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Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases

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Abstract

We report two cases of sclerosing epithelioid fibrosarcoma occurring in the deep soft tissue of the thigh, confirmed by molecular analysis and associated with bone metastases in the lumbar vertebrae and the iliac wing at the time of diagnosis. Synchronous bone metastases of sclerosing epithelioid fibrosarcoma are extremely difficult to diagnose because clinical and radiological features are not specific. In addition, the range of differential diagnoses is very wide, including metastatic carcinoma and osteosarcoma. At present, all but three published cases of sclerosing epithelioid fibrosarcoma with bone metastases showed bone metastases during follow-up. We confirm in our two cases that the distinct pattern of immunohistochemical staining for MUC4, associated with the absence of staining for both SATB2, a marker of osteoblastic differentiation, and pan-cytokeratin, allows differentiating between sclerosing epithelioid fibrosarcoma and metastatic carcinoma or osteosarcoma.

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References

  1. Kindblom LG, Mertens F, Coindre JM, Hornick JL, Meis JM (2013) Sclerosing epithelioid fibrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F (eds) World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon, pp. 97–98

    Google Scholar 

  2. Meis-Kindblom JM, Enzinger FM (1995) Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol 19:979–993

    Article  CAS  PubMed  Google Scholar 

  3. Lau PP, Lui PC, Lau GT, Yau DT, Cheung ET, Chan JK (2013) EWSR1-CREB3L1 gene fusion: a novel alternative molecular aberration of low-grade fibromyxoid sarcoma. Am J Surg Pathol 37(5):734–738. doi:10.1097/PAS.0b013e31827560f8

    Article  PubMed  Google Scholar 

  4. Guillou L, Benhattar J, Gengler C, Gallagher G, Ranchère-Vince D, Collin F, Terrier P, Terrier-Lacombe MJ, Leroux A, Marquès B, Aubain Somerhausen Nde S, Keslair F, Pedeutour F, Coindre JM (2007) Translocation-positive low-grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Group. Am J Surg Pathol 31(9):1387–1402

    Article  PubMed  Google Scholar 

  5. Wojcik JB, Bellizzi AM, Dal Cin P, Bredella MA, Fletcher CD, Hornicek FJ, Deshpande V, Hornick JL, Nielsen GP (2014) Primary sclerosing epithelioid fibrosarcoma of bone: analysis of a series. Am J Surg Pathol 38:1538–1544. doi:10.1097/PAS.0000000000000265

    Article  PubMed  Google Scholar 

  6. Antonescu CR, Rosenblum MK, Pereira P, Nascimento AG, Woodruff JM (2001) Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor. Am J Surg Pathol 25:699–709

    Article  CAS  PubMed  Google Scholar 

  7. Ossendorf C, Studer GM, Bode B, Fuchs B (2008) Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review. Clin Orthop Relat Res 466:1485–1491. doi:10.1007/s11999-008-0205-8

    Article  PubMed Central  PubMed  Google Scholar 

  8. Wang WL, Evans HL, Meis JM, Liegl-Atzwanger B, Bovee JV, Goldblum JR, Billings SD, Rubin BP, López-Terrada D, Lazar AJ (2012) FUS rearrangements are rare in ‘pure’ sclerosing epithelioid fibrosarcoma. Mod Pathol 25:846–853. doi:10.1038/modpathol.2011.214

    Article  PubMed  Google Scholar 

  9. Bilsky MH, Schefler AC, Sandberg DI, Dunkel IJ, Rosenblum MK (2000) Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases. Neurosurgery 47:956–959

    Article  CAS  PubMed  Google Scholar 

  10. Grunewald TG, von Luettichau I, Weirich G, Wawer A, Behrends U, Prodinger PM, Jundt G, Bielack SS, Gradinger R, Burdach S (2010) Sclerosing epithelioid fibrosarcoma of the bone: a case report of high resistance to chemotherapy and a survey of the literature. Sarcoma 2010:431627. doi:10.1155/2010/431627

    Article  PubMed Central  PubMed  Google Scholar 

  11. Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, Hornick JL (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. doi:10.1097/PAS.0b013e3182562bf8

    Article  PubMed  Google Scholar 

  12. Arbajian E, Puls F, Magnusson L, Thway K, Fisher C, Sumathi VP, Tayebwa J, Nord KH, Kindblom LG, Mertens F (2014) Recurrent EWSR1-CREB3L1 gene fusions in sclerosing epithelioid fibrosarcoma. Am J Surg Pathol 38:801–808. doi:10.1097/PAS.0000000000000158

    Article  PubMed  Google Scholar 

  13. Prieto-Granada C, Zhang L, Chen HW, Sung YS, Agaram NP, Jungbluth AA, Antonescu CR (2015) A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: a pathologic and molecular study of 18 cases. Genes Chromosom Cancer 54:28–38. doi:10.1002/gcc.22215

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  14. Ohlmann CH, Brecht IB, Junker K, van der Zee JA, Nistor A, Bohle RM, Stöckle M, Metzler M, Hartmann A, Agaimy A (2015) Sclerosing epithelioid fibrosarcoma of the kidney: clinicopathologic and molecular study of a rare neoplasm at a novel location. Ann Diagn Pathol. doi:10.1016/j.anndiagpath.2015.04.005

    PubMed  Google Scholar 

  15. Hanson IM, Pearson JM, Eyden BP, Slawik S, Harris M (2001) Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma. J Clin Pathol 54:721–723

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Watanabe K, Suzuki T (2004) Epithelioid fibrosarcoma of the ovary. Virchows Arch 445:410–413

    Article  PubMed  Google Scholar 

  17. Righi A, Gambarotti M, Longo S, Benini S, Gamberi G, Cocchi S, Vanel D, Picci P, Bertoni F, Simoni A, Franchi A, Dei Tos AP (2015) Small cell osteosarcoma: clinicopathologic, immunohistochemical, and molecular analysis of 36 cases. Am J Surg Pathol 39(5):691–699. doi:10.1097/PAS.0000000000000412

    Article  PubMed  Google Scholar 

  18. Christensen DR, Ramsamooj R, Gilbert TJ (1997) Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skelet Radiol 26:619–621

    Article  CAS  Google Scholar 

  19. Conner JR, Hornick JL (2013) SATB2 is a novel marker of osteoblastic differentiation in bone and soft tissue tumours. Histopathology 63(1):36–49. doi:10.1111/his.12138

    Article  PubMed  Google Scholar 

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Conflict of interest

The authors declare that they have no competing interests.

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Authors and Affiliations

  1. Department of Pathology, Rizzoli Institute, Via di Barbiano 1/10, 40136, Bologna, Italy

    A. Righi, M. Gambarotti, S. Benini, G. Gamberi, S. Cocchi, P. Picci, D. Vanel & A. P. Dei Tos

  2. Oncologic Department, Rizzoli Orthopaedic Institute, Bologna, Italy

    M. Manfrini & R. Casadei

  3. Department of Pathology, Treviso Regional Hospital, Treviso, Italy

    A. P. Dei Tos

  4. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy

    G. Gamberi

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  1. A. Righi
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  2. M. Gambarotti
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  3. M. Manfrini
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  4. S. Benini
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  5. G. Gamberi
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  6. S. Cocchi
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  7. R. Casadei
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  8. P. Picci
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  9. D. Vanel
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  10. A. P. Dei Tos
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Correspondence to A. Righi.

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Righi, A., Gambarotti, M., Manfrini, M. et al. Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases. Virchows Arch 467, 339–344 (2015). https://doi.org/10.1007/s00428-015-1810-z

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  • DOI: https://doi.org/10.1007/s00428-015-1810-z

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