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

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(Refer to the PDQ summary on Nasopharyngeal Cancer Treatment for more information.)

Esthesioneuroblastoma

Esthesioneuroblastoma (olfactory neuroblastoma) is a small round-cell tumor arising from the nasal neuroepithelium that is distinct from primitive neuroectodermal tumors.[23,24,25,26] In children, esthesioneuroblastoma is a very rare malignancy with an estimated incidence of 0.1 per 100,000 children younger than 15 years.[27] Despite its rarity, esthesioneuroblastoma is the most common cancer of the nasal cavity in pediatric patients, accounting for 28% of all cases.[27,28] In a series of 511 patients from the SEER database, there was a slight male predominance, the mean age at presentation was 53 years, and only 8% of cases were younger than 25 years.[29] Most patients were white (81%) and the most common tumor sites were the nasal cavity (72%) and ethmoid sinus (13%).[29]

Most children present in the second decade of life with symptoms that include nasal obstruction, epistaxis, hyposmia, exophthalmos, or a nasopharyngeal mass, which may have local extension into the orbits, sinuses, or frontal lobe. Most patients present with advanced-stage disease (Kadish stages B and C).[27,28] Recent reports suggest that positron emission tomography–computed tomography (PET-CT) may aid in staging the disease.[30]

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.[31]

The mainstay of treatment has been surgery and radiation.[32] Newer techniques such as endoscopic sinus surgery may offer similar short-term outcomes to open craniofacial resection.[29] Other techniques such as stereotactic radiosurgery and proton-beam therapy (charged-particle radiation therapy) may also play a role in the management of this tumor.[33] Nodal metastases are seen in about 5% of patients. Routine neck dissection and nodal exploration are not indicated in the absence of clinical or radiological evidence of disease.[34] Management of cervical lymph node metastases has been addressed in a review article.[34]

Reports indicate the increasing use of neoadjuvant or adjuvant chemotherapy in patients with advanced-stage disease with promising results.[23,24,35,36,37]; [38][Level of evidence: 3iii] Chemotherapy regimens that have been used with efficacy include etoposide with ifosfamide and cisplatin;[39] vincristine, actinomycin D, and cyclophosphamide with and without doxorubicin; ifosfamide/etoposide; cisplatin plus etoposide or doxorubicin; [35] and irinotecan plus docetaxel.[40][Level of evidence: 3iiA]

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