Case Report: Primary Squamous Cell Carcinoma of the Orbit in a Patient With Carney's Syndrome Treated With Multidisciplinary Approaches

Squamous cell carcinoma (SCC) is a rare malignancy of invasive epithelium with keratinocyte differentiation, and it is the most common form of eyelid malignant neoplasm, comprising 5%–10% of malignancies. While SCC rarely affects the orbit, it may be involved through local invasion from a cutaneous primary site or extension by perineural invasion. Only 12 cases of primary orbital SCC have been reported until now. Here, we present a case of primary carcinoma of the right orbit with coexisting Carney's syndrome, a rare genetic disorder associated with multiple endocrine neoplasias (MEN) syndromes.

A 62-year-old South Asian male presented with a painful swelling in the lateral aspect of the right eyebrow and protrusion of the eyeball in August 2020. He had a history of excision of Right atrial Myxoma in March 2020. Orbital computerized tomography (CT) and positron emission tomography (PET-CT) scans revealed an enhancing soft tissue lesion in the right orbit with the involvement of frontal and ethmoid sinuses. Biopsy confirmed HPV-related poorly differentiated SCC, positive for HPV-related markers. The patient received concurrent chemo irradiation with Cisplatin. The patient's proptosis resolved, and subsequent PET-CT and magnetic resonance imaging (MRI) scans did not show any residual or recurrent disease.

The findings emphasize the importance of early detection and management of this uncommon and life-threatening condition, providing hope for patients and aiding in the prevention of recurrence.