Abstract
Malignant orbital tumors represent a broad spectrum of tumors which include primary tumors, secondary tumors (extension from adjacent structures), and metastatic tumors. In addition, orbital inflammation and infection may clinically simulate an orbital neoplasm. In a recent survey of 1264 consecutive patients with suspected orbital tumor referred to an ophthalmic oncology center, 36% were malignant tumors. The percentage of malignant tumors increases with age, due to higher incidence of lymphoma and metastasis in the older age groups. Rhabdomyosarcoma is the most frequent primary malignant orbital tumor in children, and lymphoproliferative disorders including lymphoma are most frequent in older adults. Malignant orbital tumors may also arise from the lacrimal gland and lacrimal sac. Malignant tumors of vascular, neural, fibrous, and osseous origin are rare in the orbit.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology. 2004;111(5):997–1008.
Laforest C, Selva D, Crompton J, et al. Orbital invasion by esthesioneuroblastoma. Ophthal Plast Reconstr Surg. 2005;21(6):435–40.
Loevner LA, Sonners AI. Imaging of neoplasms of the paranasal sinuses. Magn Reson Imaging Clin N Am. 2002;10(3):467–93.
Lyons CJ, McNab AA, Garner A, et al. Orbital malignant peripheral nerve sheath tumors. Br J Ophthalmol. 1989;73:731–8.
Bhargava R, Parham DM, Lasater OE, et al. MR differentiation of benign and malignant peripheral nerve sheath tumors: use of the target sign. Pediatr Radiol. 1997;27:124–9.
Erzurum SA, Melen O, Lissner G, et al. Orbital malignant peripheral nerve sheath tumors. Treatment with surgical resection and radiation therapy. J Clin Neuroophthalmol. 1993;13:1–7.
Ordonez NG, Mackay B. Alveolar soft-part sarcoma: a review of the pathology and histogenesis. Ultrastruct Pathol. 1998;22:275–92.
Jordan DR, MacDonald H, Noel L, et al. Alveolar soft-part sarcoma of the orbit. Ophthalmic Surg. 1995;26:269–70.
Sherman N, Valvilala M, Pollock R, et al. Radiation therapy for alveolar soft-part sarcoma. Med Pediatr Oncol. 1994;22:380–3.
Mark RJ, Sercarz JA, Tran L, et al. Osteogenic sarcoma of the head and neck. The UCLA experience. Arch Otolaryngol. 1991;117:761–6.
Abramson DH, Ronner HJ, Ellsworth RM. Second tumors in non-irradiated bilateral retinoblastoma. Am J Ophthalmol. 1979;87:624–7.
Jacobiec FA, Howard GM, Jones IS, et al. Fibrous histiocytomas of the orbit. Am J Ophthalmol. 1974;77:333–45.
Folberg R, Cleaseby G, Flanagan JA, et al. 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.
Kaltreider SA, Destro M, Lemke BN. Leiomyosarcoma of the orbit. A case report and review of the literature. Ophthal Plast Reconstr Surg. 1987;3:35–41.
Jakobiec FA, Howard G, Rosen M, et al. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol. 1975;80:1028–42.
Cai YC, McMenamin ME, Rose G, et al. Primary liposarcoma of the orbit: a clinicopathologic study of seven cases. Ann Diagn Pathol. 2001;5:255–66.
Tehrani AH, Heegaard S, Prause JU, et al. Liposarcoma metastatic to the orbit. Eur J Ophthalmol. 2003;13:108–12.
Jakobiec FA, Rini F, Char D, et al. Primary liposarcoma of the orbit. Problems in the diagnosis and management of five cases. Ophthalmology. 1989;96:180–91.
Costas A, Castro P, Munoz JM. Primary orbital liposarcoma: case report and review of the literature. Int J Oral Maxillofac Surg. 2001;30:170–2.
Cockerham KP, Kennerdell JS, Celin SE, et al. Liposarcoma of the orbit: a management challenge. Ophthal Plast Reconstr Surg. 1998;14:370–4.
Howard GR, Nerad JA, Carter KD, et al. Clinical characteristics associated with orbital invasion of cutaneous basal cell and squamous cell tumors of the eyelid. Am J Ophthalmol. 1992;113:123–33.
Reifler DM, Hornblass A. Squamous cell carcinoma of the eyelid. Surv Ophthalmol. 1986;30:349–65.
Goldberg RA, Rootman J, Cline RA. Tumors metastatic to the orbit: a changing picture. Surv Ophthalmol. 1990;35:1–24.
Yin VT, Pfeiffer ML, Esmaeli B. Targeted therapy for orbital and periocular basal cell carcinoma and squamous cell carcinoma. Ophthal Plast Reconstr Surg. 2013;29(2):87–92.
Goldberg RA, Rootman J. Clinical characteristics of metastatic orbital tumors. Ophthalmology. 1990;97:620–4.
Levy WJ. Neuroblastoma. Br J Ophthalmol. 1957;41(1):48–53.
Ahmed AA, Zhang L, Reddivalla N, et al. Neuroblastoma in children: update on clinicopathologic and genetic prognostic factors. Pediatr Hemat. 2017;34(3):165–85.
Dutton JJ, Rose JG Jr, DeBacker CM, et al. Orbital Ewing’s sarcoma of the orbit. Ophthal Plast Reconstr Surg. 2000;16(4):292–300.
Bosma SE, Ayu O, Fiocco M, et al. Prognostic factors for survival in Ewing sarcoma: a systematic review. Surg Oncol. 2018;27(4):603–10.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Patel, B.C.K. (2019). Malignant Orbital Tumors. In: Hwang, C.J., Patel, B.C., Singh, A.D. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-13558-4_16
Download citation
DOI: https://doi.org/10.1007/978-3-030-13558-4_16
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-13557-7
Online ISBN: 978-3-030-13558-4
eBook Packages: MedicineMedicine (R0)