Maxillary undifferentiated carcinoma with rhabdoid features.

: The rhabdoid malignant tumor was individualized by Haas et al. as an independent entity within the kidney malignant neoplasms in 1981. From its histopathological characteristics, different cases of rhabdoid extra-renal tumor were documented. This aspect has been also recognized in a wide heterogeneous group of tumors (carcinoma, melanoma, mesothelioma and mesenchymal tumors). The diagnosis of extrarenal rhabdoid tumor is based on the presence of a proliferation of epithelioid cells with large nuclei, prominent nucleoli, abundant eosinophilic cytoplasm, and conspicuous cytoplasmic intermediate filaments, which compress the nuclei. In this article we present the case of a 50-year-old male patient with a malignant maxillary tumor with characteristic rhabdoid features. Its differential diagnosis will be discussed. The literature will also be reviewed.