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Esthesioneuroblastoma medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

The predominant therapy for esthesioneuroblastoma is surgical resection followed by postoperative irradiation. Adjunctive radiation/chemotherapy may be required. The optimal therapy for esthesioneuroblastoma depends on the stage at diagnosis and regional or distant metastasis.[1][2][3][4][5][6][7][8][9]

Medical Therapy

Surgery, radiation therapy (RT), and/or chemotherapy have all been used in the treatment of primary olfactory neuroblastomas.[5] 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.[4][5][10][11]

Radiation therapy

  • In a number of series, radiation therapy alone has been used for the initial treatment of patients with olfactory neuroblastoma, but results have generally been less satisfactory than when radiation therapy (RT) is used in combination with surgery
  • Standard techniques include 3-field technnique and a external megavoltage beam; an anterior port is combined with wedged lateral fields to provide a homogeneous dose distribution.
  • The dose of radiotherapy varies from 5500-6500cGy. The majority of patients receive less than 6000 cGy. These doses are close to or exceed the maximum radiation dose recommended for sensitive structures such as the optic chiasma, optic nerve, brainstem, retina, and lens. Therefore, these patients are susceptible to cataract formation and glaucoma.
  • A possible role of intensity-modulated radiotherapy, proton beam radiotherapy, and stereotactic radiation has been suggested. Several studies have reported that intensity-modulated radiotherapy can provide good tumor control with low rates of radiation-induced toxicity, in both adults and children. There are case reports which describe the use of CT-guided interstitial high-dose-rate brachytherapy.[12][5][13][14][15]
  • Proton beam therapy may be especially important in children with developing soft tissue, bone, and neurological structures. Proton beam therapy is also being studied as a way to intensify dose and thus improve tumor control particularly in patients with unresectable disease or positive margins. However, there was greater neurological toxicity in patients receiving charged particle therapy compared with those receiving photon therapy.[16]

Surgery plus radiation therapy

A combined neurological anterior craniofacial and otolaryngologic resection followed by postoperative radiotherapy is the most widely used approach for patients with localized olfactory neuroblastoma. A minimum dose of at least 54 Gy in 30 treatments over six weeks is recommended for treatment of esthesioneuroblastoma.[1][2][3][4][5][6][7][8][9]

Adjuvant Chemotherapy

The role of chemotherapy, either before or after radiotherapy (RT) or surgery, is unclear. Many studies have used various chemotherapy regimens in an effort to improve outcomes. However, it is still unclear whether this actually improves results compared with a combined radiotherapy RT and craniofacial resection.[12][17][18][19][20][21]

Systemic disease

Because of the rarity of olfactory neuroblastomas, combined with the favorable prognosis following aggressive local regional therapy, there is 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 various case series. These reports have included a mixture of patients with locoregional disease and disseminated disease where chemotherapy was used alone or in combination with radiotherapy RT and/or surgery. Cisplatin-based combination regimens (particularly cisplatin and etoposide) have often been chosen, because of their activity in patients with head and neck squamous cell cancer (SCC) or related neuroendocrine type tumors. Non-platinum combinations, such as irinotecan plus docetaxel or doxorubicin, vincristine, and [ifosfamide]], may also be active. Generally, responses in patients with disseminated disease have been of short duration.
  • Molecularly targeted therapy — An understanding of the molecular pathogenesis of esthesioneuroblastomas may lead to the use of targeted therapies in patients with advanced disease:

