Kaposi’s sarcoma: a single-center experience on 38 patients

aposi’s Sarcoma (KS) is a multifocal angioproliferative neolasm associated with infection by Human Herpes Virus type (HHV8).1,2 We retrospectively studied 38 biopsy-proven KS diagosed at our department between 2010 and 2019. The patients’ mean age was 60.5 years (range 35--84). he population was mostly male (n = 33, 86.8%) and Cauasian (n = 31, 81.6%). The epidemic (HIV-associated) subtype predominated (16 ases; 42.1%), followed by the classic (n = 12; 31.6%) and vinorelbine in 1 (4%). Fourteen patients (36.8%) needed second and/or third-line therapies, with local (laser CO2, cryotherapy, radiotherapy, intralesional vinblastine) and/or systemic (doxorubicin, bleomycin, vinorelbine, vinblastine, alfa interferon, paclitaxel) approaches in variable combinations. Partial and/or complete response was achieved in the majority (65.8%) of cases. A third developed -mainly mild (venous stasis and/or lymphedema) -complications. However, transformation to an anaplastic variant arose in one case (Fig. 2) and another patient was subsequently diagnosed with non-Hodgkin lymphoma. Although overall mortality was 36.8%, mortality directly related to KS was only 8% (n = 3). Two patients had classic KS: one died aged 83 following transformation to anaplastic KS despite four cycles of bleomycin; the other died due to visceral p i s T t i b

Partial and/or complete response was achieved in the majority (65.8%) of cases. A third developed ---mainly mild (venous stasis and/or lymphedema) ---complications. However, transformation to an anaplastic variant arose in one case (Fig. 2) and another patient was subsequently diagnosed with non-Hodgkin lymphoma. Although overall mortality was 36.8%, mortality directly related to KS was only 8% (n = 3). Two patients had classic KS: one died aged 83 following transformation to anaplastic KS despite four cycles of bleomycin; the other died due to visceral progression of the disease (gastrointestinal and pulmonary involvement already present at the moment of diagnosis, later progressing with liver metastases --- Fig. 3). The third patient was heart transplanted, dying with gastrointestinal and pulmonary metastases of KS, even after immunosuppression adjustment and 6 cycles of vinorelbine.
We also compared the group of immunocompetent/classic KS patients with immunocompromised ones. Results are summarised in Table 1.
In our study, KS was almost seven times more frequent in men (6.6:1), which is a greater ratio than reported in the literature (2---5:1, at least for the classic variant); ethnicity and age distribution were similar to other European reports. 1,3,4 A potentially intriguing result is the relatively high percentage of epidemic cases compared to classic KS, which would be expected to predominate in a Caucasian European population like ours. This is likely explained by the fact that the great majority of our KS patients come from the Infectious Disease department. Moreover, our department has a differentiated consultation for immunosuppressed patients, which further explains this specific scenario.
Cutaneous lesions did not differ from what is described in the literature. On the contrary, the percentage of extracutaneous involvement was quite elevated (37%), especially when we compare to other recent studies (15% in a single-Turkish center study published in 2018; and 16.8% in a retrospective study from a tertiary hospital in Barcelona from 1987---2016, which included many HIV patients with advanced disease in the pre-ART era). 3,4 This may be due to the greater number of immunosuppressed patients in our sample (n = 26, vs. n = 10 classic KS), particularly HIV, which is generally associated with greater extra-cutaneous involvement (as described in the literature and also seen in this study). 1,5 In accordance with literature data, the prognosis of KS was good: the overall response was observed in 25 cases, stabilization of the disease in two, and progression in four. Disease-specific mortality was 8%, closer to other published studies (e.g. 6.5% in a Turkish study and 5.2% in a Spanish one). 3,4   Comparing the groups of immunocompetent and immunocompromised patients, the second was significantly younger than the first, which is in accordance with the literature. 6 As expected, there was a greater prevalence of extracutaneous involvement in immunocompromised patients, although not a statistically significant one (which may be due to the small sample size). There was also no statistical difference in specific mortality by KS.
As far as we know, this is the largest study on KS in the Portuguese population and the first concerning the dermatologic perspective. The study's main limitations are its retrospective nature and the relatively small sample size.