A Prospective Protocol for Nasopharyngeal Carcinoma in Children and Adolescents: The Italian Rare Tumors in Pediatric Age (TREP) Project
Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated subtype and by an advanced clinical stage at onset and better chances of survival. The risk of long-term treatment-related toxicity also may be a...
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Published in | Cancer Vol. 118; no. 10; pp. 2718 - 2725 |
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Main Authors | , , , , , , , , , , , |
Format | Journal Article |
Language | English |
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15.05.2012
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Abstract | Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated subtype and by an advanced clinical stage at onset and better chances of survival. The risk of long-term treatment-related toxicity also may be a more important issue in younger individuals.
A prospective chemoradiotherapy protocol for pediatric NPC was started in Italy in 2000 within the framework of the Rare Tumors in Pediatric Age (TREP) project. Three courses of cisplatin/5-fluorouracil induction chemotherapy were followed by radiotherapy (doses up to 65 grays) with concomitant cisplatin.
Forty-six patients (ages 9-17 years) were considered eligible for the study over a 10-year period. The ratio of observed to expected cases based on epidemiological data was approximately 1 for both children and adolescents. All but 1 patient had lymph node involvement, and 5 patients had distant metastases. The rate of response to primary chemotherapy was 90%. The 5-year overall and progression-free survival rates were 80.9% and 79.3%, respectively (median follow-up, 62 months). The only statistically significant prognostic variable was the presence or absence of distant metastases. A 65% incidence of late sequelae was reported.
This study demonstrates the feasibility and efficacy of a prospective protocol even for such rare tumors as pediatric NPC. The use of lower radiotherapy doses than those used in adults did not affect locoregional failure rates. Long-term follow-up will be needed to obtain more information on both survival and treatment sequelae. The next objective will be to establish broader, international prospective cooperation schemes. |
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AbstractList | Abstract
BACKGROUND:
Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated subtype and by an advanced clinical stage at onset and better chances of survival. The risk of long‐term treatment‐related toxicity also may be a more important issue in younger individuals.
METHODS:
A prospective chemoradiotherapy protocol for pediatric NPC was started in Italy in 2000 within the framework of the Rare Tumors in Pediatric Age (TREP) project. Three courses of cisplatin/5‐fluorouracil induction chemotherapy were followed by radiotherapy (doses up to 65 grays) with concomitant cisplatin.
RESULTS:
Forty‐six patients (ages 9‐17 years) were considered eligible for the study over a 10‐year period. The ratio of observed to expected cases based on epidemiological data was approximately 1 for both children and adolescents. All but 1 patient had lymph node involvement, and 5 patients had distant metastases. The rate of response to primary chemotherapy was 90%. The 5‐year overall and progression‐free survival rates were 80.9% and 79.3%, respectively (median follow‐up, 62 months). The only statistically significant prognostic variable was the presence or absence of distant metastases. A 65% incidence of late sequelae was reported.
CONCLUSIONS:
This study demonstrates the feasibility and efficacy of a prospective protocol even for such rare tumors as pediatric NPC. The use of lower radiotherapy doses than those used in adults did not affect locoregional failure rates. Long‐term follow‐up will be needed to obtain more information on both survival and treatment sequelae. The next objective will be to establish broader, international prospective cooperation schemes. Cancer 2011. © 2011 American Cancer Society.
The current results indicate the feasibility and efficacy of a prospective chemoradiotherapy protocol for patients with pediatric nasopharyngeal carcinoma that was developed between 2000 and 2009 within the framework of the Italian Rare Tumors in Pediatric Age (TREP) project. Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated subtype and by an advanced clinical stage at onset and better chances of survival. The risk of long-term treatment-related toxicity also may be a more important issue in younger individuals. A prospective chemoradiotherapy protocol for pediatric NPC was started in Italy in 2000 within the framework of the Rare Tumors in Pediatric Age (TREP) project. Three courses of cisplatin/5-fluorouracil induction chemotherapy were followed by radiotherapy (doses up to 65 grays) with concomitant cisplatin. Forty-six patients (ages 9-17 years) were considered eligible for the study over a 10-year period. The ratio of observed to expected cases based on epidemiological data was approximately 1 for both children and adolescents. All but 1 patient had lymph node involvement, and 5 patients had distant metastases. The rate of response to primary chemotherapy was 90%. The 5-year overall and progression-free survival rates were 80.9% and 79.3%, respectively (median follow-up, 62 months). The only statistically significant prognostic variable was the presence or absence of distant metastases. A 65% incidence of late sequelae was reported. This study demonstrates the feasibility and efficacy of a prospective protocol even for such rare tumors as pediatric NPC. The use of lower radiotherapy doses than those used in adults did not affect locoregional failure rates. Long-term follow-up will be needed to obtain more information on both survival and treatment sequelae. The next objective will be to establish broader, international prospective cooperation schemes. |
Author | FERRARI, Andrea BISOGNO, Gianni BASSO, Eleonora CATALDO, Andrea Di INDOLFI, Paolo COLLINI, Paola FAVINI, Francesca D'ANGELO, Paolo GANDOLA, Lorenza CASANOVA, Michela POTEPAN, Paolo CECCHETTO, Giovanni |
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Keywords | Human rare pediatric tumors Pediatrics nasopharyngeal carcinoma Nasopharynx cancer Malignant tumor Nasopharynx carcinoma Radiotherapy Prospective sequelae Chemotherapy Treatment Cancerology Adolescent children and adolescents ENT disease Pharynx disease Child Age Cancer Rare Tumors in Pediatric Age (TREP) project |
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References | Fleming ID (e_1_2_6_21_2) 1997 e_1_2_6_31_2 e_1_2_6_30_2 e_1_2_6_18_2 e_1_2_6_19_2 e_1_2_6_12_2 e_1_2_6_35_2 e_1_2_6_13_2 e_1_2_6_34_2 e_1_2_6_10_2 e_1_2_6_33_2 e_1_2_6_11_2 e_1_2_6_32_2 e_1_2_6_16_2 e_1_2_6_39_2 e_1_2_6_17_2 e_1_2_6_38_2 e_1_2_6_14_2 e_1_2_6_37_2 e_1_2_6_15_2 e_1_2_6_36_2 Saeed H (e_1_2_6_27_2) 2009; 10 e_1_2_6_20_2 e_1_2_6_40_2 e_1_2_6_8_2 e_1_2_6_7_2 e_1_2_6_9_2 e_1_2_6_29_2 e_1_2_6_4_2 e_1_2_6_3_2 e_1_2_6_6_2 e_1_2_6_5_2 e_1_2_6_24_2 e_1_2_6_23_2 e_1_2_6_2_2 e_1_2_6_22_2 e_1_2_6_28_2 e_1_2_6_26_2 e_1_2_6_25_2 |
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Snippet | Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated... Abstract BACKGROUND: Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing,... |
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SubjectTerms | Adolescent Biological and medical sciences Carcinoma Chemoradiotherapy Child Disease-Free Survival DNA, Viral - analysis Female Herpesvirus 4, Human - isolation & purification Humans Male Medical sciences Nasopharyngeal Carcinoma Nasopharyngeal Neoplasms - mortality Nasopharyngeal Neoplasms - pathology Nasopharyngeal Neoplasms - therapy Nasopharyngeal Neoplasms - virology Neoplasm Staging Otorhinolaryngology. Stomatology Prospective Studies Treatment Outcome Tumors Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology |
Title | A Prospective Protocol for Nasopharyngeal Carcinoma in Children and Adolescents: The Italian Rare Tumors in Pediatric Age (TREP) Project |
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