Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

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].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Oct 06, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Travis WD, Churg A, Aubry MC, et al. Mesenchymal tumours. In: Tumours of the LUng, Pleura, Thus and Heart, Travis WD, Brambilla E, Muller-Hermelink HK, and Harris CC (Eds), IARC Press, Lyon 2004. p.142.
  2. Fletcher CD, Bridge JA, Lee JC. Extrapleural solitary fibrous tumor. In: WHO Classification of Tumours of Soft Tissue and Bone, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), IARC Press, Lyon 2013. p.80.
  3. Klemperer P, Rabin CB. Primary neoplasms of the pleura: a report of five cases. Arch Pathol 1931; 11:385.
  5. Fletcher CDM, Bridge JA, Lee, J-C. Extrapleural solitary fibrous tumor. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th, Fletcher CDM, Bridge, JA, Hogendoorn CW, Mertens F (Eds), International Agency for Research on Cancer, Lyon 2013.
  6. Travis WD, Churg A, Aubry MC, Ordonez NG. Mesenchymal Tumours. In: Pathology and Genetics of Tumours of the Lung, Pleura, Thymus, and Heart, 1st, Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC (Eds), International Agency for Research on Cancer, Lyon 2004. p.141.
  7. Thompson LDR, Fanburg-Smith JC, Wenig BM. Borderline and low malignant potential tumours of soft tissues.. In: WHO Classification of Tumours: Pathology and Genetics of HEad and Neck Tumours, 1st, Barnes L, Eveson JW, Reichart P, Sidransky D (Eds), International Agency for Research on Cancer, Lyon 2005. p.43.
  8. Gold JS, Antonescu CR, Hajdu C, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer 2002; 94:1057.
  9. Chick JF, Chauhan NR, Madan R. Solitary fibrous tumors of the thorax: nomenclature, epidemiology, radiologic and pathologic findings, differential diagnoses, and management. AJR Am J Roentgenol 2013; 200:W238.
  10. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981; 47:2678.
  11. Sung SH, Chang JW, Kim J, et al. Solitary fibrous tumors of the pleura: surgical outcome and clinical course. Ann Thorac Surg 2005; 79:303.
  12. Cardillo G, Lococo F, Carleo F, Martelli M. Solitary fibrous tumors of the pleura. Curr Opin Pulm Med 2012; 18:339.
  13. Lu C, Ji Y, Shan F, et al. Solitary fibrous tumor of the pleura: an analysis of 13 cases. World J Surg 2008; 32:1663.
  14. Tapias LF, Mercier O, Ghigna MR, et al. Validation of a scoring system to predict recurrence of resected solitary fibrous tumors of the pleura. Chest 2015; 147:216.
  15. Meittinen Markku. Chapter 12: Solitary fibrous tumor, hemaogiopericytoma, and related tumors.. In: Modern Soft Tissue Pathology: Tumours and Non-Neoplastic Conditions, Miettinen, Markku (Eds), Cambridge University Press, New York 2010. p.335.
  16. Moran CA, Ishak KG, Goodman ZD. Solitary fibrous tumor of the liver: a clinicopathologic and immunohistochemical study of nine cases. Ann Diagn Pathol 1998; 2:19.
  17. Demicco EG, Park MS, Araujo DM, et al. Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. Mod Pathol 2012; 25:1298.
  18. Espat NJ, Lewis JJ, Leung D, et al. Conventional hemangiopericytoma: modern analysis of outcome. Cancer 2002; 95:1746.
  19. Hasegawa T, Matsuno Y, Shimoda T, et al. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol 1999; 30:1464.
  20. van Houdt WJ, Westerveld CM, Vrijenhoek JE, et al. Prognosis of solitary fibrous tumors: a multicenter study. Ann Surg Oncol 2013; 20:4090.
  21. Brunnemann RB, Ro JY, Ordonez NG, et al. Extrapleural solitary fibrous tumor: a clinicopathologic study of 24 cases. Mod Pathol 1999; 12:1034.
  22. Daigeler A, Lehnhardt M, Langer S, et al. Clinicopathological findings in a case series of extrathoracic solitary fibrous tumors of soft tissues. BMC Surg 2006; 6:10.
  23. Erdag G, Qureshi HS, Patterson JW, Wick MR. Solitary fibrous tumors of the skin: a clinicopathologic study of 10 cases and review of the literature. J Cutan Pathol 2007; 34:844.
  24. Lococo F, Cesario A, Cardillo G, et al. Malignant solitary fibrous tumors of the pleura: retrospective review of a multicenter series. J Thorac Oncol 2012; 7:1698.
