Myoepithelioma‐like tumor of the vulvar region showing infiltrative growth and harboring only a few estrogen receptor‐positive cells: A case report

Recently, a new entity “myoepithelioma‐like tumor of the vulvar region (MELTVR)” was proposed as a rare mesenchymal neoplasm arising in vulvar regions of adult women. While MELTVRs morphologically resemble soft tissue myoepitheliomas and extraskeletal myxoid chondrosarcomas, they have a unique immun...

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Bibliographic Details
Published in:Pathology international 2019-03, Vol.69 (3), p.172-176
Main Authors: Kojima, Yui, Tanabe, Mikiko, Kato, Ikuma, Motoi, Toru, Kimura, Mariko, Sawazumi, Tomoe, Tanaka, Reiko, Chiba, Sawako, Otani, Masako, Inayama, Yoshiaki
Format: Article
Language:English
Subjects:
Biomarkers, Tumor - genetics
Calmodulin-Binding Proteins - genetics
Case reports
estrogen receptor
Estrogens
FLI-1 protein
Fluorescence
Fluorescence in situ hybridization
Gene expression
Gene rearrangement
Gene Rearrangement - genetics
Glial fibrillary acidic protein
Humans
immunohistochemistry
Immunohistochemistry - methods
infiltrative growth
INI1
Mesenchyme
Mucin-1 - immunology
Myoepithelioma - diagnosis
Myoepithelioma - pathology
myoepithelioma‐like tumor of the vulvar region
Neoplasia
Proteins
Receptors, Estrogen - metabolism
S100 protein
SMARCB1
Soft tissues
Tumors
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Summary:Recently, a new entity “myoepithelioma‐like tumor of the vulvar region (MELTVR)” was proposed as a rare mesenchymal neoplasm arising in vulvar regions of adult women. While MELTVRs morphologically resemble soft tissue myoepitheliomas and extraskeletal myxoid chondrosarcomas, they have a unique immunohistochemical profile (positive for epithelial membrane antigen and estrogen receptor, negative for S100 protein and glial fibrillary acidic protein, and loss of INI1/SMARCB1 expression), and lack EWSR1 and NR4A3 gene rearrangement, as seen by fluorescence in situ hybridization. MELTVRs are usually well‐demarcated tumors, with no reports of extensive infiltrative growth. In the current report, we present an unusual case of MELTVR showing infiltrative growth and harboring only a few estrogen receptor‐positive cells, which might indicate a variation in this rare tumor.
ISSN:1320-5463
1440-1827
DOI:10.1111/pin.12765