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

Francesco D’Amico,Alessandra Bertacco,Giorgio Caturegli,Gabriel Gondolesi,Maurizio Cesari,Umberto Cillo,Claudia Mescoli

doi:10.1186/s13256-017-1554-2

Francesco D’Amico, Alessandra Bertacco + Show 5 more

Open Access

https://doi.org/10.1186/s13256-017-1554-2

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Abstract

BackgroundMalignant 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 presentationWe 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.ConclusionsWe 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.

Highlights

Malignant extrarenal rhabdoid tumor of the gastrointestinal tract is rarely reported in the literature
Similar lesions arising in soft tissue and other sites have been reported and referred to as malignant extrarenal rhabdoid tumors (MERTs), which have been accepted as a clinical-pathological entity of their own
malignant rhabdoid tumor (MRT) were later seen to arise at different locations, such as the central nervous system (CNS) [5], liver [6], genitourinary tract [7], and GI tract [8], known with the name of extrarenal rhabdoid tumors (MERTs)

Summary

Conclusions

The current case report is, to our knowledge, the only case of a patient with colonic rhabdoid tumor with extended surgical resection with a survival of 4 years without recurrence or metastatic disease evidenced in control CT scans. All the published cases exhibit similar clinical-pathological features, as well as the characteristic histological features of sarcoma differentiation of rhabdoid cells, which appear to indicate aggressive biological behavior. Further investigations into this highly aggressive colonic carcinoma showing rhabdoid features are required in order to determine specific and effective treatment for this type of tumor

Background

Discussion

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Findings

Funding No grant supported this work