Primary tonsillar kaposi sarcoma in an HIV-negative, immunocompetent patient: A case-based review

Kaposi sarcoma (KS) is a rare vascular neoplasm associated with human herpesvirus-8 (HHV-8) infection and is most commonly encountered in immunocompromised individuals. Primary tonsillar involvement in HIV-negative, immunocompetent patients is exceptionally rare, with only a limited number of cases reported in the literature.

To present a rare case of primary tonsillar Kaposi sarcoma in an HIV-negative, immunocompetent patient with long-term follow-up, and to review the relevant literature focusing on clinical presentation, diagnostic approach, and management strategies.

We report the clinical course of a 36-year-old HIV-negative male who presented with a unilateral tonsillar mass and underwent tonsillectomy. The diagnosis of Kaposi sarcoma was established through histopathological evaluation and confirmed by HHV-8 immunohistochemical staining. Long-term clinical follow-up was performed, and a targeted review of previously published cases of tonsillar KS was conducted.

Following surgical excision, the patient remained disease-free for 16 years. Subsequently, cutaneous recurrences of Kaposi sarcoma developed and were successfully treated with oral etoposide chemotherapy and localized radiotherapy, resulting in effective disease control without further progression.

Primary tonsillar Kaposi sarcoma in HIV-negative, immunocompetent patients is an extremely rare clinical entity but should be considered in the differential diagnosis of tonsillar masses. HHV-8 immunostaining is essential for accurate diagnosis. Surgical excision, combined with systemic and local therapies for recurrence, can provide durable disease control. Long-term follow-up is crucial due to the potential for late multifocal recurrence.