Abstract
The orbit and ocular adnexa are important sites for primary and secondary orbital diseases. All tissue types including bone, vascular, neural, muscular, and glandular tissues may be involved with specific pathologies. Tumors or inflammations can secondarily invade the orbit from periorbital regions such as the paranasal sinuses, eyelids, and intracranial compartment. The volume of the orbit is small and confined by bony walls on all sides except anteriorly, and within this space, numerous structures subserve visual functions. It is not surprising, therefore, that ophthalmic symptoms are common presenting findings with most orbital diseases. In this chapter, we will restrict our discussion to benign and malignant tumors and to some of the more common nonneoplastic lesions.
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Introduction
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Vascular Lesions: Cavernous Hemangioma
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Vascular Lesions: Lymphangioma
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Vascular Lesions: Varices
Beyer R, Levine MR, Sternberg I. Orbital varices: a surgical approach. Ophthal Plast Reconstr Surg. 1985;1:205–10.
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Vascular Lesions: Angiosarcoma
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Neural Tumors: Carcinoid
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Neural Tumors: Malignant Peripheral Nerve Sheath Tumor
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Neural Tumors: Meningocele
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Neural Tumors: Neuroblastoma
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Neural Tumors: Optic Pathway Glioma
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Neural Tumors: Optic Nerve Sheath Meningioma
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Neural Tumors: Plexiform Neurofibroma
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Neural Tumors: Schwannoma
Allman M, Frayer W, Hedges T. Orbital neurilemoma. Ann Ophthalmol. 1977;9:1409–13.
Bavetta S, McFall MR, Afshar F, Hutchinson I. Schwannoma of the anterior cranial fossa and paranasal sinuses. Br J Neurosurg. 1993;7:697–700.
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Neural Tumors: Solitary Neurofibroma
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Neural Tumors: Sphenoid Wing Meningioma
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Mesenchymal Tumors: Fibrosarcoma
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Mesenchymal Tumors: Fibrous Histiocytoma
Al-Hazzaa SA, Specht CS, McLean IW, Holds JB, Anderson RL. Benign orbital fibrous histiocytoma simulating a lacrimal gland tumor. Ophthalmic Surg Lasers. 1996;27:140–2.
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Jakobiec FA, Klapper D, Maher E, Krebs W. Infantile subconjunctival and anterior orbital fibrous histiocytoma. Ultrastructural and immunohistochemical studies. Ophthalmology. 1988;95:516–25.
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Krohel GB, Gregor D. Fibrous histiocytoma. J Pediatr Ophthalmol. 1980;17:37–9.
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Mesenchymal Tumors: Juvenile Xanthogranuloma
Borrego O, Hidayat AA. Solitary juvenile xanthogranuloma of the orbit. Orbit. 1996;15:41–5.
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Mesenchymal Tumors: Leiomyosarcoma
Arora R, Betharia SM. Aspiration cytology of smooth-muscle tumours of the orbit. Orbit. 1990;9:35–41.
Bakri SJ, Krohel GB, Peters GB, Farber MG. Spermatic cord leiomyosarcoma metastatic to the orbit. Am J Ophthalmol. 2003;136:213–5.
Conlon MR, Rubin PA, Samy CN, Albert DM. Metastatic orbital leiomyosarcoma. Can J Ophthalmol. 1994;29:85–9.
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Folberg R, Cleaseby G, Flanagan JA, Spencer WH, Zimmerman LE. Orbital leiomyosarcoma after radiation therapy for bilateral retinoblastoma. Arch Ophthalmol. 1983;101:1562–5.
Font RL, Jurco S III, Brechner RJ. Postradiation leiomyosarcoma of the orbit complicating bilateral retinoblastoma. Arch Ophthalmol. 1983;101:1557–61.
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Jakobiec FA, Mitchell JP, Chauhan PM, Iwamoto T. Mesectodermal leiomyosarcoma of the antrum and orbit. Am J Ophthalmol. 1978;85:51–7.
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Mihara F, Gupta KL, Kartchner ZA, Kogutt MS, Robinson AE. Leiomyosarcoma after retinoblastoma radiotherapy. Radiat Med. 1991;9:183–4.
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Mesenchymal Tumors: Lipoblastomatosis
Adams RJ, Drwiega PJ, Rivera CA. Congenital orbital lipoblastoma: a pathologic and radiologic study. J Pediatr Ophthalmol Strabismus. 1997;34:194–6.
