Malignant myoepithelioma (myoepithelial carcinoma) of soft tissue

Malignant myoepithelioma of soft tissue is extremely rare. Presented herein is a case arising in a 17-year-old man. The tumor was initially noticed as a painless deep soft-tissue mass in the right forearm when the patient was aged 3 years. Thereafter, it grew without remarkable symptoms, such as pain or tenderness, until his visit to the hospital because of swelling of his forearm when he was 17 years old. An excisional biopsy specimen disclosed an invasive tumor exhibiting a lobular architecture. The tumor cells were arranged in a reticular and/or trabecular fashion with a myxoid background, and nuclear atypia was evident. Mitoses and tumor necrosis were also observed. Immunohistochemically, S-100 protein and epithelial markers were diffusely positive. Faint intercellular junctions and basal laminae were identified by electronmicroscopy. On the basis of these findings, the tumor was diagnosed as a malignant myoepithelioma of soft tissue. Six months later, multiple lung metastases were observed, and an open biopsy revealed a neoplasm displaying the same histological feature as the previously biopsied specimens. The patient died of his disease 18 months after the lung biopsy. Malignant myoepithelioma should be kept in mind in diagnosis of deep soft-tissue tumors with epithelioid features.