RT Journal Article
SR Electronic
T1 Sinonasal and Laryngeal Carcinoma in Children: Correlation of Imaging Characteristics with Clinicopathologic and Cytogenetic Features
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 257
OP 261
DO 10.3174/ajnr.A1800
VO 31
IS 2
A1 C.D. Robson
A1 R. Rahbar
A1 S.O. Vargas
A1 K.J. Marcus
A1 J.W. Mack
A1 G.R. Licameli
A1 H.E. Grier
YR 2010
UL http://www.ajnr.org/content/31/2/257.abstract
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.