  25. Lahon B, Mercier O, Fadel E, et al. Solitary fibrous tumor of the pleura: outcomes of 157 complete resections in a single center. Ann Thorac Surg 2012; 94:394.
  26. Tapias LF, Mino-Kenudson M, Lee H, et al. Risk factor analysis for the recurrence of resected solitary fibrous tumours of the pleura: a 33-year experience and proposal for a scoring system. Eur J Cardiothorac Surg 2013; 44:111.
  27. de Perrot M, Kurt AM, Robert JH, et al. Clinical behavior of solitary fibrous tumors of the pleura. Ann Thorac Surg 1999; 67:1456.
  28. Bernardini FP, de Conciliis C, Schneider S, et al. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology 2003; 110:1442.
  29. Shanbhogue AK, Prasad SR, Takahashi N, et al. Somatic and visceral solitary fibrous tumors in the abdomen and pelvis: cross-sectional imaging spectrum. Radiographics 2011; 31:393.
  30. Jang JG, Chung JH, Hong KS, et al. A case of solitary fibrous pleura tumor associated with severe hypoglycemia: doege-potter syndrome. Tuberc Respir Dis (Seoul) 2015; 78:120.
  31. Tay CK, Teoh HL, Su S. A common problem in the elderly with an uncommon cause: hypoglycaemia secondary to the Doege-Potter syndrome. BMJ Case Rep 2015; 2015.
  32. Meng W, Zhu HH, Li H, et al. Solitary fibrous tumors of the pleura with Doege-Potter syndrome: a case report and three-decade review of the literature. BMC Res Notes 2014; 7:515.
  33. Fung EC, Crook MA. Doege-Potter syndrome and 'big-IGF2': a rare cause of hypoglycaemia. Ann Clin Biochem 2011; 48:95.
  34. Herrmann BL, Saller B, Kiess W, et al. Primary malignant fibrous histiocytoma of the lung: IGF-II producing tumor induces fasting hypoglycemia. Exp Clin Endocrinol Diabetes 2000; 108:515.
  35. Tominaga N, Kawarasaki C, Kanemoto K, et al. Recurrent solitary fibrous tumor of the pleura with malignant transformation and non-islet cell tumor-induced hypoglycemia due to paraneoplastic overexpression and secretion of high-molecular-weight insulin-like growth factor II. Intern Med 2012; 51:3267.
  36. Cardillo G, Facciolo F, Cavazzana AO, et al. Localized (solitary) fibrous tumors of the pleura: an analysis of 55 patients. Ann Thorac Surg 2000; 70:1808.
  37. Rena O, Filosso PL, Papalia E, et al. Solitary fibrous tumour of the pleura: surgical treatment. Eur J Cardiothorac Surg 2001; 19:185.
  38. Magdeleinat P, Alifano M, Petino A, et al. Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome. Eur J Cardiothorac Surg 2002; 21:1087.
  39. Norton SA, Clark SC, Sheehan AL, et al. Solitary fibrous tumour of the diaphragm. J Cardiovasc Surg (Torino) 1997; 38:685.
  40. Versluis PJ, Lamers RJ. Localized pleural fibroma: radiological features. Eur J Radiol 1994; 18:124.
  41. Harris GN, Rozenshtein A, Schiff MJ. Benign fibrous mesothelioma of the pleura: MR imaging findings. AJR Am J Roentgenol 1995; 165:1143.
  42. Lee KS, Im JG, Choe KO, et al. CT findings in benign fibrous mesothelioma of the pleura: pathologic correlation in nine patients. AJR Am J Roentgenol 1992; 158:983.
  43. Stark P. [Fibrous pleural mesothelioma (pleura fibroma) -- a contribution to the differential diagnosis of pleural tumours (author's transl)]. Rofo 1981; 134:614.
  44. Desser TS, Stark P. Pictorial essay: solitary fibrous tumor of the pleura. J Thorac Imaging 1998; 13:27.
  45. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. Am J Surg Pathol 1989; 13:640.
  46. Okike N, Bernatz PE, Woolner LB. Localized mesothelioma of the pleura: benign and malignant variants. J Thorac Cardiovasc Surg 1978; 75:363.
  47. Saifuddin A, Da Costa P, Chalmers AG, et al. Primary malignant localized fibrous tumours of the pleura: clinical, radiological and pathological features. Clin Radiol 1992; 45:13.
  48. Yousem SA, Flynn SD. Intrapulmonary localized fibrous tumor. Intraparenchymal so-called localized fibrous mesothelioma. Am J Clin Pathol 1988; 89:365.