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Mesenchymal Tumors: Benign Cartilaginous Tumors
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Mesenchymal Tumors: Lipodermoid
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Mesenchymal Tumors: Lipoma
Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol. 1985;100:605–9.
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Mesenchymal Tumors: Liposarcoma
Abdalla MI, Ghaly AF, Hosni F. Liposarcoma with orbital metastases: case report. Br J Ophthalmol. 1966;50:426–8.
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Mesenchymal Tumors: Rhabdomyosarcoma
Abramson DH, Notis CM. Visual acuity after radiation for orbital rhabdomyosarcoma. Am J Ophthalmol. 1994;118:808–9.
Boparai MS, Dash RG. Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcoma with management. Indian J Ophthalmol. 1991;39:129–31.
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Mesenchymal Tumors: Solitary Fibrous Tumor
Alexandrakis G, Johnson TE. Recurrent orbital solitary fibrous tumor in a 14-year-old girl. Am J Ophthalmol. 2000;130:373–6.
Bernardini FP, de Conciliis C, Schneider S, et al. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology. 2003;110:1442–8.
Carrera M, Prat J, Quintana M. Malignant solitary fibrous tumor of the orbit. Report of a case with 8 years of follow-up. Eye. 2001;15:102–4.
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DeBacker CM, Bodker F, Putterman AM, Beckmann E. Solitary fibrous tumor of the orbit. Am J Ophthalmol. 1996;121:447–9.
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Tumors of Bone: Aneurysmal Bone Cyst
Bealer LA, Cibis GW, Barker BF, et al. Aneurysmal bone cyst: report of a case mimicking orbital tumor. J Pediatr Ophthalmol Strabismus. 1993;30:199–200.
Borkar SA, Kasliwal MK, Sinha S, Sharma BS. MR imaging in aneurismal bone cyst of the orbit. Turk Neurosurg. 2008;18:183–6.
Citardi MJ, Janjua T, Abrahams JJ, Sasaki CT. Orbitoethmoid aneurysmal bone cyst. Otolaryngol Head Neck Surg. 1996;114:466–70.
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Fite JD, Schwarts JF, Calhoun FP. Aneurysmal bone cyst of the orbit. A clinicopathologic case report. Trans Am Acad Ophthalmol Otolaryngol. 1968;72:614–8.
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Hunter JV, Yokoyama C, Moseley IF, Wright JE. Aneurysmal bone cyst of the sphenoid with orbital involvement. Br J Ophthalmol. 1990;74:505–8.
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Johnson TE, Bergin DJ, McCord CD. Aneurysmal bone cyst of the orbit. Ophthalmology. 1988;95:86–9.
Klepach GL, Ho RE, Kelly JK. Aneurysmal bone cyst of the orbit. A case report. J Clin Neuroophthalmol. 1984;4:49–52.
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Patel BC, Sabir DI, Flaharty PM, Anderson RL. Aneurysmal bone cyst of the orbit and ethmoid sinus. Arch Ophthalmol. 1993;111:586–7.
Powell JO, Glaser JS. Aneurysmal bone cyst of the orbit. Arch Ophthalmol. 1975;93:340–2.
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Tumors of Bone: Cholesterol Granuloma
Aferzon M, Millman B, O’Donnell TR. Cholesterol granuloma of the frontal bone. Otolaryngol Head Neck Surg. 2002;127:578–81.
Arat YO, Chaudhry IA, Boniuk M. Orbitofrontal cholesterol granuloma: distinct diagnostic features and management. Ophthal Plast Reconstr Surg. 2003;19:382–7.
Chow LP, McNab AA. Orbitofrontal cholesterol granuloma. J Clin Neurosci. 2005;12:206–9.
Daus W, Voges J, Schwechheimer K, et al. Cholesterol granuloma of the orbit. Klin Monatsbl Augenheilkd. 1988;193:195–9.
Dickey JB, Mullenix CD, O’Grady RB. Atypical magnetic resonance findings in an orbitofrontal cholesterol granuloma. Ophthal Plast Reconstr Surg. 1992;8:215–20.
Dobben GD, Philip B, Mafee MF, et al. Orbital subperiosteal hematoma, cholesterol granuloma, and infection. Radiol Clin N Am. 1998;36:1185–200.
Eijpe AA, Koornneef L, Verbeeten B Jr, et al. Cholesterol granuloma of the frontal bone: CT diagnosis. J Comput Assist Tomogr. 1990;14:914–7.
