Malignant rhabdoid tumor (MRT) is a very rare liver tumor, with only a few cases reported in the literature. MRT generally occurs in pediatric patients and prognosis is usually very poor. Here we report a very rare case of MRT occurring in a young adult who is still alive with no sign of recurrence at 41 months of follow-up. MRI and computed tomography scans revealed a voluminous heterogeneous mass in the left liver with no specific pattern. The mass included necrotic and fibrous components. Histology showed fusiform, loosely cohesive cells with abundant eosinophilic cytoplasm resulting in eccentric nuclei, thus creating the characteristic rhabdoid appearance. Immunohistochemical studies revealed a lack of nuclear INI1 protein expression. The patientu27s treatment included a major left liver resection associated with chemotherapy. A thorough search of the literature revealed one case of MRT in a young adult who died at 48 months of follow-up. A less malignant nature of the tumor in young adults may be suspected, but a longer disease-free survival may also be the fruit of aggressive surgical and oncological treatment.
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