Sarcomatoid Carcinoma of Orbit in a Patient With Xeroderma Pigmentosum

Spindle cell carcinoma (SpCC), also referred to as sarcomatoid carcinoma, makes up less than 3% of all head and neck squamous cell carcinomas (SCCs). It is an uncommon and unusual biphasic malignant tumor primarily affecting the upper aero-digestive tract. SpCC consists of spindled or pleomorphic tumor cells. Mostly, these tumors are seen in the fifth or sixth decades of life and are strongly associated with smoking and alcohol. We hereby report an infrequently encountered case of SpCC in a patient with xeroderma pigmentosum (XP), who was young, nonsmoker, and did not consume alcohol. The mass arose from the right orbit and involved the entire right face. The postoperative histopathological report showed SpCC. Surgical excision of the mass was performed. We aimed to add to the prevailing literature by reporting this case.