Skip to main content

Advertisement

Log in

STAT6 Reliably Distinguishes Solitary Fibrous Tumors from Myofibromas

  • Original Paper
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Solitary fibrous tumors (SFT) and myofibromas (MF) historically have belonged to the same morphologic spectrum and have been lumped together under the nonspecific umbrella term, “hemangiopericytoma” along with other pericytic/myoid tumors. While current evidence shows clear distinction between the two entities, they frequently remain in the same histopathologic differential diagnosis. This diagnostic dilemma especially is common for smaller incisional biopsies from the oral cavity. STAT6 immunohistochemistry (IHC) recently was established as a reliable method to detect solitary fibrous tumor; however, the literature is sparse regarding STAT6 reactivity in MFs. The authors report ten new cases of oral solitary fibrous tumor, discuss histopathologic similarities and differences between the two tumors, and list respective STAT6 IHC expressivity. After IRB approval, 10 cases diagnosed as SFT and 24 cases of MF were collected from the University of Florida Oral and Maxillofacial Pathology Biopsy Service between the years 1994 and 2016. The original hematoxylin and eosin slides and related IHC were reviewed. IHC with STAT6 antibody was performed on all 34 samples, and the findings were analyzed. All cases were from the oral cavity or perioral regions. 10/10 SFTs expressed STAT6 nuclear reactivity, while no cases of MF showed nuclear expression of STAT6. STAT6 is a dependable marker to differentiate SFTs from MFs.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology. 2006;48(1):63–74.

    Article  CAS  PubMed  Google Scholar 

  2. Klemperer P, Rabin CB. Primary neoplasm of the pleura: a report of 5 cases. Arch Pathol. 1931;11:28.

    Google Scholar 

  3. Stout AP, Murray MR. Hemangiopericytoma: a vascular tumor featuring zimmermann’s pericytes. Ann Surg. 1942;116(1):26–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Fletcher CDM, Bridge JA, Lee JC. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.

    Google Scholar 

  5. Chmielecki J, Crago AM, Rosenberg M, O’Connor R, Walker SR, Ambrogio L, Auclair D, McKenna A, Heinrich MC, Frank DA, Meyerson M. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet. 2013;45(2):131–2. doi:10.1038/ng.2522.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Mohajeri A, Tayebwa J, Collin A, Nilsson J, Magnusson L, von Steyern FV, Brosjö O, Domanski HA, Larsson O, Sciot R, Debiec-Rychter M, Hornick JL, Mandahl N, Nord KH, Mertens F. 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(10):873–86. doi:10.1002/gcc.22083.

    Article  CAS  PubMed  Google Scholar 

  7. Robinson DR, Wu YM, Kalyana-Sundaram S, Cao X, Lonigro RJ, Sung YS, Chen CL, Zhang L, Wang R, Su F, Iyer MK, Roychowdhury S, Siddiqui J, Pienta KJ, Kunju LP, Talpaz M, Mosquera JM, Singer S, Schuetze SM, Antonescu CR, Chinnaiyan AM. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013;45(2):180–5. doi:10.1038/ng.2509.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Demicco EG, Harms PW, Patel RM, Smith SC, Ingram D, Torres K, Carskadon SL, Camelo-Piragua S, McHugh JB, Siddiqui J, Palanisamy N, Lucas DR, Lazar AJ, Wang WL. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672–82. doi:10.1309/AJCPN25NJTOUNPNF.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Doyle LA, Vivero M, Fletcher CD, Mertens F, Hornick JL. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol. 2014;27(3):390–5. doi:10.1038/modpathol.2013.164.

    Article  CAS  PubMed  Google Scholar 

  10. Hornick JL. Novel uses of immunohistochemistry in the diagnosis and classification of soft tissue tumors. Mod Pathol. 2014;27(Suppl 1):S47–63. doi:10.1038/modpathol.2013.177.

    Article  CAS  PubMed  Google Scholar 

  11. Koelsche C, Schweizer L, Renner M, Warth A, Jones DT, Sahm F, Reuss DE, Capper D, Knösel T, Schulz B, Petersen I, Ulrich A, Renker EK, Lehner B, Pfister SM, Schirmacher P, von Deimling A, Mechtersheimer G. Nuclear relocation of STAT6 reliably predicts NAB2-STAT6 fusion for the diagnosis of solitary fibrous tumour. Histopathology. 2014;65(5):613–22. doi:10.1111/his.12431.

    Article  PubMed  Google Scholar 

  12. Vogels RJ, Vlenterie M, Versleijen-Jonkers YM, Ruijter E, Bekers EM, Verdijk MA, Link MM, Bonenkamp JJ, van der Graaf WT, Slootweg PJ, Suurmeijer AJ, Groenen PJ, Flucke U. Solitary fibrous tumor - clinicopathologic, immunohistochemical and molecular analysis of 28 cases. Diagn Pathol. 2014;9:224. doi:10.1186/s13000-014-0224-6.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Yoshida A, Tsuta K, Ohno M, Yoshida M, Narita Y, Kawai A, Asamura H, Kushima R. STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol. 2014;38(4):552–9. doi:10.1097/PAS.0000000000000137.

