Clinical Orthopaedics and Related Research

, Volume 466, Issue 6, pp 1485–1491

Sclerosing Epithelioid Fibrosarcoma: Case Presentation and a Systematic Review

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

DOI: 10.1007/s11999-008-0205-8

Cite this article as:
Ossendorf, C., Studer, G.M., Bode, B. et al. Clin Orthop Relat Res (2008) 466: 1485. doi:10.1007/s11999-008-0205-8

Abstract

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.

Supplementary material

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

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