Title: Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review
Authors: Schoofs, G ×
Braeye, L
Vanheste, R
Verswijvel, G
Debiec-Rychter, Maria
Sciot, Raf #
Issue Date: Dec-2011
Publisher: Acta Medica Belgica
Series Title: Acta Gastro-Enterologica Belgica vol:74 issue:4 pages:576-581
Abstract: 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.
ISSN: 0001-5644
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Department of Human Genetics - miscellaneous
Laboratory for Genetics of Malignant Disorders
Translational Cell & Tissue Research
× corresponding author
# (joint) last author

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