Classification of Orbital Tumors
Orbital tumors represent approximately 0.1% of all body tumors and approximately one-fifth of all orbital diseases. Classification schemes vary and stratify orbital tumors based on demographics, site of origin, anatomic location within the orbit, histopat
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Classification of Orbital Tumors Alexander D. Blandford and Julian D. Perry
Introduction Orbital tumors represent approximately 0.1% of all body tumors and approximately one-fifth of all orbital diseases. Classification schemes vary and stratify orbital tumors based on demographics, site of origin, anatomic location within the orbit, histopathologic features, clinical course, and imaging findings. Defining orbital neoplasia presents difficulties, as choristomas, hamartomas, and inflammatory lesions can present as space-occupying lesions and behave as benign and even malignant, neoplasms. In general, neoplasms of the orbit may be classified as primary, secondary (infiltration from an adjacent structure), or metastatic (from distant structures). Orbital neoplasia can be divided into histological categories that include benign, benign but locally aggressive, and malignant. In some cases, especially lymphoproliferative lesions, a spectrum from benign to malignant exists. This chapter aims to classify orbital tumors on clinical grounds in order to provide a frame-
A. D. Blandford (*) · J. D. Perry Department of Ophthalmology and Oculofacial Plastic Surgery, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA e-mail: [email protected]
© Springer Nature Switzerland AG 2019 C. J. Hwang et al. (eds.), Clinical Ophthalmic Oncology, https://doi.org/10.1007/978-3-030-13558-4_2
work to conceptualize space-occupying orbital lesions to determine an evaluation and treatment algorithm.
ifferential Diagnosis of Orbital D Tumors Masquerading processes, such as infectious and inflammatory diseases, can resemble an orbital tumor and must be excluded during the workup of a space-occupying orbital lesion. Many nonneoplastic processes can be excluded based on a combination of demographic, clinical, and imaging characteristics (Box 2.1).
Box 2.1 Lesions that May Simulate an Orbital Neoplasm
• Infectious –– Acute bacterial orbital cellulitis –– Invasive fungal infection –– Mycobacterial infection • Inflammatory –– Idiopathic orbital inflammation –– Dysthyroid orbitopathy –– Systemic vasculitides • Other –– Amyloidosis
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A. D. Blandford and J. D. Perry
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Clinicopathological Classification of Orbital Tumors Cystic Lesions Dermoid cysts are the most common cystic lesions of the orbit [1]. They represent congenital lesions that form from epithelial cells trapped beneath the surface epithelium during embryogenesis. They often occur along the orbital rim superotemporally at the zygomaticofrontal suture, but they can occur at other bony sutures or in deeper orbital tissues. Other orbital cystic lesions include colobomatous cyst, congenital cystic eye, meningocele, and teratoma. Several other orbital neoplasms may present with cystic components (Table 2.1).
Table 2.2 Orbital vascular lesions More common Capillary hemangioma Cavernous hemangioma Hemangiopericytoma Lymphangioma (type 1) Varix (type 2) AVM (type 3)
Less common Angiosarcoma Cholesterol granuloma Hemangioendothelioma Hemangiosarcoma Kaposi’s sarcoma Kimura’s dise
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