Clinical Orthopaedics and Related Research

, Volume 466, Issue 6, pp 1485–1491 | Cite as

Sclerosing Epithelioid Fibrosarcoma: Case Presentation and a Systematic Review

  • Christian Ossendorf
  • Gabriela M. Studer
  • Beata Bode
  • Bruno Fuchs
Survey Tumor


In sclerosing epithelioid fibrosarcoma (SEF), a rare variant of low-grade fibrosarcoma, treatment results and therapeutic options are poorly characterized. We systematically analyzed the data of all 89 patients (43 female, 46 male; mean age, 47 years [range, 14–87 years]) reported in the literature concerning clinical presentation, histopathology, differential diagnosis, treatment, survival rates, and prognosis, and we present an additional case. Information detailing treatment, disease control, and followup was available in 60 (67%), 75 (84%), and 68 patients (76%), respectively. Case history was variable with one-third of patients reporting a painful, enlarging mass. Ten patients (13%) presented with metastases, 23 (31%) had metastases develop after diagnosis, and 28 (37%) had local recurrence. Low cellularity, mild pleomorphy, and sclerotic hyaline matrix of SEF suggest a benign clinical behavior, and cell morphology allows for the wide differential diagnosis of benign, pseudosarcomatous, and malignant proliferations. In addition to surgery, 11 patients (15%) had chemotherapy, 22 (29%) had postoperative radiation therapy, and three (4%) had a combination of both. Twenty-three patients (34%) died from their disease after a mean of 46 months, 24 (35%) were alive with disease, and 20 (31%) were alive without evidence of disease. Patients with SEF of the head and neck had the worst prognosis.

Level of Evidence: Level III, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.


Myositis Ossificans Granulocytic Sarcoma Average Tumor Size Intralesional Excision Sclerosing Epithelioid Fibrosarcoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



We thank Felix Regenfelder, MD, for advice and assistance in performing statistics.

Supplementary material

11999_2008_205_MOESM1_ESM.xls (53 kb)
ESM 1 (XLS 53 kb)


