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Background  Malignant extrarenal rhabdoid tumor of the gastrointestinal tract is rarely reported in the literature. It is characterized by poor prognosis and aggressive metastatic features.  A literature review evidenced only 19 cases, with poor outcome.    Case presentation  We report a case of a colonic “pure” malignant extrarenal rhabdoid tumor with metastatic nodes in a 65-year-old Caucasian man. He was treated surgically with no recurrence, no adjuvant chemotherapy, and with 4-year survival without disease at the time of the submission of this article.    Conclusions  We present an extraordinary case of long-term survival due to the extended surgical treatment.  We believe that the absence of organ metastasis at presentation is a positive prognostic factor, although pathology confirmed node involvement (13/38 positive) on microscopy.

Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature