Rhabdomyosarcoma of the Middle Ear and Mastoid: A Case Report and Review of the Literature

Abstract

We report a case ofrhabdomyosarcoma ofthe middle ear and mastoid in a S-y ear-old boy. The patient was treated according to Int ergroup Rhabdomyosarcoma Study IV protocol (chemo-andradioth erapy), andhe expe rienced a complete remission. However; 7months after the completion oftreatment, he expe rienced a recurrence at theprimary site that spread to the brain. Despite treatm ent, the patient died ofprogressive metastasis to the lung 4 months later:

Rhabdomyosarcoma in middle ear of an adult: a rare presentation

“…Rhabdomyosarcoma is one of the most common tumors of pediatric age group (1). They exhibit a bimodal pattern of age distribution: peaking between 2 to 5 yrs age group and then a spike in late adolescence (1).…”

“…They exhibit a bimodal pattern of age distribution: peaking between 2 to 5 yrs age group and then a spike in late adolescence (1). Overall 63% cases are under 10 yrs of age (2).…”

“…It was advised that surgical resection should only be done if major morbidity can be avoided. This protocol was advised in 1978 (IRS II), 1984 (IRS III) and 1991 (IRS IV)(1). The basic difference is the increasing and better role of chemotherapeutic agents.…”

“…Rhabdomyosarcoma is the most common soft-tissue malignancy in pediatric age group arising mainly in the head and neck, most commonly in the orbit and the nasopharynx (1). Ear is comparatively a rare site for this neoplasm and accounts for less than 10% of all cases of head and neck (2).…”

“…It is an unusual occurrence in adult population, even more rare to involve the middle ear and mastoid (3). Rhabdomyosarcoma frequently looks like a polypoid mass on examination, so easily misdiagnosed as aural polyp and therefore, advanced disease with meningeal involvement is common at the time of diagnosis (1). …”