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

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 colon...

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Detalles Bibliográficos
Autores principales: D’Amico, Francesco, Bertacco, Alessandra, Cesari, Maurizio, Mescoli, Claudia, Caturegli, Giorgio, Gondolesi, Gabriel, Cillo, Umberto
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5816521/
https://www.ncbi.nlm.nih.gov/pubmed/29452605
http://dx.doi.org/10.1186/s13256-017-1554-2
Descripción
Sumario: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.