Pediatric Myoepithelial Carcinoma of the Hand

Case: A 2-year-old boy with a painless volar left hand mass was diagnosed with myoepithelial carcinoma. Pathologic evaluation demonstrated positive S100 and cytokeratin AE1/AE3, negative glial fibrillary acidic protein (GFAP), and scarce epithelial membrane antigen (EMA)-positive cells. After tumor excision, the patient received 10 rounds of chemotherapy without radiation. He demonstrated no local recurrence approximately 3 years postoperatively; however, treatment is ongoing for metastatic disease. Conclusion: Myoepithelial carcinomas of the soft tissue are extremely rare, yet highly aggressive tumors with a tendency for local recurrence and metastasis. Timely diagnosis and treatment are a multidisciplinary challenge paramount for optimizing patient outcomes.