Kaposi's sarcoma (KS) is a vascular sarcoma derived from vascular endothelial cells and presents with multiple lesions. It mainly appears on the skin and oral mucosa, usually in the face, oral mucosa, and genitals. Very few cases of primary lesions in the nasal cavity have been reported. It is often difficult to diagnose only by imaging examination. Here, we describe a case of KS in a patient who was human immunodeficiency virus (HIV)-negative, in which the primary sites were the nasal mucosa and nasal septum. A diagnosis was made according to the patient's clinical presentation, physical examination, laboratory examination, imaging examination, and histopathological results. We used surgical resection combined with chemotherapy, with 6 months' postoperative follow-up without recurrence. We reviewed the relevant literature to identify similar cases and summarize the findings reported on this rare manifestation of KS. We recommend that, where possible, antiviral therapy such as interferon, and regular review should continue, to improve the survival rate and patients' quality of life.
Keywords: HIV seronegativity; Kaposi sarcoma; chemotherapy; nasal cavity; surgery.