Literature review current through: Sep 2017. | This topic last updated: Oct 06, 2017.
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