Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review.

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.

• Authors: G Schoofs, L Braeye, R Vanheste, G Verswijvel, M Debiec-Rychter, R Sciot
• Journal: Acta gastro-enterologica Belgica (Acta Gastroenterol Belg) Vol. 74 Issue 4 Pg. 576-81 (Dec 2011) ISSN: 1784-3227 [Print] Belgium
• PMID: 22319971 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Fatal Outcome
• Female
• Humans
• Immunohistochemistry
• In Situ Hybridization, Fluorescence
• Liver Neoplasms (drug therapy, metabolism, pathology, surgery)
• Magnetic Resonance Imaging
• Middle Aged
• Rhabdomyosarcoma, Alveolar (drug therapy, metabolism, pathology, surgery)