  49. Spizarny DL, Gross BH, Shepard JA. CT findings in localized fibrous mesothelioma of the pleural fissure. J Comput Assist Tomogr 1986; 10:942.
  50. Rosenkrantz AB, Hindman N, Melamed J. Imaging appearance of solitary fibrous tumor of the abdominopelvic cavity. J Comput Assist Tomogr 2010; 34:201.
  51. Bowe SN, Wakely PE Jr, Ozer E. Head and neck solitary fibrous tumors: diagnostic and therapeutic challenges. Laryngoscope 2012; 122:1748.
  52. Ganly I, Patel SG, Stambuk HE, et al. Solitary fibrous tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg 2006; 132:517.
  53. Liu Y, Li K, Shi H, Tao X. Solitary fibrous tumours in the extracranial head and neck region: correlation of CT and MR features with pathologic findings. Radiol Med 2014; 119:910.
  54. Le CP, Jones S, Valenzuela AA. Orbital solitary fibrous tumor: a case series with review of the literature. Orbit 2014; 33:145.
  55. Blandamura S, Alaggio R, Bettini G, et al. Four cases of solitary fibrous tumour of the eye and orbit: one with sarcomatous transformation after radiotherapy and one in a 5-year-old child's eyelid. J Clin Pathol 2014; 67:263.
  56. Mosquera JM, Fletcher CD. Expanding the spectrum of malignant progression in solitary fibrous tumors: a study of 8 cases with a discrete anaplastic component--is this dedifferentiated SFT? Am J Surg Pathol 2009; 33:1314.
  57. Nielsen GP, Dickersin GR, Provenzal JM, Rosenberg AE. Lipomatous hemangiopericytoma. A histologic, ultrastructural and immunohistochemical study of a unique variant of hemangiopericytoma. Am J Surg Pathol 1995; 19:748.
  58. Lee JC, Fletcher CD. Malignant fat-forming solitary fibrous tumor (so-called "lipomatous hemangiopericytoma"): clinicopathologic analysis of 14 cases. Am J Surg Pathol 2011; 35:1177.
  59. de Perrot M, Fischer S, Bründler MA, et al. Solitary fibrous tumors of the pleura. Ann Thorac Surg 2002; 74:285.
  60. Schweizer L, Koelsche C, Sahm F, et al. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol 2013; 125:651.
  61. Doyle LA, Vivero M, Fletcher CD, et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol 2014; 27:390.
  62. Koelsche C, Schweizer L, Renner M, et al. Nuclear relocation of STAT6 reliably predicts NAB2-STAT6 fusion for the diagnosis of solitary fibrous tumour. Histopathology 2014; 65:613.
  63. Yoshida A, Tsuta K, Ohno M, et al. STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol 2014; 38:552.
  64. Demicco EG, Harms PW, Patel RM, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol 2015; 143:672.
  65. Doyle LA, Tao D, Mariño-Enríquez A. STAT6 is amplified in a subset of dedifferentiated liposarcoma. Mod Pathol 2014; 27:1231.
  66. Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet 2013; 45:180.
  67. Chmielecki J, Crago AM, Rosenberg M, et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet 2013; 45:131.
  68. Mohajeri A, Tayebwa J, Collin A, et al. Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. Genes Chromosomes Cancer 2013; 52:873.
  69. Barthelmeß S, Geddert H, Boltze C, et al. Solitary fibrous tumors/hemangiopericytomas with different variants of the NAB2-STAT6 gene fusion are characterized by specific histomorphology and distinct clinicopathological features. Am J Pathol 2014; 184:1209.
  70. Schirosi L, Lantuejoul S, Cavazza A, et al. Pleuro-pulmonary solitary fibrous tumors: a clinicopathologic, immunohistochemical, and molecular study of 88 cases confirming the prognostic value of de Perrot staging system and p53 expression, and evaluating the role of c-kit, BRAF, PDGFRs (alpha/beta), c-met, and EGFR. Am J Surg Pathol 2008; 32:1627.
  71. Tai HC, Chuang IC, Chen TC, et al. NAB2-STAT6 fusion types account for clinicopathological variations in solitary fibrous tumors. Mod Pathol 2015; 28:1324.
  72. Huang SC, Li CF, Kao YC, et al. The clinicopathological significance of NAB2-STAT6 gene fusions in 52 cases of intrathoracic solitary fibrous tumors. Cancer Med 2016; 5:159.
  73. Bahrami A, Lee S, Schaefer IM, et al. TERT promoter mutations and prognosis in solitary fibrous tumor. Mod Pathol 2016; 29:1511.