Elmaleh C, D’Hermies F, Barraco P, Pouliquen Y. Cholesteatoma of the orbit: a rare etiology of orbital tumor. J Fr Ophtalmol. 1989;12:139–42.
Fukata K, Jackson IT. Epidermal cyst and cholesterol granuloma of the orbit. Br J Surg. 1990;43:521–7.
Hill CA, Moseley IF. Imaging of orbitofrontal cholesterol granuloma. Clin Radiol. 1992;46:237–42.
Jordan DR, Spitellie P, Brownstein S. Orbital cholesterol granuloma and cholesteatoma: significance of differentiating the two. Ophthal Plast Reconstr Surg. 2007;23:415–7.
Kersten RC, Kersten JL, Bloom BR, Kulwin DR. Chronic hematic cyst of the orbit. Role of magnetic resonance imaging in diagnosis. Ophthalmology. 1988;95:1549–53.
McNab AA, Wright GE. Orbitofrontal cholesterol granuloma. Ophthalmology. 1990;97:28–32.
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Parke DW 2nd, Font RL, Boniuk M, McCrary JA 3rd. Cholesterol granuloma of the orbit. Arch Ophthalmol. 1992;100:612–6.
Prabhakaran VC, Hsuan J, Selva D. Endoscopic-assisted removal of orbital roof lesions via a skin crease approach. Skull Base. 2007;17:341–5.
Selva D, Chen C. Endoscopic approach to orbitofrontal cholesterol granuloma. Orbit. 2004;23:49–52.
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Tumors of Bone: Ewing’s Sarcoma
Bajaj MS, Pushker N, Sen S, et al. Primary Ewing’s sarcoma of the orbit: a rare presentation. J Pediatr Ophthalmol Strabismus. 2003;40:101–4.
Dutton JJ, Rose JG Jr, DeBacker CM, Gayre G. Orbital Ewing’s sarcoma of the orbit. Ophthal Plast Reconstr Surg. 2000;16:292–300.
Eggli KD, Quiogue TM, Moser RP. Ewing’s sarcoma. Radiol Clin N Am. 1993;31:325–37.
Frandsen E. Ewing’s sarcom I orbiti. Nord Med. 1952;48:1673.
Green DM, Marinello MJ, Fisher J, et al. Ewing’s sarcoma of the scapula with metastases to the lung and eye. Am J Pediatr Hematol Oncol. 1986;8:134–43.
Guzowski M, Tumuluri K, Walker DM, Maloof A. Primary orbital Ewing sarcoma in a middle-aged man. Ophthal Plast Reconstr Surg. 2005;21:449–51.
Harbert F, Tabor GL Jr. Ewing’s tumor of the orbit. Am J Ophthalmol. 1950;33:1219–55.
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Jereb B, Ong RL, Mohan M, Caparros B, Exelby P. Redefined role of radiation in combined treatment of Ewing’s sarcoma. Pediatr Hematol Oncol. 1986;3:11–118.
Kissane JM, Askin FB, Foulkes M, Stratton LB, Shirley SF. Ewing’s sarcoma of bone: clinicopathologic aspects of 303 cases from the intergroup Ewing’s sarcoma study. Hum Pathol. 1983;14:773–9.
Li T, Goldberg RA, Becker B, McCann J. Primary orbital extraskeletal Ewing sarcoma. Arch Ophthalmol. 2003;121:1049–51.
Mansfield JB. Primary Ewing’s sarcoma of the skull. Surg Neurol. 1982;18:286–8.
Pang NK, Bartley GB, Giannini C. Primary Ewing sarcoma of the orbit in an adult. Ophthal Plast Reconstr Surg. 2007;23:153–4.
Rootman J. Diseases of the orbit: a multidisciplinary approach. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2003. p. 299–301.
Sharma A, Garg A, Mishra NK, Gaikwad SB, et al. Primary Ewing’s sarcoma of the sphenoid bone with unusual imaging features: a case report. Neurol Neurosurg. 2005;107:528–31.
Wilson DJ, Dailey RA, Griffeth MT, Newton CJ. Primary Ewing sarcoma of the orbit. Ophthal Plast Reconstr Surg. 2001;17:300–3.
Woodruff G, Thorner P, Skarf B. Primary Ewing’s sarcoma of the orbit presenting with visual loss. Br J Ophthalmol. 1988;72:786–92.
