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Periorbital Skull Base Neoplasms

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Orbital Apex and Periorbital Skull Base Diseases

Abstract

The anatomical relationship of the orbit and specifically the orbital apex to the skull base makes it vulnerable to neoplastic invasion or extension by skull base and primary periorbital tumors. These lesions, although rare, provide a challenge in terms of diagnosis and treatment given their location. Patients may present with proptosis, ophthalmoplegia, and/or vision loss. Workup involves imaging and biopsy in many cases. They can range from benign histology such as meningiomas to neoplastic pathologies such as aggressive sarcomas. Surgery remains the primary treatment modality in most cases. Characteristics of several tumors and neoplasms that may involve the orbit or orbital apex are discussed in this chapter including sphenoid wing meningiomas, trigeminal schwannomas, sellar region tumors, sinonasal tumors, cavernous angiomas, lymphangiomas, lymphomas, soft tissue neoplasms, and metastases.

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References

  1. Kannan S, Hasegawa M, Yamada Y, Kawase T, Kato Y. Tumors of the orbit: case report and review of surgical corridors and current options. Asian J Neurosurg. 2019;14(3):678–85. https://doi.org/10.4103/ajns.AJNS_51_19.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Jørgensen M, Heegaard S. A review of nasal, paranasal, and skull base tumors invading the orbit. Surv Ophthalmol. 2018;63(3):389–405. https://doi.org/10.1016/j.survophthal.2017.07.001.

    Article  PubMed  Google Scholar 

  3. Gonen L, Nov E, Shimony N, Shofty B, Margalit N. Sphenoorbital meningioma: surgical series and design of an intraoperative management algorithm. Neurosurg Rev. 2018;41(1):291–301. https://doi.org/10.1007/s10143-017-0855-7.

    Article  PubMed  Google Scholar 

  4. Saeed P, Rootman J, Nugent RA, White VA, Mackenzie IR, Koornneef L. Optic nerve sheath meningiomas. Ophthalmology. 2003;110(10):2019–30. https://doi.org/10.1016/S0161-6420(03)00787-5.

    Article  PubMed  Google Scholar 

  5. Kiyofuji S, Casabella AM, Graffeo CS, Perry A, Garrity JA, Link MJ. Sphenoorbital meningioma: a unique skull base tumor. Surgical technique and results. J Neurosurg. 2019;23:1–8. https://doi.org/10.3171/2019.6.JNS191158.

    Article  Google Scholar 

  6. Hakuba A, Tanaka K, Suzuki T, Nishimura S. A combined orbitozygomatic infratemporal epidural and subdural approach for lesions involving the entire cavernous sinus. J Neurosurg. 1989;71(5 Pt 1):699–704. https://doi.org/10.3171/jns.1989.71.5.0699.

    Article  CAS  PubMed  Google Scholar 

  7. Honeybul S, Neil-Dwyer G, Lang DA, Evans BT, Ellison DW. Sphenoid wing meningioma en plaque: a clinical review. Acta Neurochir (Wien). 2001;143(8):749–757; discussion 758. https://doi.org/10.1007/s007010170028.

    Article  CAS  PubMed  Google Scholar 

  8. Shrivastava RK, Sen C, Costantino PD, Rocca RD. Sphenoorbital meningiomas: surgical limitations and lessons learned in their long-term management. J Neurosurg. 2005;103(3):491–7. https://doi.org/10.3171/jns.2005.103.3.0491.

    Article  PubMed  Google Scholar 

  9. Boari N, Gagliardi F, Spina A, Bailo M, Franzin A, Mortini P. Management of spheno-orbital en plaque meningiomas: clinical outcome in a consecutive series of 40 patients. Br J Neurosurg. 2013;27(1):84–90. https://doi.org/10.3109/02688697.2012.709557.

    Article  PubMed  Google Scholar 

  10. Talacchi A, De Carlo A, D’Agostino A, Nocini P. Surgical management of ocular symptoms in spheno-orbital meningiomas. Is orbital reconstruction really necessary? Neurosurg Rev. 2014;37(2):301–9; discussion 309–10. https://doi.org/10.1007/s10143-014-0517-y.

    Article  PubMed  Google Scholar 

  11. Kim BS, Im Y-S, Woo KI, Kim Y-D, Lee J-I. Multisession gamma knife radiosurgery for orbital apex tumors. World Neurosurg. 2015;84(4):1005–13. https://doi.org/10.1016/j.wneu.2015.04.042.

    Article  PubMed  Google Scholar 

  12. Pollack IF, Sekhar LN, Jannetta PJ, Janecka IP. Neurilemomas of the trigeminal nerve. J Neurosurg. 1989;70(5):737–45. https://doi.org/10.3171/jns.1989.70.5.0737.