  74. Liau JY, Lee JC, Tsai JH, et al. Comprehensive screening of alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas. Mod Pathol 2015; 28:1545.
  75. Koelsche C, Renner M, Johann P, et al. Differential nuclear ATRX expression in sarcomas. Histopathology 2016; 68:738.
  76. Killela PJ, Reitman ZJ, Jiao Y, et al. TERT promoter mutations occur frequently in gliomas and a subset of tumors derived from cells with low rates of self-renewal. Proc Natl Acad Sci U S A 2013; 110:6021.
  77. Koelsche C, Sahm F, Capper D, et al. Distribution of TERT promoter mutations in pediatric and adult tumors of the nervous system. Acta Neuropathol 2013; 126:907.
  78. Koelsche C, Renner M, Hartmann W, et al. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities. J Exp Clin Cancer Res 2014; 33:33.
  79. Akaike K, Toda-Ishii M, Suehara Y, et al. TERT promoter mutations are a rare event in gastrointestinal stromal tumors. Springerplus 2015; 4:836.
  80. Walker CM, Takasugi JE, Chung JH, et al. Tumorlike conditions of the pleura. Radiographics 2012; 32:971.
  81. de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD. Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. Mod Pathol 1999; 12:463.
  82. Cranshaw IM, Gikas PD, Fisher C, et al. Clinical outcomes of extra-thoracic solitary fibrous tumours. Eur J Surg Oncol 2009; 35:994.
  83. Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol 1998; 22:1501.
  84. Gronchi A, Miceli R, Allard MA, et al. Personalizing the approach to retroperitoneal soft tissue sarcoma: histology-specific patterns of failure and postrelapse outcome after primary extended resection. Ann Surg Oncol 2015; 22:1447.
  85. Salas S, Resseguier N, Blay JY, et al. Prediction of local and metastatic recurrence in solitary fibrous tumor: construction of a risk calculator in a multicenter cohort from the French Sarcoma Group (FSG) database. Ann Oncol 2017; 28:1979.
  86. Wilky BA, Montgomery EA, Guzzetta AA, et al. Extrathoracic location and "borderline" histology are associated with recurrence of solitary fibrous tumors after surgical resection. Ann Surg Oncol 2013; 20:4080.
  87. Akaike K, Kurisaki-Arakawa A, Hara K, et al. Distinct clinicopathological features of NAB2-STAT6 fusion gene variants in solitary fibrous tumor with emphasis on the acquisition of highly malignant potential. Hum Pathol 2015; 46:347.
  88. Baldi GG, Stacchiotti S, Mauro V, et al. Solitary fibrous tumor of all sites: outcome of late recurrences in 14 patients. Clin Sarcoma Res 2013; 3:4.
  89. Constantinidou A, Jones RL, Olmos D, et al. Conventional anthracycline-based chemotherapy has limited efficacy in solitary fibrous tumour. Acta Oncol 2012; 51:550.
  90. Park CK, Lee DH, Park JY, et al. Multiple recurrent malignant solitary fibrous tumors: long-term follow-up of 24 years. Ann Thorac Surg 2011; 91:1285.
  91. Mohamed H, Mandal AK. Natural history of multifocal solitary fibrous tumors of the pleura: a 25-year follow-up report. J Natl Med Assoc 2004; 96:659.
  92. Chang YL, Lee YC, Wu CT. Thoracic solitary fibrous tumor: clinical and pathological diversity. Lung Cancer 1999; 23:53.
  93. Yokoi T, Tsuzuki T, Yatabe Y, et al. Solitary fibrous tumour: significance of p53 and CD34 immunoreactivity in its malignant transformation. Histopathology 1998; 32:423.
  94. Herawi M, Epstein JI. Solitary fibrous tumor on needle biopsy and transurethral resection of the prostate: a clinicopathologic study of 13 cases. Am J Surg Pathol 2007; 31:870.
  95. Nielsen GP, O'Connell JX, Dickersin GR, Rosenberg AE. Solitary fibrous tumor of soft tissue: a report of 15 cases, including 5 malignant examples with light microscopic, immunohistochemical, and ultrastructural data. Mod Pathol 1997; 10:1028.
  96. Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol 1995; 26:440.
  97. Brozzetti S, D'Andrea N, Limiti MR, et al. Clinical behavior of solitary fibrous tumors of the pleura. An immunohistochemical study. Anticancer Res 2000; 20:4701.
  98. Hasegawa T, Matsuno Y, Shimoda T, et al. Frequent expression of bcl-2 protein in solitary fibrous tumors. Jpn J Clin Oncol 1998; 28:86.
