Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review.
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.
Department of Gastroenterology, University Hospital Leuven, Belgium. goeleschoofs@hotmail.com
G
G
Schoofs
L
L
Braeye
R
R
Vanheste
G
G
Verswijvel
M
M
Debiec-Rychter
R
R
Sciot
D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008113:Liver Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018232:Rhabdomyosarcoma, Alveolar / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000601:surgery