Rhabdomyosarcoma of the ear region: A long‐term survival

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Abstract
Rhabdomyosarcoma of the ear region has been considered uniformly lethal. In view of this, two cases of rhabdomyosarcoma of the ear region are presented. Each occurred within the first four years of life. The first case was one of a two‐year‐old male with rhabdomyosarcoma of the mastoid area invading the lateral portion of the dura. He was treated with radical neck dissection and wide local excision of the tumor, including resection of dura and grafting of the defect; he also received Velban, Vincristine, and Cytoxan. He is alive and free of disease 96 months after the initial diagnosis.The second case was one of a three and one half year old male who had alveolar rhabdomyosarcoma of the post‐auricular region of the right ear. This was treated with radical neck dissection, radical local excision and an 18‐month course of Vincristine and Actinomycin D. This patient is alive and free of disease 25 months after the original diagnosis.It is encouraging that we have one long‐term survivor and one survivor of over two years, from a disease previously considered as uniformly lethal. Awareness by the otologist of the presence of this tumor and attention to the child in the first decade of life presenting with an apparent suppurative ear disease with unusual lack of systemic systems, particularly when associated with a facial nerve paralysis, will lead to earlier diagnosis and more vigorous treatment. Perhaps more of these children can be salvaged with the combination of early diagnosis, radical surgical removal and chemotherapy.

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