Case 3
A previously healthy 7-year-old female underwent OHT after developing viral induced cardiomyopathy leading to heart failure. Pre-transplant serologies were: EBV donor positive/recipient unknown. She was started tacrolimus, Azathioprine and steroids to prevent transplant rejection. The rest of the immediate post-transplant history is limited given incomplete chart.
Approximately seven months post-transplant she developed enlarged lymph nodes of the neck, axilla and groin and was diagnosed with polymorphous PTLD via biopsy. Her immunosuppression was decreased briefly, but due to graft rejection she required re-escalation of her immunosuppressive medications. One year following initial PTLD diagnosis, she again developed diffuse palpable adenopathy of the groin and axilla that was found to be recurrence of PTLD confirmed on biopsy. She concomitantly had chronic EBV DNAemia with peak documented levels of 7,365 copies/mL (presumed to be higher but records are incomplete). She underwent total lymphoid irradiation to the periaortic, splenic and pelvic lymph nodes delivering a total of 4.8 Gy in six fractions before therapy was truncated and not resumed due to ongoing medical issues surrounding chronic EBV infection. Despite radiation therapy, she developed two new liver lesions with the largest lesion measuring appx 2 cm on CT. No evidence of additional disease outside the liver was seen. Liver biopsy was consistent with EBV SMT (Fig 3). At the same time, she underwent scheduled cardiac catheterization which revealed severe coronary artery disease of her graft. She was admitted to the hospital in heart failure and re-listed for transplant. During this hospitalization, she went into cardiac arrest and required brief placement on ECMO before being re-transplanted 2 days later. After a prolonged four-month hospitalization, she was discharged home on cyclosporine, MMF, and prednisone.
Once recovered, she underwent planned resection of the known solitary hepatic SMT which was successful. Surveillance CT following resection showed recurrence of the hepatic SMT, in addition to the development of a second hepatic tumor that was deemed unresectable at the time. With increasing growth of the tumors now measuring 8.5 cm and 4.5 cm along with chronic GI complaints of diarrhea and abdominal pain, chemoembolization was briefly considered but dismissed after CT angiography demonstrated the tumors were poorly vascularized. She underwent palliative radiation to the liver and received a total tumor dose of 3500 cGy. Given the positive response to radiotherapy, she was evaluated for second resection at an outlying facility and underwent full resection of now three liver tumors with subsequent remission.
Due to progressive coronary artery disease and continued heart failure symptoms, she was re-listed for a third cardiac transplant in addition to a kidney transplant due to ESRD. Shortly thereafter, she was admitted to the hospital with new onset hematemesis secondary to new fungating mass in the gastric antrum. Upper GI series showed diffuse thickening of the esophagus thought to be secondary to recurrence of SMT although not confirmed with biopsy given her tenuous clinical state. In the setting of heart failure and ESRD, the mass was deemed inoperable. She was no longer eligible for transplant and was placed on comfort care and passed away in the hospital due to progressive disease.