  99. Suter M, Gebhard S, Boumghar M, et al. Localized fibrous tumours of the pleura: 15 new cases and review of the literature. Eur J Cardiothorac Surg 1998; 14:453.
  100. Demicco EG, Wagner MJ, Maki RG, et al. Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model. Mod Pathol 2017; 30:1433.
  101. Nomori H, Horio H, Fuyuno G, Morinaga S. Contacting metastasis of a fibrous tumor of the pleura. Eur J Cardiothorac Surg 1997; 12:928.
  102. Spitz FR, Bouvet M, Pisters PW, et al. Hemangiopericytoma: a 20-year single-institution experience. Ann Surg Oncol 1998; 5:350.
  103. Wang X, Qian J, Bi Y, et al. Malignant transformation of orbital solitary fibrous tumor. Int Ophthalmol 2013; 33:299.
  104. Yang XJ, Zheng JW, Ye WM, et al. Malignant solitary fibrous tumors of the head and neck: a clinicopathological study of nine consecutive patients. Oral Oncol 2009; 45:678.
  105. Stacchiotti S, Libertini M, Negri T, et al. Response to chemotherapy of solitary fibrous tumour: a retrospective study. Eur J Cancer 2013; 49:2376.
  106. Park MS, Ravi V, Conley A, et al. The role of chemotherapy in advanced solitary fibrous tumors: a retrospective analysis. Clin Sarcoma Res 2013; 3:7.
  107. Levard A, Derbel O, Méeus P, et al. Outcome of patients with advanced solitary fibrous tumors: the Centre Léon Bérard experience. BMC Cancer 2013; 13:109.
  108. Stacchiotti S, Tortoreto M, Bozzi F, et al. Dacarbazine in solitary fibrous tumor: a case series analysis and preclinical evidence vis-a-vis temozolomide and antiangiogenics. Clin Cancer Res 2013; 19:5192.
  109. Park MS, Patel SR, Ludwig JA, et al. Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor. Cancer 2011; 117:4939.
  110. Choi H, Charnsangavej C, Faria SC, et al. Correlation of computed tomography and positron emission tomography in patients with metastatic gastrointestinal stromal tumor treated at a single institution with imatinib mesylate: proposal of new computed tomography response criteria. J Clin Oncol 2007; 25:1753.
  111. Stacchiotti S, Negri T, Libertini M, et al. Sunitinib malate in solitary fibrous tumor (SFT). Ann Oncol 2012; 23:3171.
  112. George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 2009; 27:3154.
  113. van Krimpen C, Schoemaker RG, Cleutjens JP, et al. Angiotensin I converting enzyme inhibitors and cardiac remodeling. Basic Res Cardiol 1991; 86 Suppl 1:149.
  114. Domont J, Massard C, Lassau N, et al. Hemangiopericytoma and antiangiogenic therapy: clinical benefit of antiangiogenic therapy (sorafenib and sunitinib) in relapsed malignant haemangioperyctoma /solitary fibrous tumour. Invest New Drugs 2010; 28:199.
  115. Stacchiotti S, Tortoreto M, Baldi GG, et al. Preclinical and clinical evidence of activity of pazopanib in solitary fibrous tumour. Eur J Cancer 2014; 50:3021.
  116. Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 2009; 45:228.
  117. Therasse P, Arbuck SG, Eisenhauer EA, et al. New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst 2000; 92:205.
  118. Benjamin RS, Choi H, Macapinlac HA, et al. We should desist using RECIST, at least in GIST. J Clin Oncol 2007; 25:1760.
  119. Broto JM, Stacchiotti S, Lopez-Pousa A, et al. Multi-institutional European single-arm phase II trial of pazopanib in advanced malignant/dedifferentiated solitary fibrous tumors (SFT): A collaborative Spanish (GEIS), Italian (ISG), and French (FSG) sarcoma groups study (abstract). J Clin Oncol 35, 2017 (suppl; abstr 11003). Abstract availabe online at http://abstracts.asco.org/199/AbstView_199_182533.html (Accessed on July 13, 2017).
  120. Valentin T, Fournier C, Penel N, et al. Sorafenib in patients with progressive malignant solitary fibrous tumors: a subgroup analysis from a phase II study of the French Sarcoma Group (GSF/GETO). Invest New Drugs 2013; 31:1626.
  121. von Mehren M, Rankin C, Goldblum JR, et al. Phase 2 Southwest Oncology Group-directed intergroup trial (S0505) of sorafenib in advanced soft tissue sarcomas. Cancer 2012; 118:770.
Topic Outline