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
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Vahdani K, Rose GE. Ophthalmic presentation and outcomes for malignant sinonasal tumors. Ophthalmic Plast Reconstr Surg. 2021; https://doi.org/10.1097/IOP.0000000000001972.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Smith ET. A case of lymphangioma of the orbit. Trans Am Ophthalmol Soc. 1925;23:240–6.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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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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