Clinicopathological and prognostic features of nasopharyngeal carcinoma in children and adolescents: A retrospective study of 196 cases in South China. Issue 5 (21st September 2020)

• Record Type: Journal Article
• Authors: Zhang, Jijun; Luo, Xiaoya; Huang, Qitao; Huang, Yuhua
• Is Part Of: International journal of cancer. Volume 148:Issue 5(2021)
• Page Start: 1087
• Page End: 1096
• Publication Date: 2020-09-21
• Subjects: children and adolescents -- clinicopathologic features -- Epstein‐Barr virus -- nasopharyngeal carcinoma -- South China; Cancer -- Periodicals; Cancer -- Prevention -- Periodicals; 616.994
• DOI: 10.1002/ijc.33293
• Languages: English
• ISSNs: 0020-7136

Abstract

Abstract: Nasopharyngeal carcinoma (NPC) occurring in children and adolescence is extremely rare and till present there is a lack of understanding on their clinicopathological and prognostic features of this rare entity. For our study, data of 196 cases children and adolescents with NPC from the past 18 years at a high‐volume cancer center from South China were retrospectively analyzed. Half of the evaluated NPC patients (83/166, 50.0%) were staged as Stage IVa disease, whereas 1.2% (2/166), 27.7% (46/166), 16.9% (28/166) and 4.2% (7/166) had Stage II, III, IVb and IVc disease, respectively. Serum EBV EA‐IgA ≥1:10 and VCA‐IgA ≥1:40 were found in 67.7% (113/167) and 76.6% (128/167) of the evaluated patients, respectively, whereas 56.8% (84/148) of the patients had plasma EBV DNA ≥1000 copies/mL. Histologically, all tumors were classified as nonkeratinizing squamous cell carcinoma (NK‐SCC). Immunohistochemistrically, the expression of CK (AE1/AE3), P63, CK5/6 and P40 were observed in 100% (88/88), 93.2% (68/73), 84.1% (58/69) and 63.2% (12/19) of the detected cases, respectively. All cases show similar immunophenotype compared to that occurring in adult patients. All evaluated cases (71/71 100%) harbored EBER. Patients with plasma EBV DNA ≥1000 copies/mL and positive serum EBV antibodies had significantly inferior 3‐year OS (88% vs 100%, P = .007) compared to other corresponding groups. The combination of EBV serology and plasma EBV DNA are useful to predict the outcome of patients with NPC in children and adolescents. Abstract : What's new Nasopharyngeal carcinoma (NPC) is extremely rare in children and adolescents, and the clinicopathological characteristics and prognostic features of pediatric NPC remain largely unknown. In this retrospective study, the authors describe the largest series of NPC in children and adolescents in southern China, the most endemic area of NPC, and reveal that all cases histologically belonged to nonkeratinizing squamous cell carcinoma. Moreover, they provide evidence that the combination of Epstein‐Barr virus (EBV) serology and plasma EBV DNA is useful for predicting patient outcome, with potential clinical applications for risk group stratification and prognostic prediction for pediatric NPC patients.