Introduction: Liver transplantation traditionally emphasizes donor procurement, transplant procedures, and post-transplant immunosuppression. However, post-pediatric liver transplant malignancies receive insufficient attention despite posing significant risks to patient health. Redirecting focus towards understanding malignancy types and risk factors is crucial for improving long-term outcomes.
Objectives: Our literature review aims to determine the prevalence and types of post-pediatric liver transplant malignancies, identify potential risk factors, and explore management strategies post-diagnosis.
Methods: A comprehensive literature review was conducted using articles and published data from PubMed.
Results: Post-transplant lymphoproliferative disorder (PTLD) is the most prevalent malignancy following pediatric liver transplantation, with reported prevalence rates ranging widely from 1.4% to 80%. PTLD is typically diagnosed within 11 months to 8 years post-transplant, with pediatric patients facing a significantly higher risk compared to adults, especially in younger age groups. Incidence of PTLD is three times higher in combined liver and small bowel transplants. Non-Hodgkin lymphoma is the predominant PTLD type, accounting for approximately 71% of cases, followed by non-destructive PTLD, polymorphic, Hodgkin lymphoma, and leukemia. 90%-95% of PTLD cases are associated with Epstein-Barr virus (EBV) infection. Post-transplant immunosuppression, particularly Tacrolimus and Cyclosporin, has been implicated in increasing PTLD risk. Skin cancer, mainly squamous cell carcinoma, is the second most prevalent malignancy, with pediatric cases exhibiting higher rates of certain types compared to adults. Other observed malignancies include hepatocellular carcinoma, EBV-associated post-transplant smooth muscle tumors (EBV-PTSMT), plasmacytoma-like PTLD, and various rare non-PTLD malignancies such as gynecologic neoplasms, Kaposi's sarcoma (KS), papillary thyroid tumors, sarcomas, brain tumors, renal cell carcinoma, liver tumors, testis neoplasms, and bladder cancer. Management typically involves reducing or discontinuing immunosuppression, surgical resection for localized disease, and medication such as sirolimus or rituximab for systemic or aggressive cases. Overall survival rates following PTLD diagnosis vary widely, with studies reporting rates ranging from 9% to 64%.
Conclusion: PTLD as the primary concern following pediatric liver transplantation. PTLD tends to occur more frequently in younger age groups and is strongly associated with Epstein-Barr virus (EBV) infection. Moreover, certain immunosuppressants, particularly Tacrolimus and Cyclosporin, have been implicated in increasing PTLD risk . Recognizing these risk factors is crucial as it enables physicians handling those patients to be proactive in preventing, early detecting, and effectively managing malignancies. Increasing publications will provide a better guide to management over time.