    Article  CAS  PubMed  Google Scholar 

  13. Goel A, Muzumdar D, Raman C. Trigeminal neuroma: analysis of surgical experience with 73 cases. Neurosurgery. 2003;52(4):783–90; discussion 790. https://doi.org/10.1227/01.neu.0000053365.05795.03.

    Article  PubMed  Google Scholar 

  14. Taha JM, Tew JM, van Loveren HR, Keller JT, el-Kalliny M. Comparison of conventional and skull base surgical approaches for the excision of trigeminal neurinomas. J Neurosurg. 1995;82(5):719–25. https://doi.org/10.3171/jns.1995.82.5.0719.

    Article  CAS  PubMed  Google Scholar 

  15. Ghosh S, Das D, Varshney R, Nandy S. Orbital extension of trigeminal schwannoma. J Neurosci Rural Pract. 2015;6(1):102–4. https://doi.org/10.4103/0976-3147.143214.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Gupta P, Sharma A, Singh J. Solid cystic trigeminal schwannoma with intraorbital extension causing proptosis and vision loss. Asian J Neurosurg. 2016;11(4):456. https://doi.org/10.4103/1793-5482.181142.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Masroor FA, Gilde J, Liang J. Vidian nerve schwannoma: a rare skull-base neoplasm presenting with ocular manifestations: a case report and literature review. Perm J. 2018;22:18–021. https://doi.org/10.7812/TPP/18-021.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Kapur R, Mafee MF, Lamba R, Edward DP. Orbital schwannoma and neurofibroma: role of imaging. Neuroimaging Clin North Am. 2005;15(1):159–74. https://doi.org/10.1016/j.nic.2005.02.004.

    Article  Google Scholar 

  19. Fukaya R, Yoshida K, Ohira T, Kawase T. Trigeminal schwannomas: experience with 57 cases and a review of the literature. Neurosurg Rev. 2010;34(2):159–71. https://doi.org/10.1007/s10143-010-0289-y.

    Article  PubMed  Google Scholar 

  20. Samii M, Migliori MM, Tatagiba M, Babu R. Surgical treatment of trigeminal schwannomas. J Neurosurg. 1995;82(5):711–8. https://doi.org/10.3171/jns.1995.82.5.0711.

    Article  CAS  PubMed  Google Scholar 

  21. Shin SS, Gardner PA, Stefko ST, Madhok R, Fernandez-Miranda JC, Snyderman CH. Endoscopic endonasal approach for nonvestibular schwannomas. Neurosurgery. 2011;69(5):1046–1057; discussion 1057. https://doi.org/10.1227/NEU.0b013e3182287bb9.

    Article  PubMed  Google Scholar 

  22. Zoli M, Ratti S, Guaraldi F, et al. Endoscopic endonasal approach to primitive Meckel’s cave tumors: a clinical series. Acta Neurochir (Wien). 2018;160(12):2349–61. https://doi.org/10.1007/s00701-018-3708-4.

    Article  PubMed  Google Scholar 

  23. Park HH, Hong SD, Kim YH, et al. Endoscopic transorbital and endonasal approach for trigeminal schwannomas: a retrospective multicenter analysis (KOSEN-005). J Neurosurg. 2020;133(2):467–76. https://doi.org/10.3171/2019.3.JNS19492.

    Article  PubMed  Google Scholar 

  24. Li M, Wang X, Chen G, et al. Trigeminal schwannoma: a single-center experience with 43 cases and review of literature. Br J Neurosurg. 2021;35(1):49–56. https://doi.org/10.1080/02688697.2020.1754334.

    Article  PubMed  Google Scholar 

  25. Sheehan J, Yen CP, Arkha Y, Schlesinger D, Steiner L. Gamma knife surgery for trigeminal schwannoma. J Neurosurg. 2007;106(5):839–45. https://doi.org/10.3171/jns.2007.106.5.839.

    Article  PubMed  Google Scholar 

  26. Levy RA, Quint DJ. Giant pituitary adenoma with unusual orbital and skull base extension. AJR Am J Roentgenol. 1998;170(1):194–6. https://doi.org/10.2214/ajr.170.1.9423631.

    Article  CAS  PubMed  Google Scholar 

  27. Naguib MM, Mendoza PR, Jariyakosol S, Grossniklaus HE. Atypical pituitary adenoma with orbital invasion: case report and review of the literature. Surv Ophthalmol. 2017;62(6):867–74. https://doi.org/10.1016/j.survophthal.2017.01.005.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Dhaliwal JS, Seibold LK, Kleinschmidt-Demasters BK, et al. Orbital invasion by ACTH-secreting pituitary adenomas. Ophthalmic Plast Reconstr Surg. 2014;30(2):e28–30. https://doi.org/10.1097/IOP.0b013e31829164cb.

