4 March 2010 Case of the Week #171


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Thanks to Dr. Vladimir Osipov, Medical College of Wisconsin, Milwaukee, WI (USA), for contributing this case. This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.



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Case of the Week #171


Clinical History


A middle-aged woman presented with a vaginal nodule, which had been growing slowly over a period of several months. It was submitted as a vaginal cyst, and measured 1 cm in size.

Micro images:


  Figure 1      Figure 2


  Figure 3      Figure 4     Figure 5


   CD99       bcl2     Smooth muscle actin


What is your diagnosis?




























Spindle cell epithelioma of the vagina




The tumor is well circumscribed but unencapsulated. There are myoid spindle cells (fig 4), epithelioid cells (fig 3) and cells forming cribriform patterns with mucin production (fig 2). There are occasional spherules present (fig 5). The tumor cells are immunoreactive with SMA, bcl-2, CD99 and cytokeratin (not shown).


Spindle cell epithelioma, also called mixed tumor of the vagina, is a rare tumor characterized by a mixture of well differentiated epithelial cells and stromal cells, with no/rare atypia or mitotic figures. The tumors are usually not connected with the overlying epithelium. Unlike mixed tumors of other sites, these tumors do not have myoepithelial cells (Am J Surg Pathol 1993;17:509).


Spindle cell epithelioma typically is immunoreactive for cytokeratin, muscle actin, CD10, CD34, vimentin, bcl2 and desmin (Mod Pathol 2004;17:1243, Arch Pathol Lab Med 2001;125:547), with variable staining for h-caldesmon and EMA. They are usually negative for S100 and lack CD99 membranous staining (but often have cytoplasmic staining).


Based on the staining patterns, they are thought to arise from a pluripotential cell with the ability to differentiate in both epithelial and mesenchymal directions. Diagnosis is based on morphology, and immunostains are usually not definitive.


These tumors have benign behavior. Simple excision is generally curative (Am J Surg Pathol 1981;5:413).



Nat Pernick, M.D., President,

and Kara Hamilton, M.S., Associate Medical Editor

PathologyOutlines.com, Inc.

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