  1. 1.
    Abdulkader I, Cameselle-Teijeiro J, Fraga M, Caparrini A, Forteza J. Sclerosing epithelioid fibrosarcoma primary of the bone. Int J Surg Pathol. 2002;10:227–230.PubMedCrossRefGoogle Scholar
  2. 2.
    Antonescu CR, Baren A. Spectrum of low-grade fibrosarcomas: a comparative ultrastructural analysis of low-grade myxofibrosarcoma and fibromyxoid sarcoma. Ultrastruct Pathol. 2004;28:321–332.PubMedCrossRefGoogle Scholar
  3. 3.
    Antonescu CR, Erlandson RA. Fibrosarcoma mimicking plasmacytoma or carcinoma: an ultrastructural study of 4 cases. Ultrastruct Pathol. 2001;25:31–37.PubMedCrossRefGoogle Scholar
  4. 4.
    Arnould L, Jouannelle C, Mege F, Maillefert F, Fargeot P, Devillebichot C, Collin F. [Sclerosing epithelioid fibrosarcoma: a fibrosarcoma with a very long course.]. Ann Pathol. 2000;20:154–157.PubMedGoogle Scholar
  5. 5.
    Arya M, Garcia-Montes F, Patel HR, Emberton M, Mundy AR. A rare tumour in the pelvis presenting with lower urinary symptoms: ‘sclerosing epithelioid fibrosarcoma’. Eur J Surg Oncol. 2001;27:121–122.PubMedCrossRefGoogle Scholar
  6. 6.
    Battiata AP, Casler J. Sclerosing epithelioid fibrosarcoma: a case report. Ann Otol Rhinol Laryngol. 2005;114:87–89.PubMedGoogle Scholar
  7. 7.
    Bezic J, Tomic S, Glavina-Durdov M, Alfirevic D, Samija I, Krizanac S. Sclerosing epitheloid fibrosarcoma: a report of two cases. Pathologica. 2004;96:433–435.PubMedGoogle Scholar
  8. 8.
    Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, Ricci F, Weber K, Furlong MA, Fisher C, Montgomery E. Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol. 2005;29:653–659.PubMedCrossRefGoogle Scholar
  9. 9.
    Bilsky MH, Schefler AC, Sandberg DI, Dunkel IJ, Rosenblum MK. Sclerosing epithelioid fibrosarcomas involving the neuraxis: report of three cases. Neurosurgery. 2000;47:956–959; discussion 959–960.PubMedCrossRefGoogle Scholar
  10. 10.
    Boudova L, Michal M, Kinkor Z, Bencik V, Husek K, Mukensnabl P, Rousarova M. [Sclerosing epithelioid fibrosarcoma.] Cesk Patol. 2001;37:158–162.PubMedGoogle Scholar
  11. 11.
    Chang E, Lee A, Lee E, Shin O, Kang C, Kim JM, Chu YC. Hyalinizing spindle cell tumor with giant rosettes with pulmonary metastasis after a long hiatus: a case report. J Korean Med Sci. 2004;19:619–623.PubMedCrossRefGoogle Scholar
  12. 12.
    Choi HY, Kwon NS, Lee SJ, Lee DK, Park K, Lee CW, Jo MK. Sclerosing epithelioid fibrosarcoma of the kidney. Korean K Urol. 2007;48:986–989.Google Scholar
  13. 13.
    Chow LT, Lui YH, Kumta SM, Allen PW. Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature. J Clin Pathol. 2004;57:90–94.PubMedCrossRefGoogle Scholar
  14. 14.
    Christensen DR, Ramsamooj R, Gilbert TJ. Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging. Skeletal Radiol. 1997;26:619–621.PubMedCrossRefGoogle Scholar
  15. 15.
    Decroisette C, Melloni B, Moldovan D, Gazaille V, Fermeaux V, Bertin F, Bonnaud F. [Pleural metastases of sclerosing epithelioid fibrosarcoma] [in French]. Rev Pneumol Clin. 2006;62:179–182.PubMedGoogle Scholar
  16. 16.
    Donner LR, Clawson K, Dobin SM. Sclerosing epithelioid fibrosarcoma: a cytogenetic, immunohistochemical, and ultrastructural study of an unusual histological variant. Cancer Genet Cytogenet. 2000;119:127–131.PubMedCrossRefGoogle Scholar
  17. 17.
    Evans HL. Low-grade fibromyxoid sarcoma: a report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clin Pathol. 1987;88:615–619.PubMedGoogle Scholar
  18. 18.
    Eyden BP, Manson C, Banerjee SS, Roberts IS, Harris M. Sclerosing epithelioid fibrosarcoma: a study of five cases emphasizing diagnostic criteria. Histopathology. 1998;33:354–360.PubMedCrossRefGoogle Scholar
  19. 19.
    Farinha P, Oliveira P, Soares J. Metastasizing hyalinizing spindle cell tumour with giant rosettes: report of a case with long survival. Histopathology. 2000;36:92–93.PubMedCrossRefGoogle Scholar
  20. 20.
    Folpe AL, Lane KL, Paull G, Weiss SW. 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. 2000;24:1353–1360.PubMedCrossRefGoogle Scholar
  21. 21.
    Franchi A, Massi D, Santucci M. Hyalinizing spindle cell tumor with giant rosettes and low-grade fibromyxoid sarcoma: an immunohistochemical and ultrastructural comparative investigation. Ultrastruct Pathol. 2003;27:349–355.PubMedCrossRefGoogle Scholar
  22. 22.
    Frattini JC, Sosa JA, Carmack S, Robert ME. Sclerosing epithelioid fibrosarcoma of the cecum: a radiation-associated tumor in a previously unreported site. Arch Pathol Lab Med. 2007;131:1825–1828.PubMedGoogle Scholar
  23. 23.
    Garcia de León MA, Torres Coscoyuela M, Hernández Sánchez L. Sclerosing epithelioid fibrosarcoma: a complicated diagnosis. Rev Orthop Traumatol. 2001;45:419–422.Google Scholar
  24. 24.
    Genevay M, Coindre JM, Guillou L. [Recent entities in soft tissue tumor pathology. Part 2][in French]. Ann Pathol. 2003;23:135–148.PubMedGoogle Scholar
