Malignant epithelioid soft-tissue tumour with NRID1-MAML1 fusion

Epithelioid soft-tissue tumours are predominantly composed of rounded or polygonal tumour cells resembling epithelial cells. In the current WHO classification, epithelioid soft tissue tumors of uncertain differentiaton include myoepithelial tumour, alveolar soft part sarcoma (ASPS), and PEComa among others. There is only one published case of paediatric novel soft-tissue tumour with an NRID1-MAML1 fusion.1 We report the second case with the same NRID1-MAML1 fusion gene; however, this is the first case with malignancy in a young adult.