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A Single-Institution Retrospective Cases Series of Childhood

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A Single-Institution Retrospective Cases Series of Childhood ORIGINAL ARTICLE A Single-institution Retrospective Cases Series of Childhood Undifferentiated Embryonal Liver Sarcoma (UELS): Success of Combined Therapy and the Use of Orthotopic Liver Transplant Ashley S. Plant, MD,* Ronald W. Busuttil, MD,w Abbas Rana, MD,w Scott D. Nelson, MD,z Martin Auerbach, MD,y and Noah C. Federman, MD*8z in 2 representative cases. Although follow-up is short for some Background/Introduction: Undifferentiated embryonal liver sar- patients, overall survival in these 5 patients was 100% with follow-up coma (UELS) makes up 9% to 15% of all malignant liver tumors ranging from 21 to 68 months. Disease-free survival ranged from 8 to in children. UELS is characteristically diagnosed between the ages 46 months with no patients with residual disease. of 6 and 10 years and presents with abdominal pain, vomiting, and an abdominal mass. There is currently no standardized treatment Conclusions: UELS is an aggressive high-grade primary liver sar- for UELS except attempt at complete surgical resection. There coma with high metastatic potential. This report represents one of have been only about 150 cases of UELS reported in the literature the largest single-institution studies of UELS. Using multimodality all with historically poor overall survival of <37.5% at 5 years. therapy, patients have achieved 100% overall survival even in the This report is one of the largest single-institution reports of UELS setting of extensive disease, metastases, and recurrence. In cases of consisting of 5 patients over 2 decades. The purpose of this study is unresectable primary tumor or recurrent and refractory disease to characterize presentation and to report treatment success in isolated to the liver, OLT is a potential therapeutic option. We UELS in children, adolescents, and young adults and the use of report success with adjuvant chemotherapy and complete surgical liver transplantation and, lastly, to suggest a use of positron resection with OLT as an alternative in unresectable or refractory emission tomography/computed tomography (PET/CT) in mon- cases. We also suggest a possible utility of PET/CT in monitoring itoring of this disease process. treatment response in this disease. Methods: We conducted an Institutional Review Board–approved Key Words: sarcoma, soft tissue, undifferentiated, liver, childhood, retrospective chart review. Data were collected from UELS patients transplant younger than 21 years seen at the University of California Los (J Pediatr Hematol Oncol 2013;35:451–455) Angeles over the past 20 years (January 2001 to September 2011). Descriptive analysis was conducted including multiple parameters of patient demographics, tumor characteristics, treatment modal- ities, and morbidity and mortality. ndifferentiated embryonal liver sarcoma (UELS) is a Results: Five patients with UELS were identified. Patients initially Urare malignant liver tumor of childhood making up 9% 1 presented with fever, abdominal pain, or nausea. Ages ranged from to 15% of all hepatic malignancies. Diagnosis is usually 10 to 19 years old (median age 13 y old), and there was a 4:1 male-to- made between 6 and 10 years of age and typical presenting female predominance. Tumor size ranged from 6 to 22 cm in largest signs and symptoms include nausea, anorexia, right upper diameter. One patient presented with metastatic disease to the lungs quadrant mass, abdominal pain, and fever. Imaging typi- and heart and 1 patient recurred 2 years from diagnosis with bilateral cally reveals a large hepatic mass in the right lobe more paraspinal masses. Treatment included local control surgery with often than the left lobe, made up of both cystic and solid neoadjuvant and adjuvant chemotherapy with an anthracycline/ components, and occasionally involving the portal system alkylating agent combination. One patient with recurrent and 2 refractory disease achieved local control with an orthotopic liver making them notoriously difficult to resect. Newer studies transplantation (OLT). Metastatic disease was controlled with sur- suggest an older age distribution with average age of pre- 1 gery and radiation therapy. 18-Fluorodeoxyglucose PET/CT was a sentation being at 10.5 years of age. Histology in the useful imaging tool for judging response to therapy with complete majority of cases contains spindle or stellate cells, pleomor- loss of metabolic activity in tumor after neoadjuvant chemotherapy phic and prominent eosinophilia, with variable immunohis- tochemistry staining.1,3,4 Serum a-fetoprotein is normal. Negative myogenin staining typically differentiates UELS Received for publication May 30, 2012; accepted August 29, 2012. from rhabdomyosarcomas.5 Some have argued that UELS is From the *Department of Pediatrics, Division of Hematology/ on a spectrum with mesenchymal hamartoma.6 There have w Oncology; Department of Surgery, Division of Liver and Pancreas also been reports of association with p53 mutations.7 UELS Transplantation; Departments of zPathology; yMedical