Epithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions.

2.50
Hdl Handle:
http://hdl.handle.net/10541/69763
Title:
Epithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions.
Authors:
Eyden, Brian P; Wang, Guofeng; Yao, Li Fang
Abstract:
Epithelioid sarcoma is an uncommon but well-described malignancy, which is found predominantly in the soft tissues of the young and middle-aged, and which pursues an indolent to aggressive course. It shows a degree of both mesenchymal and epithelial differentiation. Myofibroblastic differentiation has been recorded in epithelioid sarcoma for some time, the evidence being based mainly on the presence of smooth-muscle-type myofilaments and, more recently, on alpha-smooth-muscle actin and muscle-specific actin immunostaining. Myofibroblastic differentiation based on the stricter criterion of the fibronectin fibril/fibronexus junction has not so far been demonstrated except for a single atypical case with spindle-cell morphology and a cytokeratin-negative immunophenotype. The authors describe a conventional epithelioid sarcoma showing myofibroblastic differentiation based on the presence of fibronectin fibrils and fibronexus junctions. The patient was a 35-year-old Chinese male with a tumor that initially developed on his left forefinger: it recurred, then metastasised first to the left arm and eventually to the scalp. Histologically, the tumors had the typical features of epithelioid sarcoma: by immunohistochemistry, cytokeratin, epithelial membrane antigen, and vimentin were positive. By electron microscopy, rough endoplasmic reticulum, intermediate filaments, peripheral myofilaments, and fibronexus junctions were observed. This is the first epithelioid sarcoma of conventional histological type to show myofibroblastic differentiation on the basis of the more stringent criterion of the fibronexus. The findings are discussed in relation to the unusually varied differentiation reported for this tumor.
Affiliation:
Department of Histopathology, The Christie NHS Foundation Trust, Manchester, UK.
Citation:
Epithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions., 33 (2):61-6 Ultrastruct Pathol
Journal:
Ultrastructural Pathology
Issue Date:
2009
URI:
http://hdl.handle.net/10541/69763
DOI:
10.1080/01913120802625798
PubMed ID:
19274582
Type:
Article
Language:
en
ISSN:
1521-0758
Appears in Collections:
All Christie Publications ; Pathology

Full metadata record

DC FieldValue Language
dc.contributor.authorEyden, Brian P-
dc.contributor.authorWang, Guofeng-
dc.contributor.authorYao, Li Fang-
dc.date.accessioned2009-06-05T10:13:14Z-
dc.date.available2009-06-05T10:13:14Z-
dc.date.issued2009-
dc.identifier.citationEpithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions., 33 (2):61-6 Ultrastruct Patholen
dc.identifier.issn1521-0758-
dc.identifier.pmid19274582-
dc.identifier.doi10.1080/01913120802625798-
dc.identifier.urihttp://hdl.handle.net/10541/69763-
dc.description.abstractEpithelioid sarcoma is an uncommon but well-described malignancy, which is found predominantly in the soft tissues of the young and middle-aged, and which pursues an indolent to aggressive course. It shows a degree of both mesenchymal and epithelial differentiation. Myofibroblastic differentiation has been recorded in epithelioid sarcoma for some time, the evidence being based mainly on the presence of smooth-muscle-type myofilaments and, more recently, on alpha-smooth-muscle actin and muscle-specific actin immunostaining. Myofibroblastic differentiation based on the stricter criterion of the fibronectin fibril/fibronexus junction has not so far been demonstrated except for a single atypical case with spindle-cell morphology and a cytokeratin-negative immunophenotype. The authors describe a conventional epithelioid sarcoma showing myofibroblastic differentiation based on the presence of fibronectin fibrils and fibronexus junctions. The patient was a 35-year-old Chinese male with a tumor that initially developed on his left forefinger: it recurred, then metastasised first to the left arm and eventually to the scalp. Histologically, the tumors had the typical features of epithelioid sarcoma: by immunohistochemistry, cytokeratin, epithelial membrane antigen, and vimentin were positive. By electron microscopy, rough endoplasmic reticulum, intermediate filaments, peripheral myofilaments, and fibronexus junctions were observed. This is the first epithelioid sarcoma of conventional histological type to show myofibroblastic differentiation on the basis of the more stringent criterion of the fibronexus. The findings are discussed in relation to the unusually varied differentiation reported for this tumor.en
dc.language.isoenen
dc.subjectCancer Recurrenceen
dc.subjectSoft Tissue Sarcomaen
dc.subjectTumour Markersen
dc.subject.meshAdult-
dc.subject.meshCell Transformation, Neoplastic-
dc.subject.meshCytoplasm-
dc.subject.meshFibroblasts-
dc.subject.meshFibronectins-
dc.subject.meshFingers-
dc.subject.meshHumans-
dc.subject.meshIntercellular Junctions-
dc.subject.meshMale-
dc.subject.meshMicroscopy, Electron, Transmission-
dc.subject.meshMuscle Fibers, Skeletal-
dc.subject.meshNeoplasm Recurrence, Local-
dc.subject.meshSarcoma-
dc.subject.meshSoft Tissue Neoplasms-
dc.subject.meshTumor Markers, Biological-
dc.titleEpithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions.en
dc.typeArticleen
dc.contributor.departmentDepartment of Histopathology, The Christie NHS Foundation Trust, Manchester, UK.en
dc.identifier.journalUltrastructural Pathologyen
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