Tumors of Bone: Fibrous Dysplasia
Bibby K, McFadzean R. Fibrous dysplasia of the orbit. Br J Ophthalmol. 1994;78:266–70.
Casselman JW, De Jonge I, Neyt L, De Clercq C, D’Hont G. MRI in craniofacial fibrous dysplasia. Neuroradiology. 1993;35:234–7.
Chung EM, Murphey MD, Specht CS, et al. From the archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit. Radiographics. 2008;28:1193.
Cruz AA, Constanzi M, de Castro FA, dos Santos AC. Apical involvement with fibrous dysplasia: implications for vision. Ophthal Plast Reconstr Surg. 2007;23:450–4.
Daffner RH, Kirks DR, Gehweiler JA, Heaston DK. Computed tomography of fibrous dysplasia. Am J Roentgenol. 1982;139:943–8.
Donoso LA, Margargal LE, Eiferman RA. Fibrous dysplasia of the orbit with optic nerve decompression. Ann Ophthalmol. 1982;14:80–3.
Gass JDM. Orbital and ocular involvement in fibrous dysplasia. Case report. South Med J. 1965;58:324–9.
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Katz BJ, Nerad JA. Ophthalmic manifestations of fibrous dysplasia: a disease of children and adults. Ophthalmology. 1998;105:2207–15.
Liakos GM, Walker CB, Carruth JAS. Ocular complications in craniofacial fibrous dysplasia. Br J Ophthalmol. 1979;63:611–6.
McCluskey P, Wingate R, Benger R, McCarthy S. Monostotic fibrous dysplasia of the orbit: an unusual lacrimal fossa mass. Br J Ophthalmol. 1993;77:54–6.
Melen O, Weinberg PE, Kim KS, et al. Fibrous dysplasia of bone with active visual loss. Ann Ophthalmol. 1980;12:734–9.
Moore RT. Fibrous dysplasia of the orbit. Review. Surv Ophthalmol. 1969;13:321–34.
Moore AT, Buncic JR, Munro IR. Fibrous dysplasia of the orbit in childhood. Clinical features and management. Ophthalmology. 1985;92:12–20.
Papay FA, Morlaes L Jr, Flaharty P, Smith SJ, et al. Optic nerve decompression in cranial base fibrous dysplasia. J Craniofac Surg. 1995;6:5–10. Discussion 11–14.
Ronner HJ, Trokel SL, Hilal SK. Acute blindness in a patient with fibrous dysplasia. Orbit. 1982;1:231–4.
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Weisman JS, Hepler RS, Vinters HV. Reversible visual loss caused by fibrous dysplasia. Am J Ophthalmol. 1990;110:244–9.
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Tumors of Bone: Giant Cell Reparative Granuloma
D’Ambrosio AL, Williams SC, Lignelli A, et al. Clinicopathological review: giant cell reparative granuloma of the orbit. Neurosurgery. 2005;57:773–8.
Font RL, Blanco G, Soparkar CN, et al. Giant cell reparative granuloma of the orbit associated with cherubism. Ophthalmology. 2003;110:1846–9.
Hirschl S, Katz A. Giant cell reparative granuloma outside the jawbone. Diagnostic criteria and review of the literature with the first case description in the temporal bone. Hum Pathol. 1974;5:171–81.
Hoopes PC, Anderson RL, Blodi FC. Giant cell (reparative) granuloma of the orbit. Ophthalmology. 1981;88:1361–6.
Hyver SW, Ellis DS, Stewart WB, Spencer WH, Bartlett PC. Sino-orbital giant cell reparative granuloma. Ophthal Plast Reconstr Surg. 1998;14:178–81.
Mercado GV, Shields CL, Gunduz K, et al. Giant cell reparative granuloma of the orbit. Am J Ophthalmol. 1999;127:485–7.
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Sebag J, Chapman P, Truman J, Riemersma RR. Giant cell granuloma of the orbit with intracranial extension. Neurosurgery. 1985;16:75–8.
Spraul CW, Wojno TH, Grossniklaus HE, Lang GK. Reparative giant cell granuloma with orbital involvement. Klin Monatsbl Augenheilkd. 1997;21:133–4.
Tumors of Bone: Ossifying Fibroma
Baumann I, Zimmerman R, Dammann F, et al. Ossifying fibroma of the ethmoid involving the orbit and the skull base. Otolaryngol Head Neck Surg. 2005;133:158–9.