References

  1. ↑ 1.0 1.1 Ward PD, Heth JA, Thompson BG, Marentette LJ (2009). “Esthesioneuroblastoma: Results and Outcomes of a Single Institution’s Experience”. Skull Base. 19 (2): 133–40. doi:10.1055/s-0028-1096195. PMC 2671304. PMID 19721769.
  2. ↑ 2.0 2.1 Diaz EM, Johnigan RH, Pero C, El-Naggar AK, Roberts DB, Barker JL; et al. (2005). “Olfactory neuroblastoma: the 22-year experience at one comprehensive cancer center”. Head Neck. 27 (2): 138–49. doi:10.1002/hed.20127. PMID 15654688.
  3. ↑ 3.0 3.1 Bachar G, Goldstein DP, Shah M, Tandon A, Ringash J, Pond G; et al. (2008). “Esthesioneuroblastoma: The Princess Margaret Hospital experience”. Head Neck. 30 (12): 1607–14. doi:10.1002/hed.20920. PMID 18798301.
  4. ↑ 4.0 4.1 4.2 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.
  5. ↑ 5.0 5.1 5.2 5.3 5.4 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.
  6. ↑ 6.0 6.1 Lund VJ, Howard D, Wei W, Spittle M (2003). “Olfactory neuroblastoma: past, present, and future?”. Laryngoscope. 113 (3): 502–7. doi:10.1097/00005537-200303000-00020. PMID 12616204.
  7. ↑ 7.0 7.1 Theilgaard SA, Buchwald C, Ingeholm P, Kornum Larsen S, Eriksen JG, Sand Hansen H (2003). “Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000”. Acta Otolaryngol. 123 (3): 433–9. PMID 12737303.
  8. ↑ 8.0 8.1 Ozsahin M, Gruber G, Olszyk O, Karakoyun-Celik O, Pehlivan B, Azria D; et al. (2010). “Outcome and prognostic factors in olfactory neuroblastoma: a rare cancer network study”. Int J Radiat Oncol Biol Phys. 78 (4): 992–7. doi:10.1016/j.ijrobp.2009.09.019. PMID 20231062.
  9. ↑ 9.0 9.1 Foote RL, Morita A, Ebersold MJ, Olsen KD, Lewis JE, Quast LM; et al. (1993). “Esthesioneuroblastoma: the role of adjuvant radiation therapy”. Int J Radiat Oncol Biol Phys. 27 (4): 835–42. PMID 8244813.
  10. ↑ Dulguerov P, Calcaterra T (1992). “Esthesioneuroblastoma: the UCLA experience 1970-1990”. Laryngoscope. 102 (8): 843–9. PMID 1495347.
  11. ↑ Levine PA, McLean WC, Cantrell RW (1986). “Esthesioneuroblastoma: the University of Virginia experience 1960-1985”. Laryngoscope. 96 (7): 742–6. PMID 3724324.
  12. ↑ 12.0 12.1 Fitzek MM, Thornton AF, Varvares M, Ancukiewicz M, Mcintyre J, Adams J; et al. (2002). “Neuroendocrine tumors of the sinonasal tract. Results of a prospective study incorporating chemotherapy, surgery, and combined proton-photon radiotherapy”. Cancer. 94 (10): 2623–34. PMID 12173330.
  13. ↑ Madani I, Bonte K, Vakaet L, Boterberg T, De Neve W (2009). “Intensity-modulated radiotherapy for sinonasal tumors: Ghent University Hospital update”. Int J Radiat Oncol Biol Phys. 73 (2): 424–32. doi:10.1016/j.ijrobp.2008.04.037. PMID 18755554.
  14. ↑ Sterzing F, Stoiber EM, Nill S, Bauer H, Huber P, Debus J; et al. (2009). “Intensity modulated radiotherapy (IMRT) in the treatment of children and adolescents–a single institution’s experience and a review of the literature”. Radiat Oncol. 4: 37. doi:10.1186/1748-717X-4-37. PMC 2760561. PMID 19775449.
  15. ↑ Tselis N, Heyd R, Baghi M, Zamboglou N (2008). “Interstitial high-dose-rate-brachytherapy in advanced esthesioneuroblastoma”. Laryngoscope. 118 (11): 2006–10. doi:10.1097/MLG.0b013e3181801d05. PMID 18641524.
  16. ↑ Lucas JT, Ladra MM, MacDonald SM, Busse PM, Friedmann AM, Ebb DH; et al. (2015). “Proton therapy for pediatric and adolescent esthesioneuroblastoma”. Pediatr Blood Cancer. 62 (9): 1523–8. doi:10.1002/pbc.25494. PMID 25820437.
  17. ↑ Eich HT, Hero B, Staar S, Micke O, Seegenschmiedt H, Mattke A; et al. (2003). “Multimodality therapy including radiotherapy and chemotherapy improves event-free survival in stage C esthesioneuroblastoma”. Strahlenther Onkol. 179 (4): 233–40. doi:10.1007/s00066-003-1089-x. PMID 12707712.
  18. ↑ Zappia JJ, Carroll WR, Wolf GT, Thornton AF, Ho L, Krause CJ (1993). “Olfactory neuroblastoma: the results of modern treatment approaches at the University of Michigan”. Head Neck. 15 (3): 190–6. PMID 8491582.
  19. ↑ Loy AH, Reibel JF, Read PW, Thomas CY, Newman SA, Jane JA; et al. (2006). “Esthesioneuroblastoma: continued follow-up of a single institution’s experience”. Arch Otolaryngol Head Neck Surg. 132 (2): 134–8. doi:10.1001/archotol.132.2.134. PMID 16490869.
  20. ↑ Argiris A, Dutra J, Tseke P, Haines K (2003). “Esthesioneuroblastoma: the Northwestern University experience”. Laryngoscope. 113 (1): 155–60. doi:10.1097/00005537-200301000-00029. PMID 12514401.
  21. ↑ El Kababri M, Habrand JL, Valteau-Couanet D, Gaspar N, Dufour C, Oberlin O (2014). “Esthesioneuroblastoma in children and adolescent: experience on 11 cases with literature review”. J Pediatr Hematol Oncol. 36 (2): 91–5. doi:10.1097/MPH.0000000000000095. PMID 24390450.

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