Orbital Sarcoidosis With Invasion of the Lacrimal Gland

Significance

Orbital sarcoidosis can mimic tumors of the orbit and should be considered in the differential diagnosis for orbital masses. Imaging appearance on computed tomography (CT), magnetic resonance imaging (MRI), and surgical pathology guide the diagnosis of these inflammatory tumor-like masses.

Case Description

We present a case of a 65-year-old woman presenting with a painless left-sided eyelid droop accompanied by a mass on the left upper lateral eyelid. Physical exam revealed left exophthalmos and lacrimal gland prolapse with a mobile palpable mass on the left supratemporal orbit, as well as moderate orbital fat prolapse and decreased sensation of left V1 distribution.

MRI of the orbits with contrast revealed a 4.3 × 2.3 × 1.5-cm lesion within the left supratemporal, mid-anterior orbit (Figure 1). The mass appeared isointense to white matter on T1 and T2 and enhanced with contrast. An orbitotomy for tumor excision was planned, and intraoperatively, the lesion was found to be invading neighboring structures to the lacrimal gland, including the superior rectus, levator palpebrae, lateral rectus, and posterior orbit. The entire lacrimal gland and additional mass of the posterior segment were excised and sent for biopsy. Surgical pathology revealed an extensive granulomatous process affecting the supratemporal orbit and lacrimal gland, consistent with sarcoidosis.

Figure 1.

Axial (A) and coronal (B) MRI post-contrast sequences of the orbit in a 65-year-old woman show an infiltrating amorphous, enhancing lesion measuring 4.3 × 2.3 × 1.5 cm (red arrow) located in the superior lateral aspect of the left orbit with involvement of the lacrimal gland.

Discussion

Orbital sarcoidosis is a rare form of sarcoidosis, a systemic granulomatous disorder of unknown etiology. Though sarcoidosis is most associated with the lungs, it can also manifest in extra-thoracic organs and tissues such as the skin, heart, liver, spleen, central nervous system, and eyes. ¹ Since sarcoidosis is less common in the orbit, its involvement in this location presents unique challenges for diagnosis, treatment, and management.

Patients with orbital sarcoidosis often present with ocular symptoms such as eyelid swelling, pain, redness, or blurred vision.^1,2 These orbital manifestations may or may not be accompanied by ocular symptoms of sarcoidosis such as conjunctival nodules, anterior uveitis, and scleritis. ² Orbital involvement can lead to proptosis, restricted eye movements, and compressive optic neuropathy, which necessitates urgent intervention to prevent permanent visual impairment. Additionally, sarcoid granulomas can extend beyond the orbit, affecting adjacent structures such as the lacrimal glands, resulting in dry eyes and epiphora. ³ Recognizing these diverse clinical presentations is crucial for timely diagnosis and management.

The diagnosis of orbital sarcoidosis requires a multistep approach involving clinical, imaging, and pathologic assessments. Initial evaluation involves a comprehensive ophthalmic examination, including visual acuity and fundoscopy, which can then be followed by imaging modalities such as orbital CT or MRI. If imaging is positive for an orbital process, biopsy of orbital tissues can be accomplished through minimally invasive techniques like fine-needle aspiration or incisional biopsy, but often requires a more invasive procedure such as an orbitotomy. Biopsy results confirm the presence of noncaseating granulomas, fibrosis, and inflammatory signs that are characteristic of sarcoidosis. Ordering additional imaging and laboratory assessments, such as chest radiography, high-resolution CT, angiotensin-converting enzyme levels, and serum calcium levels, may provide further evidence for the diagnosis. ⁴

Management of orbital sarcoidosis focuses on alleviating symptoms, preserving visual function, and preventing complications. ⁴ While corticosteroids, both systemic and locally administered, are typically the first line of treatment in sarcoidosis, close monitoring of ocular manifestations and systemic involvement is essential to guide specific treatment decisions. Surgical intervention is warranted in selected cases, particularly decompressive procedures or orbital mass excision when patients display sight-threatening symptoms. A multidisciplinary approach, involving ophthalmologists, rheumatologists, and other specialists, ensures optimal care and long-term management.