Header

UZH-Logo

Maintenance Infos

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


Leisibach, Priska; Weder, Walter; Soltermann, Alex; Jungraithmayr, Wolfgang (2012). Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome. Lung, 190(6):691-695.

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.

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.

Statistics

Citations

Dimensions.ai Metrics
4 citations in Web of Science®
5 citations in Scopus®
2 citations in Microsoft Academic
Google Scholar™

Altmetrics

Downloads

1 download since deposited on 17 Oct 2012
0 downloads since 12 months
Detailed statistics

Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Thoracic Surgery
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2012
Deposited On:17 Oct 2012 13:57
Last Modified:21 Sep 2018 03:32
Publisher:Springer
ISSN:0341-2040
OA Status:Green
Publisher DOI:https://doi.org/10.1007/s00408-012-9401-0
PubMed ID:22766913

Download

Download PDF  'Primary Sclerosing Epithelioid Fibrosarcoma of the Lung in a Patient with Lynch Syndrome'.
Preview
Content: Published Version
Language: English
Filetype: PDF (Nationallizenz 142-005)
Size: 807kB