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PT  - JOURNAL ARTICLE
AU  - Robson, C.D.
AU  - Rahbar, R.
AU  - Vargas, S.O.
AU  - Marcus, K.J.
AU  - Mack, J.W.
AU  - Licameli, G.R.
AU  - Grier, H.E.
TI  - Sinonasal and Laryngeal Carcinoma in Children: Correlation of Imaging Characteristics with Clinicopathologic and Cytogenetic Features
AID  - 10.3174/ajnr.A1800
DP  - 2010 Feb 01
TA  - American Journal of Neuroradiology
PG  - 257--261
VI  - 31
IP  - 2
4099  - http://www.ajnr.org/content/31/2/257.short
4100  - http://www.ajnr.org/content/31/2/257.full
SO  - Am. J. Neuroradiol.2010 Feb 01; 31
AB  - BACKGROUND AND PURPOSE: Pediatric upper airway carcinoma is uncommon, symptoms are nonspecific, and diagnosis is often delayed. In this study, we describe the imaging, cytogenetics, and clinical courses of 4 patients with pediatric upper airway carcinoma. MATERIALS AND METHODS: Four patients with upper airway carcinoma were identified during a 2.5-year period. CT (n = 4) and MR imaging (n = 3) studies, tumor histopathologic features and cytogenetics, patient treatment, and clinical course were reviewed. RESULTS: Patients were aged 12 to 15 years. One tumor involved the larynx with poorly defined margins and heterogeneous enhancement; 1 heterogeneously enhancing tumor involved the epiglottis with necrotic cervical lymphadenopathy. There were 2 enhancing sinonasal tumors with bony destruction in 1 tumor. Tumors had a relatively short relaxation time on FSEIR MR imaging. Histopathologic examination revealed poorly differentiated squamous cell carcinoma (n = 3) and well-differentiated squamous cell carcinoma (n = 1). Cytogenetic analysis revealed chromosomal abnormalities in 3 tumors: 2 showed a chromosomal translocation t(15;19), and 1 showed a chromosomal translocation t(1;5) and loss of a portion of chromosome 22q. Results of in situ hybridization for EBV were negative (n = 3). Treatment included tumor resection (n = 2), chemotherapy (n = 4), and radiation therapy (n = 3). Patients with t(15;19) died months after diagnosis. Two patients were alive at 8-year follow-up. CONCLUSIONS: Childhood carcinoma of the upper airway is uncommon but should be considered in the diagnosis of upper airway tumors that display aggressive imaging characteristics. Carcinoma with t(15;19) is rare but has been reported, usually in young patients with midline carcinoma of the neck or mediastinum, with a rapidly fatal course. EBVEpstein-Barr virusEBEREpstein-Barr virus encoded RNAFSEIRfast spin-echo inversion recoveryFISHfluorescent insitu hybridizationf/ufollow-upGyGrayLNlymph nodesmosmonthsmsEFeffective millisecondsNPCnasopharyngeal carcinomaURTupper respiratory tractVCRvincristinewkweeksyyear.