and Molecular Pharmacology, Ahmanson Biological Imaging Center/ has high metastatic potential primarily to lung and lymph Nuclear Medicine; 8The UCLA Jonsson Comprehensive Cancer nodes. Recurrences are mostly locoregional in nature, but Center, David Geffen School of Medicine; and zRonald Reagan they have been reported in the lungs, bone, and brain.3 Medical Center, Mattel Children’s Hospital, University of UELS are aggressive sarcomas and historically carried California Los Angeles, Los Angeles, CA. N.C.F. is supported by St. Baldrick’s Career Development Award and a poor prognosis before development of multimodal ther- a STOP Cancer Research Award. apy with reported survival rates of <37.5%.4,8 Previous The authors declare no conflict of interest. studies have shown success with combined surgical and Reprints: Noah C. Federman, MD, Department of Pediatrics, Division adjuvant chemotherapy regimens based on treatment of of Hematology/Oncology, 6223 W 6th St Los Angeles, CA 90048 8–11 (e-mail: [email protected]). other childhood soft tissue and hepatic malignancies. Copyright r 2012 by Lippincott Williams & Wilkins There have been several case reports of success with liver J Pediatr Hematol Oncol Volume 35, Number 6, August 2013 www.jpho-online.com | 451 Plant et al J Pediatr Hematol Oncol Volume 35, Number 6, August 2013 transplantation in refractory or unresectable cases.12,13 variable a-1 antitrypsin staining. One patient presented However, there is no standard therapeutic approach to with pulmonary metastatic disease and 1 had distant recur- these malignancies. rence of disease with pulmonary and paraspinal metastases We present 5 cases of UELS in one of the largest case at 2 years from completion of therapy. series from a single institution treated successfully with combined surgical and adjuvant chemotherapy protocols. Treatment and Outcome We also report successful outcome of total hepatectomy and Surgical resection and adjuvant chemotherapy formed orthotopic liver transplantation (OLT) in a patient with the basis of treatment for the 5 patients.8–18 Chemotherapy locally recurrent/refractory disease not amenable to surgical regimens for 3 patients consisted of 5 cycles of ifosfamide resection. We report success with adjuvant chemotherapy 3 gm/m2/dÂ3 days and doxorubicin 37.5 mg/m2 on days with an ifosfamide-based and doxorubicin-based regimen 1 and 2. Patient B received 5 cycles of actinomycin-D combined with complete surgical resection as standard of 1.5 mg/m2/d on day 1, ifosfamide 2 gm/m2/d on days 1 to 5 care for treatment of UELS with total hepatectomy and of weeks 1 and 4, doxorubicin 40 mg/m2/d on days 1 and 2 OLT as an alternative in unresectable or refractory cases. of week 4, and vincristine 1.5 mg/m2/d on day 1 of weeks 1, 2, 3, and 4 per Italian Sarcoma Protocol RMS88. Patient C received 5 cycles of cisplatin and doxorubicin (375 mg/m2 METHODS cumulative anthracycline) outside the United States. Four This is an Institutional Review Board (IRB)-approved patients underwent right hepatic lobectomy and patient C [University of California Los Angeles (UCLA) IRB ap- had an extensive hepatic surgical resection after tumoral proval #11-001743] retrospective review of 5 cases of UELS rupture. Patients B and C both received salvage chemo- in patients younger than 21 years of age seen at UCLA therapy. Patient B’s salvage regimen included 7 cycles of identified in the UCLA Sarcoma and Liver Transplant etoposide 100 mg/m2/d and ifosfamide 2800 mg/m2/dÂ5 Databases. Chart review was conducted searching for mul- days followed by weekly paclitaxel 80 mg/m2 on days 1, 8, tiple parameters: age, sex, ethnicity, signs and symptoms at and 15 of 6 cycles of 28 days. Patient C received salvage presentation, date and age of diagnosis, location of tumor, chemotherapy consisting of ifosfamide, etoposide, and car- presence and location of metastases, imaging results, his- boplatin for 2 cycles followed by another 2 cycles adjuvantly tology and pathology reports, size of tumor, chemotherapy after liver transplantation with ifosfamide and carboplatin regimen, use of any salvage chemotherapy, use of surgery, at an outside hospital (dosing information was not avail- liver transplantation, recurrence and site of recurrence, able). Both patients with metastatic disease received local morbidity and mortality, and survival in months from radiation (6000 cGy) to the sites of metastatic disease. diagnosis and months of disease-free survival. Patients were Patient B received 6000 cGy of radiation to pulmonary and assigned letters; medical record numbers, names, and other paraspinal metastases, and patient E received 6000 cGy of identifying data were removed before data analysis. Results radiation to pulmonary metastases. Patient E had extension were documented as a descriptive analysis of a case series. of his tumor into the inferior vena cava but after chemo- Patient C was treated elsewhere but was included in our therapy repeat scans revealed no evidence of further disease study, because he received OLT at our institution and was with minimal scar tissue. He was placed on Arixtra for 4 followed here subsequently.
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