Visceral Kaposi's sarcoma remission after intestinal transplant. First case report and systematic literature review

Kaposi's sarcoma (KS) is an infrequent vascular neoplasm commonly diagnosed as an isolated cutaneous lesion that can involve other organs. So far, there are no data in the literature about the development of KS after intestinal transplant.

In this study, the authors describe a case of "visceral KS" with pulmonary and intestinal involvement and perform a systematic literature review of case reports and single-center series identified in MEDLINE.

This case was a 42-year-old man, diagnosed with visceral KS 9 months after receiving an isolated intestinal transplant. He was successfully treated with a combination of sirolimus and liposomal doxorubicin and achieved an 18-month disease-free survival. A total of 54 cases from 27 manuscripts and the present case were analyzed in this study. The mean time from transplant to diagnosis was 17.2 months. Lungs and gastrointestinal tract were the main organs involved. Immunosuppressants were discontinued in two of the three (66.7%) cases, and sirolimus was added in eight cases. Doxorubicin was used in 12 cases. In a univariate analysis, the use of Tacrolimus, type of transplant, and presence of cutaneous KS seem to be the significant predictors of response to therapy and survival; the addition of doxorubicin showed a reduction in graft loss.

Treatment of KS in posttransplant patients should be designed aiming to obtain a complete response, irrespective of the organ affected. Only recipients who are able to achieve a sustained response would be able to obtain long-term disease-free survival.