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Myoepithelioma-like hyalinizing epithelioid tumors of the hand with novel OGT-FOXO3 fusions

American Journal of Surgical Pathology Feb 19, 2020

Lee JC, Chou HC, Wang CH, et al. - Given that there are cases wherein the morphology is reminiscent of myoepithelial tumors, while the immunophenotype falls short, researchers here reported 2 highly similar myoepithelioma-like tumors arising in the hands of young adults. Well-demarcation was evident in both the tumors; the tumors were composed of alternating cellular areas with palely eosinophilic hyaline stroma and scattered acellular zones of densely eosinophilic collagen deposition. Mainly epithelioid cells formed the tumor cells; these were arranged in cords or small nests. The focal prominence of vacuolated cells encircling hyaline matrix globules was evident. Further, they observed a minor component of nonhyaline fibrous nodular areas composed of bland spindle cells and rich vasculature. Some areas exhibited the presence of perivascular concentric spindle cell proliferation and perivascular hyalinization. Immunostaining revealed the tumor cells as positive for CD34 and epithelial membrane antigen (focal), while these were largely negative for cytokeratin, S100, GFAP, p63, GLUT1, and claudin-1. RNA sequencing led to the identification of a novel OGT-FOXO3 fusion gene in case 1 and reverse transcription polymerase chain reaction and fluorescence confirmed in situ hybridization in both cases. The two cases thus shared the unusual clinicopathologic features and the novel fusion and hence probably represent a distinct tumor entity, whose connection with myoepithelial tumors and tumorigenic mechanisms exerted by the OGT-FOXO3 fusion require further investigation
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