Mucosal Kaposi sarcoma, a Rare Cancer Network study

Abstract

Kaposi's sarcoma (KS) most often affect the skin but occasionally affect the mucosa of different anatomic sites. The management of mucosal KS is seldom described in the literature. Data from 15 eligible patients with mucosal KS treated between 1994 and 2008 in five institutions within three countries of the Rare Cancer Network group were collected. The inclusion criteria were as follows: age >16 years, confirmed pathological diagnosis, mucosal stages I and II, and a minimum of 6 months' follow-up after treatment. Head and neck sites were the most common (66%). Eleven cases were HIV-positive. CD4 counts correlated with disease stage. Twelve patients had biopsy only while three patients underwent local resection. Radiotherapy (RT) was delivered whatever their CD4 status was. Median total radiation dose was 16.2 Gy (0-45) delivered in median 17 days (0-40) with four patients receiving no RT. Six patients underwent chemotherapy and received from 1 to 11 cycles of various regimens namely vinblastin, caelyx, bleomycine, or interferon, whatever their CD4 counts was. Five-year disease free survival were 81.6% and 75.0% in patients undergoing RT or not, respectively. Median survival was 66.9 months. Radiation-induced toxicity was at worse grade 1-2 and was manageable whatever patients' HIV status. This small series of mucosal KSs revealed that relatively low-dose RT is overall safe and efficient in HIV-positive and negative patients. Since there are distant relapses either in multicentric cutaneous or visceral forms in head and neck cases, the role of systemic treatments may be worth investigations in addition to RT of localized disease. Surgery may be used for symptomatic lesions, with caution given the risk of bleeding.

Keywords: HIV; Kaposi sarcoma; classic.; head and neck; mucosal; radiation therapy; systemic treatment.

Figure 1

Isolated Kaposi sarcoma lesion of the left tonsil in a 69 year-old Caucasian HIV-negative human herpesvirus type 8 positive male.

Figure 2

Disease free survival.

Figure 3

Treatment algorithm for localized cutaneous and mucosal Kaposi sarcoma lesions. If lesion growth is slow or stable, observation is a reasonable option. For HIV-infected patients, initiation of HAART alone may induce regression of the lesions. Systemic chemotherapy should be discussed depending on the number of lesions (>20), involvement of internal organs, associated symptoms, speed of evolution, age, performance status, ulceration and/or presence of edema. Close monitoring during radiation treatments is important for immunosuppressed patients. Absence of immunodeficiency can include HHV-8 patients. *Strict oral hygiene protocol and antifungal treatment for 6 weeks (starting 1 week before the radiotherapy). LF-EBRT, localized field external beam radiotherapy; CT, chemotherapy.