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Background: Rhabdomyosarcomas make up 5% of all pediatric malignant tumors. They are extremely rare in neonates. Furthermore, the external ear as a primary tumor site is very uncommon. In this paper, a neonate with a congenital auricular rhabdomyosarcoma is presented. Methods: At birth, a newborn male was noted to have a right posterior auricular mass. He is admitted to a children's hospital where he has diagnostic imaging, undergoes a biopsy, and then surgical resection of the auricular mass. Pathology confirms an infantile spindle cell rhabdomyosarcoma. He completes 22 weeks of chemotherapy without incident. Results: At one year of age, there is no evidence of metastasis or recurrence of the auricular tumor. Conclusions: A rare case of a neonate with a congenital auricular rhabdomyosarcoma is presented. The prognosis of congenital auricular rhabdomyosarcomas tends to be favorable.