A primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation.

2.50
Hdl Handle:
http://hdl.handle.net/10541/109362
Title:
A primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation.
Authors:
Wang, Guofeng; Eyden, Brian P
Abstract:
Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma, described initially by Meis-Kindblom et al. in 1995 (Meis-Kindblom JM, Kindblom L-G, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979-993): more than 80 cases have been documented clinicopathologically since. Bone is a rare primary site for SEF, with only 2 cases so far reported. This paper documents the detailed clinical, histological, immunohistochemical, and ultrastructural features of a case occurring in the pubic bone of a 57-year-old diabetic woman presenting with a history of pain and compromised mobility involving her hip. Radiology revealed a destructive lesion in the right pubic bone. The lesion was resected, and 7 months postoperatively it recurred. The patient died following metastases to multiple bony sites and liver, some 4 years after the onset of symptoms. Histologically, the tumor was consistent with SEF, although it showed some anomalous immunostaining, which, however, is typical of the tumor (for example, for S-100 protein and epithelial membrane antigen). By electron microscopy, some rough endoplasmic reticulum was present, but also tonofibrils and desmosomes. The overall features were of an SEF with the ultrastructural but incomplete immunohistochemical evidence for divergent epithelial differentiation. The differential diagnosis of this tumor is discussed.
Affiliation:
Department of Pathology, Second Affiliated Hospital of the Medical College, Zhejiang University, Hangzhou, China. wgf2202@yahoo.com.cn
Citation:
A primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation. 2010, 34 (2):99-104 Ultrastruct Pathol
Journal:
Ultrastructural Pathology
Issue Date:
Apr-2010
URI:
http://hdl.handle.net/10541/109362
DOI:
10.3109/01913121003605576
PubMed ID:
20192707
Type:
Article
Language:
en
ISSN:
1521-0758
Appears in Collections:
All Christie Publications

Full metadata record

DC FieldValue Language
dc.contributor.authorWang, Guofengen
dc.contributor.authorEyden, Brian Pen
dc.date.accessioned2010-08-10T10:03:51Z-
dc.date.available2010-08-10T10:03:51Z-
dc.date.issued2010-04-
dc.identifier.citationA primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation. 2010, 34 (2):99-104 Ultrastruct Patholen
dc.identifier.issn1521-0758-
dc.identifier.pmid20192707-
dc.identifier.doi10.3109/01913121003605576-
dc.identifier.urihttp://hdl.handle.net/10541/109362-
dc.description.abstractSclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma, described initially by Meis-Kindblom et al. in 1995 (Meis-Kindblom JM, Kindblom L-G, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979-993): more than 80 cases have been documented clinicopathologically since. Bone is a rare primary site for SEF, with only 2 cases so far reported. This paper documents the detailed clinical, histological, immunohistochemical, and ultrastructural features of a case occurring in the pubic bone of a 57-year-old diabetic woman presenting with a history of pain and compromised mobility involving her hip. Radiology revealed a destructive lesion in the right pubic bone. The lesion was resected, and 7 months postoperatively it recurred. The patient died following metastases to multiple bony sites and liver, some 4 years after the onset of symptoms. Histologically, the tumor was consistent with SEF, although it showed some anomalous immunostaining, which, however, is typical of the tumor (for example, for S-100 protein and epithelial membrane antigen). By electron microscopy, some rough endoplasmic reticulum was present, but also tonofibrils and desmosomes. The overall features were of an SEF with the ultrastructural but incomplete immunohistochemical evidence for divergent epithelial differentiation. The differential diagnosis of this tumor is discussed.en
dc.language.isoenen
dc.subjectBone Canceren
dc.subjectCancerous Cell Transformationen
dc.subjectBiological Tumour Markersen
dc.subject.meshBone Neoplasms-
dc.subject.meshCell Transformation, Neoplastic-
dc.subject.meshCytoplasmic Structures-
dc.subject.meshEpithelial Cells-
dc.subject.meshEpithelioid Cells-
dc.subject.meshFatal Outcome-
dc.subject.meshFemale-
dc.subject.meshFibrosarcoma-
dc.subject.meshHumans-
dc.subject.meshIlium-
dc.subject.meshMicroscopy, Electron, Transmission-
dc.subject.meshPubic Bone-
dc.subject.meshSacroiliac Joint-
dc.subject.meshSclerosis-
dc.subject.meshTomography, X-Ray Computed-
dc.subject.meshTumor Markers, Biological-
dc.titleA primary sclerosing epithelioid fibrosarcoma of the pubic bone, with evidence of divergent epithelial differentiation.en
dc.typeArticleen
dc.contributor.departmentDepartment of Pathology, Second Affiliated Hospital of the Medical College, Zhejiang University, Hangzhou, China. wgf2202@yahoo.com.cnen
dc.identifier.journalUltrastructural Pathologyen

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