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Fakadej A, Boynton JR. Juvenile ossifying fibroma of the orbit. Ophthal Plast Reconstr Surg. 1996;12:174–7.
Hartstein ME, Grove AS Jr, Woog JJ, Shore JW, Joseph MP. The multidisciplinary management of psammomatoid ossifying fibroma of the orbit. Ophthalmology. 1998;105:591–5.
Jordan DR, Farmer J, DaSilva V. Psammomatoid ossifying fibroma of the orbit. Can J Ophthalmol. 1992;27:194–6.
Khalil MK, Leib ML. Cemento-ossifying fibroma of the orbit. Can J Ophthalmol. 1979;14:195–200.
Margo CE, Ragsdale BD, Perman KI, Zimmerman LE, Sweet DE. Psammomatoid (juvenile) ossifying fibroma of the orbit. Ophthalmology. 1985;92:150–9.
Margo CE, Weiss A, Habal MB. Psammomatoid ossifying fibroma. Arch Ophthalmol. 1986;104:1347.
Shields JA, Nelson LB, Brown JF, Dolinskas C. Clinical, computed tomographic, and histopathologic characteristics of juvenile ossifying fibroma with orbital involvement. Am J Ophthalmol. 1983;96:650–3.
Shields JA, Peyster RG, Handler SD, Augsburger JJ, Kapustiak J. Massive juvenile ossifying fibroma of maxillary sinus with orbital involvement. Br J Ophthalmol. 1985;69:392–5.
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Tumors of Bone: Osteoma
Alper M, Gurler T, Bilkay U, Songur E, Mutluer S. Intraorbital osteoma and surgical strategy. J Craniofac Surg. 1998;9:464–7.
Grove AS Jr. Osteomas of the orbit. Ophthalmic Surg. 1978;9:23–39.
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Lachanas VA, Koutsopoulos AV, Hajiioannou JK, et al. Osteoid osteoma of the ethmoid bone associated with dacryocystitis. Head Face Med. 2006;2:23.
Livaoglu M, Cakir E, Karacal N. Large orbital osteoma arising from orbital roof: excision through an upper blepharoplasty incision. Orbit. 2009;28:200–2.
Maiuri F, Iaconetta G, Giamundo A, Stella L, Lamaida E. Fronto-ethmoid and orbital osteomas with intracranial extension. Report of two cases. J Neurosurg Sci. 1996;40:65–70.
McNab AA. Orbital osteoma in Gardner’s syndrome. Aust N Z J Ophthalmol. 1998;26:169–70.
Miller NR, Gray J, Snip R. Giant mushroom-shaped osteoma of the orbit originating from the maxillary sinus. Am J Ophthalmol. 1977;83:587–91.
Milman MC, Bayindir T, Akarcay M, et al. Endoscopic removal technique of a huge ethmoido-orbital osteoma. J Craniofac Surg. 2009;20:1403–6.
Mortada A. Orbital osteoma within the domain of ophthalmic surgery. Can J Ophthalmol. 1969;68:258–65.
Rawe SE, VanGilder JC. Surgical removal of orbital osteoma; case report. J Neurosurg. 1976;44:233–6.
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Selva D, Chen C, Wormald PJ. Frontoethmoidal osteoma: a stereotactic-assisted sino-orbital approach. Ophthal Plast Reconstr Surg. 2003;19:237–8.
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Whitson WE, Orcutt JC, Walkinshaw MD. Orbital osteoma in Gardner’s syndrome. Am J Ophthalmol. 1986;101:236–41.
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Tumors of Bone: Osteosarcoma
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Lacrimal Gland Tumors: Adenocarcinoma
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Lacrimal Gland Tumors: Adenoid Cystic Carcinoma
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Lacrimal Gland Tumors: Mucoepidermoid Carcinoma
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Lacrimal Gland Tumors: Pleomorphic Adenoma
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Metastatic Tumors: Breast Carcinoma
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Metastatic Tumors: Bronchogenic Carcinoma
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Metastatic Tumors: Malignant Melanoma
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Secondary Tumors: Basal Cell Carcinoma
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Dutton, J.J., Sines, D.T., Elner, V.M. (2021). Orbital Tumors. In: Servat, J.J., Black, E.H., Nesi, F.A., Gladstone, G.J., Calvano, C.J. (eds) Smith and Nesi’s Ophthalmic Plastic and Reconstructive Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-41720-8_56
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