Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France.
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Chirat
Service d'anatomo-pathologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France.
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Dainese
Service d'onco-hématologie, hôpital Armand-Trousseau, Paris VI University, 26, avenue du Dr Arnold-Netter, 75012 Paris, France.
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S
Fasola
Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France.
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Couloigner
Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France.
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Denoyelle
Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France.
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EN
Garabedian
Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France. Electronic address: nicolas.leboulanger@nck.aphp.fr.
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Leboulanger
D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D004428:Ear Neoplasms / Q000473:pathology* / Q000628:therapy; D004431:Ear, External / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D018714:Radiotherapy, Adjuvant; D012074:Remission Induction; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000473:pathology* / Q000628:therapy
Auricle; Child; Outer ear; Rhabdomyosarcoma