Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature.
Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature.
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DOI：
10.1186/s13256-017-1554-2
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发表时间：
2018-02-17
影响因子：
1
通讯作者：
Cillo U
中科院分区：
文献类型：
--
作者：
D'Amico F;Bertacco A;Cesari M;Mescoli C;Caturegli G;Gondolesi G;Cillo U
恶性肾外横纹肌样肿瘤的胃肠道很少报道在文献中。它的特点是预后差和具有侵袭性转移特征。文献回顾仅证实19例，预后较差。我们报告一例结肠“纯”恶性肾外横纹肌样肿瘤转移淋巴结在一个65岁的白人男性。他接受了手术治疗，无复发，无辅助化疗，在提交本文时无疾病生存4年。我们提出了一个特殊的长期生存的情况下，由于延长的手术治疗。我们认为，尽管病理证实显微镜下淋巴结受累（13/38阳性），但表现时没有器官转移是一个积极的预后因素。
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. 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. 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.