Markers and Immunoprofile of Fibroblastic, Myofibroblastic, and Fibrohistiocytic Tumors

, Hans Guski2 and Glen Kristiansen3

Carl-Thiem-Klinikum, Institut für Pathologie, Cottbus, Germany

Vivantes Klinikum Neukölln, Institut für Pathologie, Berlin, Germany

Universität Bonn, UKB, Institut für Pathologie, Bonn, Germany


Diagnostic Antibody Panel for Fibroblastic, Myofibroblastic, and Fibrohistiocytic Tumors

Vimentin, actin, desmin, CD34, and CD68


Expression pattern: cytoplasmic

Main diagnostic use

Expression in other tumors

Expression in normal cells

Mesenchymal tumors

Metaplastic carcinoma, endometrioid carcinoma, carcinomas of salivary glands, follicular thyroid carcinoma, clear cell renal cell carcinoma, hepatocellular carcinoma, poorly differentiated carcinomas of different origin

Cells of mesenchymal origin: fibrocytes and fibroblasts, lipocytes, smooth muscle cells, endothelium, macrophages, myoepithelial cells, thyroid follicular cells, adrenal cortex, renal tubules, mesangial cells of renal glomerulus, pancreatic acinar cells, melanocytes, lymphocytes, astrocytes, Schwann cells

Positive control: appendix

Diagnostic Approach

Vimentin is a 57-kDa protein, a member of the type III family of intermediate filaments, expressed in all mesenchymal cells forming an important part of the cytoskeleton of these cells. The type III family of intermediate filaments includes vimentin, desmin, GFAP, and peripherin. Vimentin is generally expressed in all primitive cells in the early embryogenesis and be replaced by other intermediate filaments during maturation and differentiation.

Diagnostic Pitfalls

The use of vimentin as a single marker is of limited diagnostic value as the co-expression of vimentin with other different cytokeratins has been demonstrated in many types of epithelial cells and tumors such as carcinomas of the lung, salivary glands, liver and biliary tract, thyroid gland, adrenal cortex, kidney, endometrium, gonads and meningioma (Fig. 22.1). Generally, poorly differentiated carcinomas may acquire vimentin expression with loss of keratins and finally resulting a sarcomatoid phenotype. For diagnostic purposes, vimentin can be only used as a part of diagnostic antibody panel.


Fig. 22.1
Neoplastic glands of endometrioid carcinoma exhibiting strong vimentin expression


STAT-6 is a member of the STAT family of cytoplasmic transcription factors involved in the modulation of signal transmission between DNA promoters and cell receptors. The inv. (12)(q13;q13) is a chromosomal aberration characteristic for solitary fibrous tumor generating the NAB2-STAT6 fusion transcript causing the overexpression of the STAT-6 protein, which is a characteristic immunohistochemical marker for solitary fibrous tumor (Fig. 22.2). This chromosomal abnormality affects also the promoter of the ERG-1 gene causing the overexpression of the ERG-1 transcription factor, which can be a further marker for this tumor identity [1, 2].


Fig. 22.2
Strong nuclear STAT-6 expression in the cells of solitary fibrous tumor

Diagnostic Pitfalls

The overexpression of STAT-6 is also found in a limited number of other mesenchymal tumors including meningeal hemangiopericytoma that carries the same genetic abnormality, subset of dedifferentiated liposarcoma, and desmoid tumor [35].


MUC-4 is a transmembrane mucoprotein mentioned in a previous chapter with other mucins. In addition to glandular epithelial tumors, the expression of MUC-4 is also a characteristic marker for low-grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma, and glandular components in biphasic synovial sarcoma [6, 7].

Immunoprofile of fibroblastic and myofibroblastic tumors

Tumor type

+ in >90% (+)

+ in 50–90% (+/−)

+ in 10–50% (−/+)

+ in < 10% (−)

Nodular fasciitis :


Actin, CD68
Desmin, S100, CD34, pan-CK, EMA

Proliferative fasciitis :

Vimentin, myoglobin

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Dec 25, 2017 | Posted by in ONCOLOGY | Comments Off on Markers and Immunoprofile of Fibroblastic, Myofibroblastic, and Fibrohistiocytic Tumors

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