  25. 25.
    Gisselsson D, Andreasson P, Meis-Kindblom JM, Kindblom LG, Mertens F, Mandahl N. Amplification of 12q13 and 12q15 sequences in a sclerosing epithelioid fibrosarcoma. Cancer Genet Cytogenet. 1998;107:102–106.PubMedCrossRefGoogle Scholar
  26. 26.
    Goodlad JR, Mentzel T, Fletcher CD. Low grade fibromyxoid sarcoma: clinicopathological analysis of eleven new cases in support of a distinct entity. Histopathology. 1995;26:229–237.PubMedCrossRefGoogle Scholar
  27. 27.
    Guillou L, Benhattar J, Gengler C, Gallagher G, Ranchere-Vince D, Collin F, Terrier P, Terrier-Lacombe MJ, Leroux A, Marques B, Aubain Somerhausen Nde S, Keslair F, Pedeutour F, Coindre JM. 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. 2007;31:1387–1402.PubMedCrossRefGoogle Scholar
  28. 28.
    Hansen T, Katenkamp K, Brodhun M, Katenkamp D. Low-grade fibrosarcoma: report on 39 not otherwise specified cases and comparison with defined low-grade fibrosarcoma types. Histopathology. 2006;49:152–160.PubMedCrossRefGoogle Scholar
  29. 29.
    Hanson IM, Pearson JM, Eyden BP, Slawik S, Harris M. Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma. J Clin Pathol. 2001;54:721–723.PubMedGoogle Scholar
  30. 30.
    Hindermann W, Katenkamp D. [Sclerosing epithelioid fibrosarcoma][in German]. Pathologe. 2003;24:103–108.PubMedGoogle Scholar
  31. 31.
    Hu WW, Lai RQ, Wang J, Feng XD, Li GM. [Sclerosing epithelioid fibrosarcoma: a clinicopathologic study of eight cases][in Chinese]. Zhonghua Bing Li Xue Za Zhi. 2004;33:337–341.PubMedGoogle Scholar
  32. 32.
    Jiao J, Rhodes DG, Burgess DJ. Multiple emulsion stability: pressure balance and interfacial film strength. J Colloid Interface Sci. 2002;250:444–450.PubMedCrossRefGoogle Scholar
  33. 33.
    Karaki S, Mochida J, Lee YH, Nishimura K, Tsutsumi Y. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy. Pathol Int. 1999;49:820–825.PubMedCrossRefGoogle Scholar
  34. 34.
    Lane KL, Shannon RJ, Weiss SW. Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma. Am J Surg Pathol. 1997;21:1481–1488.PubMedCrossRefGoogle Scholar
  35. 35.
    Magro G, Fraggetta F, Manusia M, Mingrino A. Hyalinizing spindle cell tumor with giant rosettes: a previously undescribed lesion of the lung. Am J Surg Pathol. 1998;22:1431–1433.PubMedCrossRefGoogle Scholar
  36. 36.
    Meis-Kindblom JM, Kindblom LG, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979–993.PubMedCrossRefGoogle Scholar
  37. 37.
    Miettinen M. From morphological to molecular diagnosis of soft tissue tumors. Adv Exp Med Biol. 2006;587:99–113.PubMedCrossRefGoogle Scholar
  38. 38.
    O’Sullivan MJ, Sirgi KE, Dehner LP. Low-grade fibrosarcoma (hyalinizing spindle cell tumor with giant rosettes) with pulmonary metastases at presentation: case report and review of the literature. Int J Surg Pathol. 2002;10:211–216.PubMedCrossRefGoogle Scholar
  39. 39.
    Oda Y, Takahira T, Kawaguchi K, Yamamoto H, Tamiya S, Matsuda S, Tanaka K, Iwamoto Y, Tsuneyoshi M. Low-grade fibromyxoid sarcoma versus low-grade myxofibrosarcoma in the extremities and trunk: a comparison of clinicopathological and immunohistochemical features. Histopathology. 2004;45:29–38.PubMedCrossRefGoogle Scholar
  40. 40.
    Ogose A, Kawashima H, Umezu H, Hotta T, Gu W, Yamagiwa H, Ito T, Tohyama T, Nishijima H, Endo N. Sclerosing epithelioid fibrosarcoma with der(10)t(10;17)(p11;q11). Cancer Genet Cytogenet. 2004;152:136–140.PubMedCrossRefGoogle Scholar
  41. 41.
    Perigny M, Dion N, Couture C, Lagace R. [Low grade fibromyxoid sarcoma: a clinico-pathologic analysis of 7 cases] [in French]. Ann Pathol. 2006;26:419–425.PubMedCrossRefGoogle Scholar
  42. 42.
    Reid R, Barrett A, Hamblen DL. Sclerosing epithelioid fibrosarcoma. Histopathology. 1996;28:451–455.PubMedCrossRefGoogle Scholar
  43. 43.
    Watanabe K, Suzuki T. Epithelioid fibrosarcoma of the ovary. Virchows Arch. 2004;445:410–413.PubMedCrossRefGoogle Scholar
  44. 44.
    Weiss SW, Goldblum JR. Enzinger and Weiss’ Soft Tissue Tumors. 4th ed. St Louis MO: Mosby; 2001.Google Scholar
  45. 45.
    Woodruff JM, Antonescu CR, Erlandson RA, Boland PJ. Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized. Am J Surg Pathol. 1999;23:1423–1428.PubMedCrossRefGoogle Scholar

Copyright information

© The Association of Bone and Joint Surgeons 2008

Authors and Affiliations

  • Christian Ossendorf
    • 1
  • Gabriela M. Studer
    • 2
  • Beata Bode
    • 3
  • Bruno Fuchs
    • 1
  1. 1.Department of OrthopedicsBalgrist University Hospital, University of ZurichZurichSwitzerland
  2. 2.Department of Radiation OncologyUniversity of ZurichZurichSwitzerland
  3. 3.Institute of Surgical PathologyUniversity of ZurichZurichSwitzerland

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