Background: Mucosal involvement in HIV-negative Kaposi's sarcoma (KS) is uncommon but has potentially serious repercussions on patient care. Evidence regarding its epidemiology and optimal management is limited. Invasive endoscopic staging at diagnosis and periodically during follow-up is currently recommended by major guidelines. Materials and methods: We reviewed the clinical records of 1,308 HIV-negative KS patients followed at our dedicated KS outpatient service. Demographics, clinical characteristics, and treatment outcomes for cases with biopsy proven mucosal lesions were collected. Results: Mucosal involvement was documented in 53 patients (4.1% of our cohort), being present at diagnosis in 28 (52.8%) and occurring at a later time in the remaining 25 (47.2%) patients, with a mean latency of 8 years (±7.7). Oral cavity (43.4%) and glans penis (39.6%) were the most frequently involved anatomical sites. Of those with available treatment response data, complete response (CR) of mucosal KS was appreciated in 41 cases (93.2%), while partial response (PR) and stable disease (SD) were documented in one (2.3%) and two cases (4.5%), respectively. Same-site recurrences were noticed in seven patients (17.1%). Conclusion: Mucosal involvement in HIV-negative KS is rare, and its recurrence, if properly treated, appears to be infrequent. Thus, routine invasive monitoring in this setting may be unnecessary. We propose a tailored approach based on the clinical manifestations of each patient, limiting the indication of invasive procedures to the first evaluation and in case of significant clinical worsening or to monitor known mucosal localizations.
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