RESUMEN
BACKGROUND: After solid organ transplantation, children are at risk for Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. Little is known about the clinical course, Epstein-Barr viral load variations, and optimal treatment for such patients. We set forth to understand the course of repeated episodes of post-transplant lymphoproliferative disorder and smooth muscle tumors. METHODS: We performed a retrospective chart review of patients up to 21 years old with solid organ transplantation and post-transplant lymphoproliferative disorder at the Children's Hospital of Philadelphia from January 2003 through June 30, 2020. RESULTS: Six patients had multiple episodes of Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. When the second episode was discovered, only one patient was symptomatic. Histology differed from diagnosis in four patients. Treatment included viral-specific T-lymphocytes (2), rituximab (3), reduction in immunosuppression alone (1). Five patients had complete response, and one had stable disease, but three patients developed a subsequent tumor. Two patients developed Epstein-Barr virus-associated smooth muscle tumors. Of these six patients, four are alive. The deaths were not related to their tumors. CONCLUSIONS: Despite a complete response to initial therapy, children are at risk for repeated episodes of Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. Histology and location were not typically consistent with initial diagnosis, suggesting these are second primaries rather than recurrences. Disease may be managed with individualized treatment plans but EBV-specific T cells need further study in such tumors.
RESUMEN
Background: Epstein Barr virus-associated smooth muscle tumors (EBV-SMT) are rare neoplasms that can occur in immunocompromised individuals. The native or transplanted liver is the most commonly involved site in post transplant patients. Systemic therapies have been utilized in EBV-SMT with modest activity. Case Description: We describe a 23-year-old female kidney transplant recipient who presented with acute myeloid leukemia (AML) and hepatic myeloid sarcoma (MS). Although it was not recognized initially, her liver biopsy revealing MS at diagnosis was posthumously found to have synchronous EBV-SMT. She underwent anthracycline based induction and achieved a complete remission of her AML by bone marrow biopsy. Due to a persistent hepatic mass, she was given salvage chemotherapy including fludarabine, etoposide, cytarabine, decitabine, and venetoclax for presumed refractory MS. Re-biopsy of the liver revealed the absence of MS and presence of EBV-SMT, which subsequently grew rapidly and precluded her from a liver tumor resection. The patient underwent sirolimus mammalian target of rapamycin (mTOR) therapy with palliative intent, but the patient's EBV-SMT progressed shortly after. At time of autopsy, the patient remained in complete remission from AML/MS, but was found to have multifocal progressive metastatic EBV-SMT. Conclusions: To our knowledge this is the first reported case of synchronous AML/MS and post transplant hepatic EBV-SMT that underwent treatment for AML/MS. Our report suggests that the chemotherapeutic agents utilized for AML/MS may have poor efficacy against EBV-SMT.