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
Surgery, radiation therapy (RT), and/or chemotherapy have all been used in the treatment of primary olfactory neuroblastomas.[1] [38]. Observational studies have indicated that combining surgery and radiotherapy RT has resulted in prolonged disease-free and overall survival compared with either surgery or radiotherapy RT alone.
Surgery
Surgical resection of esthesioneuroblastoma originally used a transfacial approach. However, various multiple observational studies have found that a combined craniofacial approach improved the ability to achieve an en bloc resection and resulted in better local control of disease and improved survival compared with a transfacial approach.[2][3][1][4]
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.
References
- ↑ 1.0 1.1 Nichols AC, Chan AW, Curry WT, Barker FG, Deschler DG, Lin DT (2008). "Esthesioneuroblastoma: the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy". Skull Base. 18 (5): 327–37. doi:10.1055/s-2008-1076098. PMC 2637063. PMID 19240832.
- ↑ Dulguerov P, Calcaterra T (1992). "Esthesioneuroblastoma: the UCLA experience 1970-1990". Laryngoscope. 102 (8): 843–9. PMID 1495347.
- ↑ Dulguerov P, Allal AS, Calcaterra TC (2001). "Esthesioneuroblastoma: a meta-analysis and review". Lancet Oncol. 2 (11): 683–90. doi:10.1016/S1470-2045(01)00558-7. PMID 11902539.
- ↑ Levine PA, McLean WC, Cantrell RW (1986). "Esthesioneuroblastoma: the University of Virginia experience 1960-1985". Laryngoscope. 96 (7): 742–6. PMID 3724324.