| Autor: |
G, Schoofs, L, Braeye, R, Vanheste, G, Verswijvel, M, Debiec-Rychter, R, Sciot |
| Rok vydání: |
2012 |
| Předmět: |
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| Zdroj: |
Acta gastro-enterologica Belgica. 74(4) |
| ISSN: |
1784-3227 |
| Popis: |
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. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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