Related Experiment Video
Updated: Aug 14, 2025
An In Vitro Model for Studying Cellular Transformation by Kaposi Sarcoma Herpesvirus
Published on: August 25, 2017
Kaposi sarcoma in three pediatric liver transplantation recipients
Catarina Cordeiro1, Sandra Ferreira2, Susana Nobre2
1Department of Pediatrics, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Insights
Kaposi sarcoma (KS) is a rare tumor in children post-liver transplant (LT). Early diagnosis and treatment are vital for survival in pediatric LT patients with KS.
Area of Science:
- Oncology
- Pediatric Gastroenterology
- Transplantation Immunology
Background:
- Kaposi sarcoma (KS) is an endothelial cell tumor, exceptionally rare in pediatric populations.
- Solid organ transplantation, particularly liver transplantation (LT), significantly increases the risk of KS development.
- The incidence of KS post-LT is notably higher compared to the general pediatric population.
Observation:
- This report details three pediatric cases of Kaposi sarcoma (KS) following liver transplantation (LT).
- Patients presented with varied symptoms including lymphadenopathy, splenomegaly, cytopenias, and viral loads (EBV, HHV-8, CMV).
- Histopathological examination confirmed KS, with co-occurrences of Castleman's disease and plasmablastic lymphoma in one case.
Findings:
- All three pediatric liver transplant recipients developed Kaposi sarcoma (KS).
- Human herpesvirus 8 (HHV-8) infection was confirmed in all cases.
- Discontinuation of tacrolimus and initiation of sirolimus led to recovery and good graft function in all patients.
Implications:
- Kaposi sarcoma (KS) is an uncommon but serious complication after pediatric liver transplantation (LT).
- Prompt recognition of clinical signs and diagnostic confirmation of KS are critical for effective management.
- Immunosuppression modulation, including switching to sirolimus, appears beneficial for managing post-LT KS in children.
Background:
Kaposi sarcoma (KS) is an endothelial cell tumor, rare in children. It is 200 times more frequent after solid organ transplantation than in the general population.
Methods:
We report three cases of pediatric patients who developed KS after liver transplantation (LT).
Results:
Case 1, a 4-year-old boy undergoing LT due to familial intrahepatic cholestasis. Five months after LT, he presented with fever, dyspnea, and cough with enlarged lymph nodes and splenomegaly, anemia, thrombocytopenia, elevated liver enzymes, and positive EBV viral load. Lymph node biopsy diagnosed KS with an elevated HHV8 viral load. Case 2, a 4-year-old boy who underwent LT due to secondary biliary cirrhosis resulting from extrahepatic biliary atresia. Two years later, graft dysfunction was noticed with positive EBV viral load, thrombocytopenia, massive cervical lymph node enlargement, and splenomegaly. Lymph node biopsy diagnosed KS, Castleman's disease, and plasmablastic lymphoma related to HHV8 infection. Case 3, a 15-month-old girl, who received two LT due to biliary cirrhosis. Six months later, she presented with diarrhea, abdominal distension, anemia, thrombocytopenia, enlarged lymph nodes, splenomegaly, and positive CMV viral load. Axillary lymph node biopsy diagnosed KS and HHV8 infection was confirmed. In all three cases, tacrolimus was discontinued and, after diagnosis, sirolimus was started. All recovered without relapse and have a good graft function.
Conclusions:
Kaposi sarcoma is a rare disease post-LT in children. Recognizing keywords and early diagnosis is crucial for timely treatment and survival.
Related Concept Videos
01:26Kidney Transplant II: Surgical Procedure
01:28Kidney Transplant I: Introduction