Treatment of Undifferentiated Embryonal Sarcoma of the Liver

Treatment Options
Treatment Options Under Clinical Evaluation for Undifferentiated Embryonal Sarcoma of the Liver

Undifferentiated embryonal sarcoma of the liver is so rare that only small series have been published regarding treatment. However, use of aggressive chemotherapy regimens seems to have improved the overall survival. The generally accepted approach is to resect the primary tumor mass in the liver when possible. Neoadjuvant chemotherapy can be effective in decreasing an unresectable primary tumor mass, resulting in resectability.[1-4] There are relatively few survivors even after complete resection of disease.[5] In several small series of patients receiving adjuvant chemotherapy, the survival has been somewhat better.[4,6-9] Liver transplantation has on occasion been used successfully to treat an otherwise unresectable primary tumor.[10,11] In a prospective series from the Italian and German Soft Tissue Sarcoma Cooperative Groups, patients were treated with conservative surgery or biopsy followed by neoadjuvant chemotherapy consisting of varying combinations of vincristine, cyclophosphamide, dactinomycin, doxorubicin, and ifosfamide. Disease evaluation, usually after four cycles of chemotherapy, was followed by second-look surgery when appropriate to try to remove residual primary tumor followed by additional and/or adjuvant chemotherapy. Ten of 17 patients survived in their first complete remission, and one patient survived in their third complete remission.[6]

The following treatment option is under investigation in a Children's Oncology Group (COG) clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

• COG study COG-ARST0332 (Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma) for treatment of embryonal sarcoma of the liver. Patients with resectable disease receive adjuvant chemotherapy; those with unresectable disease receive neoadjuvant chemotherapy. Chemotherapy consists of doxorubicin and ifosfamide. Unlike the treatment for other non-rhabdomyosarcoma soft tissue sarcomas, radiation therapy is optional.

References

1. Chowdhary SK, Trehan A, Das A, et al.: Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst. J Pediatr Surg 39 (1): E9-12, 2004.  [PUBMED Abstract]
   
   
2. Baron PW, Majlessipour F, Bedros AA, et al.: Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection. J Gastrointest Surg 11 (1): 73-5, 2007.  [PUBMED Abstract]
   
   
3. Kim DY, Kim KH, Jung SE, et al.: Undifferentiated (embryonal) sarcoma of the liver: combination treatment by surgery and chemotherapy. J Pediatr Surg 37 (10): 1419-23, 2002.  [PUBMED Abstract]
   
   
4. Webber EM, Morrison KB, Pritchard SL, et al.: Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center. J Pediatr Surg 34 (11): 1641-4, 1999.  [PUBMED Abstract]
   
   
5. Stocker JT: Hepatic tumors in children. Clin Liver Dis 5 (1): 259-81, viii-ix, 2001.  [PUBMED Abstract]
   
   
6. Bisogno G, Pilz T, Perilongo G, et al.: Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer 94 (1): 252-7, 2002.  [PUBMED Abstract]
   
   
7. Horowitz ME, Etcubanas E, Webber BL, et al.: Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children. Results of therapy. Cancer 59 (3): 396-402, 1987.  [PUBMED Abstract]
   
   
8. Walker NI, Horn MJ, Strong RW, et al.: Undifferentiated (embryonal) sarcoma of the liver. Pathologic findings and long-term survival after complete surgical resection. Cancer 69 (1): 52-9, 1992.  [PUBMED Abstract]
   
   
9. Urban CE, Mache CJ, Schwinger W, et al.: Undifferentiated (embryonal) sarcoma of the liver in childhood. Successful combined-modality therapy in four patients. Cancer 72 (8): 2511-6, 1993.  [PUBMED Abstract]
   
   
10. Okajima H, Ohya Y, Lee KJ, et al.: Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure. J Pediatr Surg 44 (2): e33-8, 2009.  [PUBMED Abstract]
    
    
11. Kelly MJ, Martin L, Alonso M, et al.: Liver transplant for relapsed undifferentiated embryonal sarcoma in a young child. J Pediatr Surg 44 (12): e1-3, 2009.  [PUBMED Abstract]