Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma

Abstract Introduction Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We...

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Bibliographic Details
Published in:European annals of otorhinolaryngology, head and neck diseases head and neck diseases, 2016-02, Vol.133 (1), p.23-26
Main Authors: Chirat, M, Dainese, L, Fasola, S, Couloigner, V, Denoyelle, F, Garabedian, E.-N, Leboulanger, N
Format: Article
Language:English
Subjects:
Adolescent
Auricle
Chemotherapy, Adjuvant
Child
Ear Neoplasms - pathology
Ear Neoplasms - therapy
Ear, External - pathology
Ear, External - surgery
Female
Humans
Male
Otolaryngology
Outer ear
Radiotherapy, Adjuvant
Remission Induction
Retrospective Studies
Rhabdomyosarcoma
Rhabdomyosarcoma - pathology
Rhabdomyosarcoma - therapy
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Summary:Abstract Introduction Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. Case reports The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. Discussion In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear.
ISSN:1879-7296
1879-730X
DOI:10.1016/j.anorl.2015.09.005