    Article  PubMed  Google Scholar 

  29. Karcioglu ZA, Aden LB, Cruz AAV, Zaslow L, Saloom RJ. Orbital invasion with prolactinoma: a clinical review of four patients. Ophthalmic Plast Reconstr Surg. 2002;18(1):64–71. https://doi.org/10.1097/00002341-200201000-00010.

    Article  PubMed  Google Scholar 

  30. Vaudaux J, Portmann L, Maeder P, Borruat F-X. Orbital invasion by a pituitary macroadenoma without visual loss: case report and review of the literature. Eye (Lond). 2003;17(9):1032–4. https://doi.org/10.1038/sj.eye.6700481.

    Article  CAS  PubMed  Google Scholar 

  31. Rehman L, Bokhari I, Siddiqi SU, Bagga V, Hussain MM. Intracranial epidermoid lesions: our experience of 38 cases. Turk Neurosurg. 2017; https://doi.org/10.5137/1019-5149.JTN.21095-17.0.

  32. Pushker N, Meel R, Kumar A, Kashyap S, Sen S, Bajaj MS. Orbital and periorbital dermoid/epidermoid cyst: a series of 280 cases and a brief review. Can J Ophthalmol. 2020;55(2):167–71. https://doi.org/10.1016/j.jcjo.2019.08.005.

    Article  PubMed  Google Scholar 

  33. Vahdani K, Rose GE. Ophthalmic presentation and outcomes for malignant sinonasal tumors. Ophthalmic Plast Reconstr Surg. 2021; https://doi.org/10.1097/IOP.0000000000001972.

  34. Suárez C, Ferlito A, Lund VJ, et al. Management of the orbit in malignant sinonasal tumors. Head Neck. 2008;30(2):242–50. https://doi.org/10.1002/hed.20736.

    Article  PubMed  Google Scholar 

  35. Iannetti G, Valentini V, Rinna C, Ventucci E, Marianetti TM. Ethmoido-orbital tumors: our experience. J Craniofac Surg. 2005;16(6):1085–91. https://doi.org/10.1097/01.scs.0000164332.81428.ba.

    Article  PubMed  Google Scholar 

  36. Cracchiolo JR, Patel K, Migliacci JC, et al. Factors associated with a primary surgical approach for sinonasal squamous cell carcinoma. J Surg Oncol. 2018;117(4):756–64. https://doi.org/10.1002/jso.24923.

    Article  PubMed  Google Scholar 

  37. Ganly I, Patel SG, Singh B, et al. Craniofacial resection for malignant tumors involving the skull base in the elderly: an international collaborative study. Cancer. 2011;117(3):563–71. https://doi.org/10.1002/cncr.25390.

    Article  PubMed  Google Scholar 

  38. Reyes C, Mason E, Solares CA, Bush C, Carrau R. To preserve or not to preserve the orbit in paranasal sinus neoplasms: a meta-analysis. J Neurol Surg B Skull Base. 2015;76(2):122–8. https://doi.org/10.1055/s-0034-1390403.

    Article  PubMed  Google Scholar 

  39. Akhter AS, El Tecle N, Alexopoulos G, Espinoza G, Coppens J. Intraosseous orbital cavernous hemangioma with frontal extension and dural involvement. Cureus. 2019;11(6):e4823. https://doi.org/10.7759/cureus.4823.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Deng C, Hu W. Multiple cavernous hemangiomas in the orbit: a case report and review of the literature. Medicine (Baltimore). 2020;99(29):e20670. https://doi.org/10.1097/MD.0000000000020670.

    Article  PubMed  Google Scholar 

  41. Hentati A, Matar N, Dridi H, Bouali S, Jemel H. Bilateral orbital cavernous hemangioma. Asian J Neurosurg. 2018;13(4):1222–4. https://doi.org/10.4103/ajns.AJNS_96_17.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Zhang L, Li X, Tang F, Gan L, Wei X. Diagnostic imaging methods and comparative analysis of orbital cavernous hemangioma. Front Oncol. 2020;10:577452. https://doi.org/10.3389/fonc.2020.577452.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Qin X, Akter F, Qin L, et al. Dumbbell shaped craniorbital cavernous hemangioma. BMC Neurol. 2020;20(1):149. https://doi.org/10.1186/s12883-020-01734-z.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Young SM, Kim KH, Kim Y-D, et al. Orbital apex venous cavernous malformation with optic neuropathy: treatment with multisession gamma knife radiosurgery. Br J Ophthalmol. 2019;103(10):1453–9. https://doi.org/10.1136/bjophthalmol-2018-312893.

