Nasopharyngeal carcinoma in children: Multimodal treatment and long‐term outcome of 92 patients in a single center over a 28‐year period

Abstract

Objectives:The aim of the study was to evaluate the long-term outcome and late effects in pediatric patients with nasopharyngeal carcinoma (NPC) treated with neoadjuvant chemotherapy (NACT), followed by radiotherapy (RT).Methods: Ninety-two children (65 male, 27 female) diagnosed with NPC between 1989 and 2017 in the Istanbul University, Institute of Oncology were evaluated retrospectively. NACT consisted of three cycles of cisplatin-containing regimen every 3 weeks, followed by RT. Results:The median age was 13 years (5-18 years). Most had locoregionally advanced disease (stage III/IVA/IVB) and five had distant metastases at presentation. At a median follow-up of 108 months (3-332 months), 5-and 10-year overall survival rates and event-free survival rates were 87.5%, 79.7% and 82.1%, 78.9%, respectively. Three patients with distant metastasis are long-term survivors. Thirteen patients relapsed at a median of 8 months (2-23 months). Hypothyroidism (36%) and xerostomia (25%) were the most frequent long-term treatment-related toxicities. Nine second malignancies developed in eight patients, eight in the irradiated field at a median of 14 years (range 5-26 years), five of whom are long-term survivors after curative surgery.Conclusions: Three courses of cisplatin-containing NACT, followed by RT lead to high survival and locoregional control rate in advanced stage NPC in children. Patients with distant metastasis should also be treated with curative intent by systemic chemotherapy and locoregional radiotherapy. Patients should be followed closely for recurrences and long-term morbidities including second malignancies, which may be treated with curative surgeries if diagnosed early.

Familial clustering of nasopharyngeal carcinoma in the family of an adolescent with nasopharyngeal carcinoma

“…No familial NPC was reported in the large childhood NPC series from Turkey. [15][16][17]26 Herein, we present the familial clustering of NPC in the family of an adolescent with NPC. To our knowledge, it is the first reported familial NPC cluster in Turkey.…”

Nasopharyngeal Carcinoma in Children, Current Treatment Approach

“…43 However, several single-arm clinical studies were performed over the last few decades showing overall survival rates of 80% to 95% using neoadjuvant chemotherapy followed by concomitant chemoradiotherapy. 9,10,13,20,34,[44][45][46]…”

“…Treatment of locally advanced nonmetastatic NPC usually includes induction chemotherapy before RT to reduce tumor mass and prevent systemic spread. 10,17,18,46,48,49 Following completion of IC, concomitant chemotherapy, and radiotherapy (CCRT) are initiated. Maintenance therapy with Interferon beta (IFNß) following completion of IC and CCRT was initially employed by the GPOH in two prospective studies with very good results.…”

A multicenter retrospective analysis of patients with nasopharyngeal carcinoma treated in IMRT era from a nonendemic population: Turkish Society for Radiation Oncology Head and Neck Cancer Group Study (TROD 01‐001)

“…The Mediterranean region, including Turkey, represents an intermediate incidence zone (1–5/100 000) for NPC 1 . In this region, the disease shows a bimodal age distribution with the first peak starting from adolescence 3–5 . World Health Organization (WHO) has defined three main histological subtypes; keratinizing squamous cell carcinoma (WHO type 1), differentiated nonkeratinizing carcinoma (WHO type 2a), or undifferentiated nonkeratinizing carcinoma (WHO type 2b).…”

“…1 In this region, the disease shows a bimodal age distribution with the first peak starting from adolescence. [3][4][5] World Health Organization (WHO) has defined three main histological subtypes; keratinizing squamous cell carcinoma (WHO type 1), differentiated nonkeratinizing carcinoma (WHO type 2a), or undifferentiated nonkeratinizing carcinoma (WHO type 2b). The nonkeratinizing undifferentiated type is seen in almost all cases in endemic regions, while the keratinizing type is common in the west.…”