Myths in the Diagnosis and Management of Orbital Tumors

Abstract

Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments.

Keywords: Adenoid Cystic Carcinoma; BRAF; Basal Cell Carcinoma; Cavernous Hemangioma; Cetuximab; Epidermal Growth Factor Receptor; Fluid-Fluid Levels; Hedgehog Pathway; Idiopathic Orbital Inflammation; MYB; Optic Glioma; Orbitotomy; Receptor Tyrosine Kinase Inhibitor; Schwannoma; Sorafenib; Squamous Cell Carcinoma; Targeted Treatment; Vismodegib.

Figure 1

A 42-year-old female presenting with painful proptosis. (a) T1-weighted axial magnetic resonance imaging shows ill-defined orbital lesion occupying the apex. The lesion is isointense to the cerebral gray matter and extraocular muscle. (b) The apical lesion shows marked contrast enhancement after gadolinium injection. (c) T2-weighted axial magnetic resonance imaging demonstrates that lesion is hypointense to the cerebral gray matter and extraocular muscle

Figure 2

(a) T1-weighted axial magnetic resonance imaging showing a well-circumscribed orbital lesion that compresses the optic nerve on the left side. (b) T1-weighted coronal magnetic resonance imaging is showing a well-circumscribed orbital lesion that does not encroach on the optic nerve on the right side. (c) Gross photograph of the tumor depicted in Figure 2a which proved to be a cavernous hemangioma on histopathologic examination after excision. (d) Gross photograph of the tumor depicted in Figure 2b which was also read as cavernous hemangioma

Figure 3

A 31-year-old male with a 5 years history of proptosis in the left eye. (a) Facial photograph shows left proptosis. (b) T1-weighted magnetic resonance imaging demonstrates a cavitary mass in the left orbit. (c) T2-weighted magnetic resonance imaging shows fluid-fluid levels in the tumor. (d) Gross photo of the excised tumor shows yellowish hemorrhagic tumor fragments. (e) Histopathologic examination demonstrates schwannoma consisting mostly of spindle shaped tumor cells with elongated nuclei forming bundles and cellular areas without obvious mitosis (Antoni A pattern) (H and E, ×400). (f) Immunohistochemically, the tumor cells diffusely stain positive with S-100 (S-100, ×100)

Figure 4

A 65-year-old female with locally advanced squamous cell carcinoma of the eyelid with orbit and bone invasion. (a) Facial photograph shows ill-defined eyelid squamous cell carcinoma affecting the lateral canthal region. (b) Anterior segment photograph of the left eye demonstrates vascularized corneal opacification and conjunctival keratinization as secondary effects of locally advanced eyelid cancer. (c) Orbital computed tomography shows orbital and bone invasion from eyelid tumor. (d) Facial photograph 6 months after orbital exenteration, bone removal, external radiotherapy, and intravenous chemotherapy (cisplatin and doxorubicin)