Cancer Health Center

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A preliminary report of the use of EBV-specific cytotoxic T-lymphocytes revealed minimal toxicity and evidence of significant antitumor activity in patients with relapsed or refractory nasopharyngeal carcinoma.[21] (Refer to the PDQ summary on Nasopharyngeal Cancer Treatment for more information.)

Treatment options under clinical evaluation

• COG-ARAR0331: This Children's Oncology Group trial is evaluating the efficacy of induction chemotherapy with cisplatin plus 5-FU followed by concomitant chemotherapy (cisplatin) plus radiation therapy with amifostine as a radioprotectant in patients with AJCC stages IIB to IV nasopharyngeal carcinoma. Patients with stages I to IIA disease will receive only radiation therapy with amifostine.

Esthesioneuroblastoma

Esthesioneuroblastoma (olfactory neuroblastoma) is a very rare, small round-cell tumor arising from the nasal neuroepithelium that is distinct from primitive neuroectodermal tumors.[22,23,24,25] Most children present with a nasopharyngeal mass, which may have local extension into the orbits, sinuses, or frontal lobe, with associated symptoms. There appears to be a male predominance, and the average age of presentation is in adolescence. The youngest child reported with this diagnosis was aged 2 years. Metastatic disease is uncommon. The long-term survival rate appears to be approximately 60% to 80%. Local recurrences may occur later in life.

A retrospective analysis of data from the Surveillance, Epidemiology, and End Results program identified 311 patients with esthesioneuroblastoma.[26] Patients were staged by the extent of the tumor. Disease limited to the nasal cavity was considered the lowest stage and involvement of regional lymph nodes or metastasis was considered the highest stage. This staging system correlated well with outcome. A meta-analysis of 26 studies with a total of 390 patients, largely adults, with esthesioneuroblastoma indicates that higher histopathologic grade and metastases to the cervical lymph nodes may correlate with adverse prognostic factors.[27]

The mainstay of treatment has been surgery and radiation.[28] Newer techniques such as endoscopic sinus surgery, stereotactic radiosurgery, and proton-beam therapy (charged-particle radiation therapy) may play a role in the management of this tumor.[29] Routine neck dissection and nodal exploration are not indicated in the absence of clinical or radiological evidence of disease.[30] Management of cervical lymph node metastases has been addressed in a review article.[30]

Reports indicate the increasing use of neoadjuvant or adjuvant chemotherapy.[22,23,31,32,33]; [34][Level of evidence: 3iii] Chemotherapy regimens that have been used with efficacy include etoposide (VP-16) with ifosfamide and cisplatin;[35] vincristine, actinomycin D, and cyclophosphamide without doxorubicin; ifosfamide/etoposide; cisplatin plus etoposide or doxorubicin; [31] and irinotecan plus docetaxel.[36][Level of evidence: 3iiA]

Thyroid Tumors

Tumors of the thyroid are classified as adenomas or carcinomas.[37,38,39,40,41] Adenomas are benign growths that may cause enlargement of all or part of the gland, which extends to both sides of the neck and can be quite large. Some of these tumors may secrete hormones. Transformation to a malignant carcinoma may occur in some cells, which then may grow and spread to lymph nodes in the neck or to the lungs. Thyroid nodules in children are five times more likely to be malignant than those in adults.[42]