title: Multimodal therapy including liver transplantation for hepatic undifferentiated embryonal sarcoma.
abstract: The outcomes of hepatic undifferentiated embryonal sarcoma (HUES) have historically been limited by persistent, unresectable disease and the subsequent development of disease resistance and dissemination. We present our institutional experience with HUES and assess current treatment trends and outcomes in the era of liver transplantation. We conducted a retrospective chart review of cases presenting with HUES at our institution over the past 10 years. The collected data included age, sex, presenting symptoms, imaging and the associated Pretreatment Extent of Disease (PRETEXT) score, pathology, chemotherapy, surgical interventions, and outcomes. Approval was obtained from the institutional review board of the Cincinnati Children's Hospital Medical Center. HUES was identified in 6 patients (4 males and 2 females) with a median age at diagnosis of 11 years (range = 7-13 years). Initial imaging was available for all but 1 patient. The PRETEXT stage for these patients ranged from II to III. One patient was diagnosed with lung metastases. Two patients underwent upfront resection, and 1 patient received neoadjuvant therapy and then conventional resection. Three patients were treated with orthotopic liver transplantation (OLT) after neoadjuvant chemotherapy (primary OLT in 2 cases and salvage OLT for local recurrence in 1 case). Two patients received posttransplant adjuvant chemotherapy. All 6 patients remained in clinical remission with a mean follow-up of 35 months (range = 12-84 months). In conclusion, OLT has rarely been reported as a treatment option for HUES. The addition of liver transplantation as a surgical option for treating patients with HUES can result in improved survival for patients whose tumors are initially unresectable or recur.
authors.affiliation: Departments of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
authors.forename: Ashley
authors.initials: A
authors.lastname: Walther
authors.forename: James
authors.initials: J
authors.lastname: Geller
authors.forename: Abigail
authors.initials: A
authors.lastname: Coots
authors.forename: Alexander
authors.initials: A
authors.lastname: Towbin
authors.forename: Jaimie
authors.initials: J
authors.lastname: Nathan
authors.forename: Maria
authors.initials: M
authors.lastname: Alonso
authors.forename: Rachel
authors.initials: R
authors.lastname: Sheridan
authors.forename: Greg
authors.initials: G
authors.lastname: Tiao
mesh_terms: D000293:Adolescent; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000628:therapy*; D016031:Liver Transplantation; D008297:Male; D020360:Neoadjuvant Therapy; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D009373:Neoplasms, Germ Cell and Embryonal / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D016879:Salvage Therapy; D012509:Sarcoma / Q000628:therapy*; D016896:Treatment Outcome