Abstract
Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon fibroblastic neoplasm with many morphologic mimics. Solitary fibrous tumor is a more common fibroblastic neoplasm, but the two rarely enter the same differential diagnosis. However, here, we report two unusual cases of LGFMS, containing dilated, hemangiopericytoma-like blood vessels, which prompted diagnostic considerations of solitary fibrous tumor. Both cases presented were confirmed to harbor FUS gene rearrangement, thereby confirming a diagnosis of LGFMS. One case is that of an 18-year-old male with a left forearm mass, and the other a 50-year-old man with a left popliteal mass. While both cases show some histologic features of LGFMS, the non-classical, dilated blood vessel pattern seen here may serve as a diagnostic pitfall, as LGFMS normally exhibits fine, curvilinear blood vessels. To our knowledge, there is only one other report of LGFMS displaying such hemangiopericytoma-like blood vessels. In summary, when encountering a bland spindle cell neoplasm with classic hemangiopericytoma-like blood vessels, it is prudent to consider a diagnosis of LGFMS besides solitary fibrous tumor—particularly in the absence of CD34 immunoreactivity as it may be a rare, mimicking variant of LGFMS.
Similar content being viewed by others
References
Evans HL (1987) Low-grade fibromyxoid sarcoma: a report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clin Pathol 88:615–619
Graadt van Roggen JF, Hogendoorn PCW, Fletcher CDM (1999) Myxoid tumors of soft tissue. Histopathol 35:291–312
Vernon SE, Bejarano P (2006) Low-grade fibromyxoid sarcoma: a brief review. Arch Pathol Lab Med 130:1358–1360
Billings S, Giblen G, Fanburg-Smith J (2005) Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population. Am J Surg Pathol 29:204–210
Lane KL, Shannon RJ, Weiss SW (1997) Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma. Am J Surg Pathol 21:1481–1488
Doyle LA, Moller E, Dal Cin P et al (2011) MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. Am J Surg Pathol 35:733–741
Folpe AL, Lane KL, Paull G et al (2000) Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol 24:1353–1360
Reid R, deSilva C, Paterson L et al (2003) Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation. Am J Surg Pathol 27:1229–1236
Devaney DM, Dervan P, O’Neill S et al (1990) Low-grade fibromyxoid sarcoma. Histopathol 17:463–465
Evans HL (1993) Low-grade fibromyxoid sarcoma: a report of 12 cases. Am J Surg Pathol 17:595–600
Goodlad JR, Mentzel T, Fletcher CD (1995) Low-grade fibromyxoid sarcoma: clinicopathological analysis of eleven new cases in support of a distinct entity. Histopathol 26:229–237
Langman G (2011) Solitary fibrous tumor: a pathological enigma and clinical dilemma. J Thorac Dis 3:86–87
Folpe AL, Hornick JL, Mertens F (2013) Low-grade fibromyxoid sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (eds) WHO classification of tumors of soft tissue and bone, 4th edn. IARC press, Lyon, pp 95–96
Schweizer L, Koelsche C, Sahm F et al (2013) Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. Acta Neuropathol 125:651–658
Robinson DR, Wu YM, Kalyana-Sundaram S et al (2013) Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet 45:180–185
Chmielecki J, Crago AM, Rosenberg M et al (2013) Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat Genet 45:131–132
Moller E, Hornick JL, Magnusson L et al (2011) FUS-CREB3L2/L1-positive sarcomas show a specific gene expression profile with upregulation of CD24 and FOXL1. Clin Cancer Res 17:2646–2656
Doyle LA, Wang WL, Dal Cin P et al (2012) MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement. Am J Surg Pathol 36:1444–1451
Fletcher CDM, Bridge JA, Hogendoorn PCW et al (2013) WHO classification of tumors of soft tissue and bone, 4th edn. IARC press, Lyon
Doyle LA, Vivero M, Fletcher CDM et al (2014) Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol 27:390–395
Yoshida A, Tsuta K, Ohno M et al (2014) STAT6 immunohistochemistry is helpful in the diagnosis of solitary fibrous tumors. Am J Surg Pathol 38:552–559
Conflict of interest
We declare that we have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Papp, S., Dickson, B.C. & Chetty, R. Low-grade fibromyxoid sarcoma mimicking solitary fibrous tumor: a report of two cases. Virchows Arch 466, 223–228 (2015). https://doi.org/10.1007/s00428-014-1684-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00428-014-1684-5