    Article  PubMed  Google Scholar 

  45. Smith ET. A case of lymphangioma of the orbit. Trans Am Ophthalmol Soc. 1925;23:240–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  46. Patel KC, Kalantzis G, El-Hindy N, Chang BY. Sclerotherapy for orbital lymphangioma - case series and literature review. In Vivo. 2017;31(2):263–6. https://doi.org/10.21873/invivo.11055.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Thavara BD, Rajagopalawarrier B, Balakrishnan S, Kidangan GS. A case of adult orbital intraconal lymphangioma. Asian J Neurosurg. 2020;15(1):168–71. https://doi.org/10.4103/ajns.AJNS_282_19.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Woo YJ, Kim CY, Sgrignoli B, Yoon JS. Orbital lymphangioma: characteristics and treatment outcomes of 12 cases. Korean J Ophthalmol. 2017;31(3):194–201. https://doi.org/10.3341/kjo.2016.0034.

    Article  PubMed  PubMed Central  Google Scholar 

  49. Sen S, Singh P, Bajaj MS, Gaur N. Management of a case of orbital lymphangioma presenting in adulthood with negative-pressure aspiration and bleomycin injection. BMJ Case Rep. 2019;12(6) https://doi.org/10.1136/bcr-2018-227697.

  50. Kothari P, Mirani N, Langer PD. Orbital lymphangioma confined to an extraocular muscle. Ophthalmic Plast Reconstr Surg. 2020;36(3):e61–2. https://doi.org/10.1097/IOP.0000000000001572.

    Article  PubMed  Google Scholar 

  51. Lagrèze WA, Joachimsen L, Gross N, Taschner C, Rössler J. Sirolimus-induced regression of a large orbital lymphangioma. Orbit. 2019;38(1):79–80. https://doi.org/10.1080/01676830.2018.1436569.

    Article  PubMed  Google Scholar 

  52. Bagheri A, Amoohashemi N, Salour H, Yazdani S. Lacrimal gland lymphangioma: report of a case and review of literature. Orbit. 2012;31(3):197–9. https://doi.org/10.3109/01676830.2011.648807.

    Article  PubMed  Google Scholar 

  53. Tsen C-L, Lin M-C, Bee Y-S, Chen J-L, Kuo N-W, Sheu S-J. Ocular adnexal lymphoma: five case reports and a literature review. Taiwan J Ophthalmol. 2015;5(2):99–102. https://doi.org/10.1016/j.tjo.2014.05.007.

    Article  PubMed  Google Scholar 

  54. Yuen CA, Pula JH, Mehta M. Primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue (MALT) lymphoma presenting as orbital apex syndrome. Neuroophthalmology. 2017;41(2):94–8. https://doi.org/10.1080/01658107.2016.1263343.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Jaiswal AK, Tripathi M, Chandra PS, Sharma MC, Mahapatra AK. An unusual case of primary lymphoma of the skull base extending from cerebellopontine angle to cavernous sinus and orbit. A case report. J Neurosurg Sci. 2000;44(3):145–148; discussion 149.

    CAS  PubMed  Google Scholar 

  56. Alkatan HM, Alaraj AM, Al-Ayoubi A. Diffuse large B-cell lymphoma of the orbit: a tertiary eye care center experience in Saudi Arabia. Saudi J Ophthalmol. 2012;26(2):235–9. https://doi.org/10.1016/j.sjopt.2011.09.004.

    Article  PubMed  Google Scholar 

  57. Yazıcı B, Sabur H, Yazıcı Z. Orbital cavitary rhabdomyosarcoma: case report and literature review. Ophthalmic Plast Reconstr Surg. 2014;30(1):e20–2. https://doi.org/10.1097/IOP.0b013e31828de376.

    Article  PubMed  Google Scholar 

  58. Dziedzic TA, Anand VK, Schwartz TH. Endoscopic endonasal approach to the lateral orbital apex: case report. J Neurosurg Pediatr. 2015;16(3):305–8. https://doi.org/10.3171/2015.2.PEDS1575.

    Article  PubMed  Google Scholar 

  59. Chaugule SS, Putambekar A, Gavade S, Deshpande R. Primary orbital leiomyosarcoma in an adult male. Ophthalmic Plast Reconstr Surg. 2019;35(2):e27–9. https://doi.org/10.1097/IOP.0000000000001293.

