Esthesioneuroblastoma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Medical Therapy
Historically, surgery, radiation therapy (RT), and/or chemotherapy have all been used in the treatment of primary olfactory neuroblastomas [38]. Observational studies generally indicate that combining surgery and RT has resulted in prolonged disease-free and overall survival compared with either surgery or RT alone.
Radiation therapy
RT alone has been used for the initial treatment of patients with olfactory neuroblastoma in a number of series, but results have generally been less satisfactory than when RT is used in combination with surgery
Surgery plus radiation therapy
A combined otolaryngologic and neurosurgical anterior craniofacial resection followed by postoperative radiotherapy is the most widely used approach for patients with localized olfactory neuroblastoma [3,4,6,23,38,52,53]. A minimum dose of at least 54 Gy in 30 treatments over six weeks is recommended [54,55].
Adjuvant Chemotherapy
The role of chemotherapy, either before or after RT or surgery, is unclear. Numerous studies have used various chemotherapy regimens in an effort to improve outcomes [57-61]. However, it is unclear whether this actually improves results compared with a combined craniofacial resection and RT. In one study of 11 children, 10 received chemotherapy prior to local therapy with a five-year overall survival in the group of 91 percent [62].
Systemic disease
The rarity of olfactory neuroblastomas, combined with the favorable prognosis following aggressive local regional therapy, has resulted in only very limited experience for patients with disseminated disease. Cytotoxic chemotherapy appears to have activity in some patients, and newer molecularly targeted approaches may become an option as the biology of olfactory neuroblastomas is better understood.
Cytotoxic chemotherapy — A variety of chemotherapy agents have been evaluated in small series. These reports have included a mixture of patients with disseminated disease and with locoregional disease where chemotherapy was used alone or in combination with surgery and/or RT.
Cisplatin-based combination regimens (particularly cisplatin and etoposide) have often been chosen, primarily because of their activity in patients with head and neck squamous cell cancer or related neuroendocrine type tumors [5,64-73]. Non-platinum combinations, such as irinotecan plus docetaxel or doxorubicin, ifosfamide, and vincristine, may also be active [72,73]. Responses in patients with disseminated disease have generally been of short duration.