Lung

, Volume 190, Issue 6, pp 691–695

Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome

  • Priska Leisibach
  • Walter Weder
  • Alex Soltermann
  • Wolfgang Jungraithmayr
Article

DOI: 10.1007/s00408-012-9401-0

Cite this article as:
Leisibach, P., Weder, W., Soltermann, A. et al. Lung (2012) 190: 691. doi:10.1007/s00408-012-9401-0

Abstract

Sclerosing epithelioid fibrosarcoma (SEF) is a rare neoplasm arising mostly in limbs and limb girdles, with a high rate of recurrence and a strong tendency to metastasize. This case study is of a 54-year-old woman with an asymptomatic mass in the upper lobe of the left lung detected by PET-CT when staging for Lynch syndrome-associated colon carcinoma. Histology of the resected tumor showed epithelioid cells arranged in nests, partly restiform within a zone of sclerosing fibrosis. Immunohistochemistry was positive for vimentin, epithelial membrane antigen, and S100-protein. Eight months after lung resection, the patient was diagnosed for basal cell carcinoma on her back. At the end of a two year follow-up period, she developed metastases to the mediastinum, vertebrae, ribs, femurs, pelvic bones, kidneys, and one lung, histologically all related to SEF. Here we report the first case of a SEF primarily arising from the lung and discuss it in the context of the current literature.

Keywords

Lung Sclerosing epithelioid fibrosarcoma Lynch syndrome Hereditary nonpolyposis colorectal cancer 

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Priska Leisibach
    • 1
  • Walter Weder
    • 1
  • Alex Soltermann
    • 2
  • Wolfgang Jungraithmayr
    • 1
  1. 1.Division of Thoracic SurgeryUniversity Hospital ZurichZurichSwitzerland
  2. 2.Institute for Surgical PathologyUniversity Hospital ZurichZurichSwitzerland