    Article  PubMed  Google Scholar 

  14. Smith MH, Reith JD, Cohen DM, Islam NM, Sibille KT, Bhattacharyya I. An update on myofibromas and myofibromatosis affecting the oral regions with report of 24 new cases. Oral Surg Oral Med Oral Pathol Oral Radiol. 2017. doi:10.1016/j.oooo.2017.03.051.

    Google Scholar 

  15. Fukunaga M, Naganuma H, Nikaido T, Harada T, Ushigome S. Extrapleural solitary fibrous tumor: a report of seven cases. Mod Pathol. 1997;10(5):443–50.

    CAS  PubMed  Google Scholar 

  16. Thway K, Ng W, Noujaim J, Jones RL, Fisher C. The current status of solitary fibrous tumor: diagnostic features, variants, and genetics. Int J Surg Pathol. 2016;24(4):281–92. doi:10.1177/1066896915627485.

    Article  CAS  PubMed  Google Scholar 

  17. Guillou L, Fletcher JA, Fletcher CDM, Mandahl N. WHO classification of tumours of soft tissue and bone. 3rd ed. Lyon: IARC Press; 2002.

    Google Scholar 

  18. Hasegawa T, Matsuno Y, Shimoda T, Hasegawa F, Sano T, Hirohashi S. Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior. Hum Pathol. 1999;30(12):1464–73.

    Article  CAS  PubMed  Google Scholar 

  19. 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(10):1028–37.

    CAS  PubMed  Google Scholar 

  20. Kiyohara T, Maruta N, Iino S, Ido H, Tokuriki A, Hasegawa M. CD34-positive infantile myofibromatosis: Case report and review of hemangiopericytoma-like pattern tumors. J Dermatol. 2016;43(9):1088–91. doi:10.1111/1346-8138.13400.

    Article  CAS  PubMed  Google Scholar 

  21. Han Y, Zhang Q, Yu X, Han X, Wang H, Xu Y, Qiu X, Jin F. Immunohistochemical detection of STAT6, CD34, CD99 and BCL-2 for diagnosing solitary fibrous tumors/hemangiopericytomas. Int J Clin Exp Pathol. 2015;8(10):13166–75.

    PubMed  PubMed Central  Google Scholar 

  22. Carlos R, de Andrade BA, Canedo NH, Abrahão AC, Agostini M, de Almeida OP, Romañach MJ. Clinicopathologic and immunohistochemical features of five new cases of solitary fibrous tumor of the oral cavity. Oral Surg Oral Med Oral Pathol Oral Radiol. 2016;121(4):390–5. doi:10.1016/j.oooo.2015.11.001.

    Article  PubMed  Google Scholar 

  23. O’Regan EM, Vanguri V, Allen CM, Eversole LR, Wright JM, Woo SB. Solitary fibrous tumor of the oral cavity: clinicopathologic and immunohistochemical study of 21 cases. Head Neck Pathol. 2009;3(2):106–15. doi:10.1007/s12105-009-0111-8.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Shine N, nor nurul Khasri M, Fitzgibbon J, O’Leary G. Solitary fibrous tumor of the floor of the mouth: case report and review of the literature. Ear Nose Throat J. 2006;85(7):437–9.

    PubMed  Google Scholar 

  25. Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg. 1988;23(4):315–8.

    Article  CAS  PubMed  Google Scholar 

  26. Ahluwalia N, Attia R, Green A, Cane P, and Routledge T. Doege–Potter syndrome. Ann R Coll Surg Engl. 2015; 97(7):e105–e107. doi:10.1308/rcsann.2015.0023.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Agaimy A, Bieg M, Michal M, Geddert H, Märkl B, Seitz J, Moskalev EA, Schlesner M, Metzler M, Hartmann A, Wiemann S, Michal M, Mentzel T, Haller F. Recurrent somatic PDGFRB mutations in sporadic infantile/solitary adult myofibromas but not in angioleiomyomas and myopericytomas. Am J Surg Pathol. 2017;41(2):195–203. doi:10.1097/PAS.0000000000000752.

    Article  PubMed  Google Scholar 

  28. Hung YP, Fletcher CDM. Myopericytomatosis: clinicopathologic analysis of 11 cases with molecular identification of recurrent PDGFRB alterations in myopericytomatosis and myopericytoma. Am J Surg Pathol. 2017. doi:10.1097/PAS.0000000000000862.

    Google Scholar 

  29. Arts FA, Chand D, Pecquet C, Velghe AI, Constantinescu S, Hallberg B, Demoulin JB. PDGFRB mutants found in patients with familial infantile myofibromatosis or overgrowth syndrome are oncogenic and sensitive to imatinib. Oncogene. 2016;35(25):3239–48. doi:10.1038/onc.2015.383.

    Article  CAS  PubMed  Google Scholar 

  30. Rechsteiner M, Wild P, Kiessling MK, Bohnert A, Zhong Q, Stahel RA, Moch H, Curioni-Fontecedro A. A novel germline mutation of PDGFR-β might be associated with clinical response of colorectal cancer to regorafenib. Ann Oncol. 2015;26(1):246–8. doi:10.1093/annonc/mdu471.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Molly Housley Smith.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Smith, M.H., Islam, N.M., Bhattacharyya, I. et al. STAT6 Reliably Distinguishes Solitary Fibrous Tumors from Myofibromas. Head and Neck Pathol 12, 110–117 (2018). https://doi.org/10.1007/s12105-017-0836-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-017-0836-8

Keywords

Navigation