Nasopharyngeal Carcinoma in the Pediatric Age Group

Abstract

Nasopharyngeal carcinoma (NPC) is rare in children, accounting for less than 1% of all malignancies. Radiation therapy has been the mainstay of treatment of many years, but to improve survival, the use of chemotherapy has been advocated. This is a retrospective analysis of 13 patients less than 20 years of age treated for NPC the Rambam Medical Center during 1989 to 2004. Eight boys and five girls with a median age of 14.5 years (range 10-19) were included. Median follow up (including patients who died) was 6.15 years (range 1-15 years). Duration of symptoms was 1 to 24 months (median 5 months). Of the 13 patients, one patient had stage I, 6 had stage III, 5 had stage IV-A, and 1 had stage IV-B disease. Ten patients (77%) had undifferentiated carcinoma (WHO type III) and three patients (23%) had nonkeratinizing carcinoma (WHO type II). Most of the children received two or three courses of neoadjuvant multiagent chemotherapy consisting of cisplatin and 5-FU, followed by radiotherapy with doses in excess of 60 Gy. One child received concomitant chemoradiation. Ten of the 13 patients (77%) are alive without disease 6 years after diagnosis (range 1-15 years). One patient developed local and distant metastases 1 year after diagnosis and is currently receiving combined radiochemotherapy. Two patients died. Overall survival was 84%; event-free survival was 77%. Nine patients (69%) developed moderate to severe long-term complications. Pediatric NPC is curable by combined radiation and chemotherapy, with doses of radiation in excess of 60 Gy. Long-term follow-up is important for early detection of second malignancies as well as for radiation-induced endocrinologic deficiencies and other normal tissue complications.

Nasopharyngeal carcinoma in children and young adults—Beyond 5‐year survival

“…Israel is considered an intermediate-risk region with a reported incidence of 1-1.9/100 000 and 3.7 per million in adults and children, respectively, and probably reflects various factors related to both race and immigration patterns unique to Israel. 1,[7][8][9][10] The clinical characteristics of the patients in our cohort, including male predominance and age at presentation, tumor histology, and advanced stage at diagnosis, are similar to other reports on childhood NPC. 3,4,6,9,11 Treatment of NPC in Israel follows the accepted combination of radiotherapy and cisplatin-based chemotherapy with varying radiation doses and chemotherapy regimens used over the years.…”

Nasopharyngeal Carcinoma in Children, Current Treatment Approach

“…Traditionally, RT in both adult and pediatric NPC used high doses (66 to 70 Gy) to the primary tumor and involved lymph nodes and a lower, prophylactic dose of 45 to 50 Gy to uninvolved cervical lymph nodes in the neck and supraclavicular region. 15,46,52 NPC patients in historical cohorts were treated with 2D or 3D conformal RT, but currently, most adults and children are treated using intensity modulated radiotherapy (IMRT) that was shown to provide improved local control and lower rate of acute and late toxicities, and is considered as the standard RT technique for NPC. 20,[53][54][55] Target volumes comprise the gross tumor volume (GTV) that includes the primary tumor and involved LN at presentation, preferably defined by the combination of local endoscopy, MRI, and FDG PET/CT at presentation, and clinical target volume (CTV) that includes the GTV and potential areas of microscopic spread.…”

“…Oxaliplatin-doxorubicin 67,75,76 4-6 y Alive at time of report Oxaliplatin-gemcitabine 67 20 mo Alive at time of report Carboplatin-gemcitabine-paclitaxel 67 7 mo 11 mo Carboplatin-docetaxel 67 4 mo 14 mo Carboplatin-5FU 67 15 mo 22 mo Carboplatin-paclitaxel-ifosfamide combined with EBV-specific lymphocytes 67 43 mo Alive at time of report Carboplatin-paclitaxel 67 3 mo Alive at time of report Doxorubicin-vincristine-cyclophosphamide-irinotecan combined with EBV-specific lymphocytes 67 49 mo Alive at time of report Nivolumab 67 12 mo Alive at time of report Gemcitabine-vinorelbine 67 2 mo 13 mo Cetuximab 67 7 mo 21 mo Irinotecan-topotecan 67 3 mo 17 mo Gemcitabine-docetaxel 13 75 mo Alive at time of report Carboplatin-ifosfamide-etoposide 77 7 mo 7 mo Carboplatine-5FU-docetaxel 18 2 y Alive at time of report EBV-CTLs+chemotherapy 78 NA NA Progress in both local and systemic treatments of pediatric NPC reduced the rates of local relapse in primary nonmetastatic disease, with recent reports showing failure of local control in 4% to 7%. 10,13,15,20,35,52 However, despite improved local control, rates of distant metastatic relapse have not significantly changed, with a distant failure rate of 10% to 13% reported in several national cohorts, reflecting the need for better systemic treatment for pediatric NPC. 10,12,13,20,35,45,71,74 The outcome for patients with relapsed disease is poor and there are no standard secondline therapies for this high-risk group.…”

“…Radiotherapy is the mainstay of treatment in NPC which are usually very radiosensitive. Traditionally, RT in both adult and pediatric NPC used high doses (66 to 70 Gy) to the primary tumor and involved lymph nodes and a lower, prophylactic dose of 45 to 50 Gy to uninvolved cervical lymph nodes in the neck and supraclavicular region 15,46,52 . NPC patients in historical cohorts were treated with 2D or 3D conformal RT, but currently, most adults and children are treated using intensity modulated radiotherapy (IMRT) that was shown to provide improved local control and lower rate of acute and late toxicities, and is considered as the standard RT technique for NPC 20,53–55 .…”

“…Progress in both local and systemic treatments of pediatric NPC reduced the rates of local relapse in primary nonmetastatic disease, with recent reports showing failure of local control in 4% to 7% 10,13,15,20,35,52 . However, despite improved local control, rates of distant metastatic relapse have not significantly changed, with a distant failure rate of 10% to 13% reported in several national cohorts, reflecting the need for better systemic treatment for pediatric NPC 10,12,13,20,35,45,71,74 .…”