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Myoepithelial neoplasms of soft tissue represent a rare entity which has been described only recently when compared to salivary gland tumors with whom they share histopathological and molecular features. The most common locations are the superficial soft tissues of the limbs and limb girdles. However, they can rarely occur in the mediastinum, abdomen, bone, skin and visceral organs. Benign forms (myoepithelioma and mixed tumor) are more frequent than myoepithelial carcinoma and the latter mostly affects children and young adults. Diagnosis is mainly based on histology, which shows a proliferation of myoepithelial cells of variable morphology with or without glandular structures in a myxoid background, and immunohistochemistry, which shows co-expression of epithelial and myoepithelial markers. Molecular tests are not mandatory, but in selected cases FISH analysis can prove useful as about 50% of myoepitheliomas show EWSR1 (or rarely FUS) rearrangements and mixed tumors show PLAG1 rearrangements. Here, we present a case of a mixed tumor of the soft tissue occuring in the hand with expression of PLAG1 in immunohistochemistry., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Abstract: Myoepithelial soft tissue tumors represent less than 1% of soft tissue tumors. Diagnosis is based on histology, but clinical and radiological features can orient the practitioner. Myoepithelial tumors do not involve the bone as they are not malignant. In this article, we report a case of a myoepithelioma occuring on the volar aspect of the right ring finger involving the first phalanx. [Copyright &y& Elsevier]