Document Detail


Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review.
MedLine Citation:
PMID:  22319971     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Rhabdomyosarcomas are malignant tumors that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children and young adults. In mature adults however there are very rare. The liver as a primary site in adults has only been described in 12 cases. We report a case of a primary alveolar rhabdomyosarcoma of the liver in a 59 year old female, confirmed by histological examination using immunohistochemical analysis (positive actin, desmin, vimentin and myogenin staining) and fluorescent in situ hybridization (FISH) analysis (positivity for PAX3/FOXO1A fusion). The patient underwent primary surgical resection, but presented a few weeks after surgery already with recurrent disease in the abdomen and bone metastasis. Despite initial good response to chemotherapy (doxorubicin/ifosfamide) and stable disease at 12 months after diagnosis, the patient died 31 months after the first presentation secondary to complicated abundant abdominal recurrent disease. We further present a review of the literature on published similar cases since 1979.
Authors:
G Schoofs; L Braeye; R Vanheste; G Verswijvel; M Debiec-Rychter; R Sciot
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Acta gastro-enterologica Belgica     Volume:  74     ISSN:  0001-5644     ISO Abbreviation:  Acta Gastroenterol. Belg.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2012-02-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0414075     Medline TA:  Acta Gastroenterol Belg     Country:  Belgium    
Other Details:
Languages:  eng     Pagination:  576-81     Citation Subset:  IM    
Affiliation:
Department of Gastroenterology, University Hospital Leuven, Belgium. goeleschoofs@hotmail.com
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