ABSTRACT Primary hepatic perivascular epithelioid cell tumor (PEComa) is rare, and management of unresectable disease is not established. A 47-year-old woman had an 8-cm central hepatic PEComa bridging both lobes with tuberous sclerosis complex 2 alterations. She received albumin-bound sirolimus for 26 months with modest size reduction but durable stability, enabling listing with a nonstandard model for end-stage liver disease exception. She underwent right trisegment orthotopic liver transplantation, and the explant showed a well-circumscribed PEComa without vascular invasion or additional lesions. This case highlights prolonged nab-sirolimus as a bridge to transplantation in selected patients with favorable tumor biology.
Cruz et al. (Wed,) studied this question.