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    Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Head and Neck Cancers

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    Diagnostic evaluation

    Diagnostic tests should determine the extent of the primary tumor and whether there are metastases. Visualization of the nasopharynx by an ear-nose-throat specialist using nasal endoscopy, examination by a neurologist, and magnetic resonance imaging of the head and neck can be used to determine the extent of the primary tumor. A diagnosis can be made from a biopsy of the primary tumor or of enlarged lymph nodes of the neck. Nasopharyngeal carcinomas must be distinguished from all other cancers that can present with enlarged lymph nodes and from other types of cancer in the head and neck area. Thus, diseases such as thyroid cancer, rhabdomyosarcoma, non-Hodgkin lymphoma, Hodgkin lymphoma, and Burkitt lymphoma must be considered, as should benign conditions such as nasal angiofibroma, which usually presents with epistaxis in adolescent males, and infectious lymphadenitis. Evaluation of the chest and abdomen by computed tomography and bone scan should also be performed to determine whether there is metastatic disease.

    Staging

    Tumor staging is performed utilizing the tumor-node-metastasis classification system of the American Joint Committee on Cancer (AJCC).[9] The majority (>90%) of children and adolescents with nasopharyngeal carcinoma present with advanced disease (stage III/IV or T3/T4).[6,10,11] Metastatic disease at diagnosis is uncommon (stage IVC). A retrospective analysis of data from the Surveillance Epidemiology and End Results (SEER) program reported that patients younger than 20 years had a higher incidence of advanced-stage disease than did older patients, higher risk of developing a second malignancy, and a superior outcome after controlling for stage.[5]

    Prognosis

    The overall survival of children and adolescents with nasopharyngeal carcinoma has improved over the last four decades; with state-of-the-art multimodal treatment, 5-year survival rates are in excess of 80%.[5,6,11,12] However, the intensive use of chemotherapy and radiation therapy results in significant acute and long-term morbidities.[6,11]

    Treatment

    Treatment of nasopharyngeal carcinoma is multimodal:

    1. Combined-modality therapy with chemotherapy and radiation: High-dose radiation therapy alone has had a role in the management of low-stage nasopharyngeal carcinoma, but studies in both children and adults show that combined modality therapy with chemotherapy and radiation is the most effective way to treat nasopharyngeal carcinoma.[6,11,12,13,14,15,16]
      1. Many randomized studies have investigated the role of chemotherapy in the treatment of adult nasopharyngeal carcinoma. In a meta-analysis of ten randomized studies and 2,450 patients, the use of concomitant chemoradiation therapy was associated with a significant survival benefit, including improved locoregional disease control and reduction in distant metastases.[15] Neoadjuvant chemotherapy resulted in a significant reduction in locoregional recurrence only, while postradiation chemotherapy did not offer any benefit.
      2. In children, four studies utilizing preradiation chemotherapy with different combinations of methotrexate, cisplatin, 5-fluorouracil (5-FU), and leucovorin with or without recombinant interferon-beta have reported response rates of more than 90%.[11,12,17,18]
        • Neoadjuvant chemotherapy with cisplatin and 5-FU (with or without leucovorin), followed by chemoradiation with single-agent cisplatin yield 5-year overall survival (OS) rates consistently above 80%.[11,12]
        • A preliminary analysis of the NPC-2003-GPOH study, which included a 6-month maintenance therapy phase with interferon-beta, reported a 30-month OS estimate of 97.1%.[12]
      3. While nasopharyngeal carcinoma is a very chemosensitive neoplasm, high radiation doses to the nasopharynx and neck (approximately 60 Gy) are required for optimal locoregional control.[6,11,12] The combination of cisplatin-based chemotherapy and high doses of radiation therapy to the nasopharynx and neck are associated with a high probability of hearing loss, hypothyroidism and panhypopituitarism, trismus, xerostomia, dental problems, and chronic sinusitis or otitis.[6,11]; [19][Level of evidence: 3iiiA]
      4. Additional drug combinations that have been used in children with nasopharyngeal carcinoma include bleomycin with epirubicin and cisplatin and cisplatin with methotrexate and bleomycin.[3]
      5. Other approaches to the management of nasopharyngeal carcinoma in children have been evaluated and include the following:
        • Incorporation of high-dose-rate brachytherapy into the chemoradiation therapy approach.[20,21]
        • Following adult data, taxanes have been incorporated into the treatment of childhood nasopharyngeal carcinoma; studies have shown good objective response rates and favorable outcomes with the use of docetaxel in combination with cisplatin.[22][Level of evidence: 3iiiDiv]
    2. Surgery: Surgery has a limited role in the management of nasopharyngeal carcinoma because the disease is usually considered unresectable due to extensive local spread.
    3. EBV-specific cytotoxic T-lymphocytes: The use of EBV-specific cytotoxic T-lymphocytes has shown to be a very promising approach with minimal toxicity and evidence of significant antitumor activity in patients with relapsed or refractory nasopharyngeal carcinoma.[23]
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