    Article  PubMed  Google Scholar 

  60. Jakhetiya A, Shukla NK, Muduly D, Kale SS. Extraskeletal orbital mesenchymal chondrosarcoma: surgical approach and mini review. BMJ Case Rep. 2017; https://doi.org/10.1136/bcr-2016-218744.

  61. Broggi G, Salvatorelli L, Reibaldi M, et al. Solitary fibrous tumor of the orbital region: report of a case with emphasis on the diagnostic utility of STAT-6. Pathologica. 2020;112(4):195–9. https://doi.org/10.32074/1591-951X-9-20.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Mitamura M, Kase S, Suzuki Y, et al. Solitary fibrous tumor of the orbit: a clinicopathologic study of two cases with review of the literature. In Vivo. 2020;34(6):3649–54. https://doi.org/10.21873/invivo.12211.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Sayit AT, Elmali M, Gul A, Sullu Y. Solitary fibrous tumor of the orbit: computed tomography and histopathological findings. J Cancer Res Ther. 2019;15(3):719–21. https://doi.org/10.4103/jcrt.JCRT_1194_16.

    Article  PubMed  Google Scholar 

  64. Alkatan HM, Alsalamah AK, Almizel A, et al. Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description. Ann Saudi Med. 2020;40(3):227–33. https://doi.org/10.5144/0256-4947.2020.227.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Brum M, Nzwalo H, Oliveira E, et al. Solitary fibrous tumors of the orbit and central nervous system: a case series analysis. Asian J Neurosurg. 2018;13(2):336–40. https://doi.org/10.4103/ajns.AJNS_111_16.

    Article  PubMed  PubMed Central  Google Scholar 

  66. Crawford L, Sharma S. Orbital apex metastasis from adenoid cystic carcinoma: acute loss of vision and subsequent recovery with the radiation. Cureus. 2017;9(11):e1869. https://doi.org/10.7759/cureus.1869.

    Article  PubMed  PubMed Central  Google Scholar 

  67. Hirunwiwatkul P, Tirakunwichcha S, Meesuaypong P, Shuangshoti S. Orbital metastasis of hepatocellular carcinoma. J Neuroophthalmol. 2008;28(1):47–50. https://doi.org/10.1097/WNO.0b013e31816754e7.

    Article  PubMed  Google Scholar 

  68. Kagusa H, Mizobuchi Y, Nakajima K, Fujihara T, Bando Y, Takagi Y. Metastatic tumor to the orbital cavity from a primary carcinoma of the uterine cervix: a case report. J Med Invest. 2019;66(3.4):355–7. https://doi.org/10.2152/jmi.66.355.

    Article  PubMed  Google Scholar 

  69. Kim HJ, Wojno TH, Grossniklaus H. Atypical bilateral orbital metastases of lobular breast carcinoma. Ophthalmic Plast Reconstr Surg. 2012;28(6):e142–3. https://doi.org/10.1097/IOP.0b013e318249d5c0.

    Article  PubMed  Google Scholar 

  70. Pecen PE, Ramey NA, Richard MJ, Bhatti MT. Metastatic pancreatic carcinoma to the orbital apex presenting as a superior divisional third cranial nerve palsy. Clin Ophthalmol. 2012;6:1941–3. https://doi.org/10.2147/OPTH.S30208.

    Article  PubMed  PubMed Central  Google Scholar 

  71. Tsuruta Y, Maeda Y, Kitaguchi Y, et al. A case of endonasal endoscopic surgery for intraorbital metastasis of gastric ring cell carcinoma. Ear Nose Throat J. 2020;2020:145561320943372. https://doi.org/10.1177/0145561320943372.

    Article  Google Scholar 

  72. Wada K, Tsuda T, Hanada Y, Maeda Y, Mori K, Nishimura H. A rare case of prostate carcinoma metastasis in the orbital apex. Ear Nose Throat J. 2020;2020:145561320973783. https://doi.org/10.1177/0145561320973783.

    Article  Google Scholar 

  73. Wadhwani M, Phuljhele S, Kumar R, Shameer A. Cervical carcinoma leading to orbital apex syndrome and blindness. BMJ Case Rep. 2019;12(3):e226587. https://doi.org/10.1136/bcr-2018-226587.

    Article  PubMed  PubMed Central  Google Scholar 

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Dowlati, E., Fleisher, M., Jean, W.C. (2023). Periorbital Skull Base Neoplasms. In: POON, T.L., MAK, C., YUEN, H.K.L. (eds) Orbital Apex and Periorbital Skull Base Diseases. Springer, Singapore. https://doi.org/10.1007/978-981-99-2989-4_13

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