一例罕见的肾外横纹肌样恶性肿瘤患者的超长无病生存期：病例报告及文献综述
Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature.
作者信息
D'Amico Francesco, Bertacco Alessandra, Cesari Maurizio, Mescoli Claudia, Caturegli Giorgio, Gondolesi Gabriel, Cillo Umberto
机构信息
Department of Surgery, Oncology and Gastroenterology, Hepatobiliary Surgery and Liver Transplantation, Padova University, Padova, Italy.
Department of Surgery, Division of Transplantation and Immunology, Yale University, New Haven, CT, USA.
出版信息
J Med Case Rep. 2018 Feb 17;12(1):39. doi: 10.1186/s13256-017-1554-2.
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.
背景
胃肠道恶性肾外横纹肌样瘤在文献中报道较少。其特点是预后差和具有侵袭性转移特征。文献综述仅证实有19例，预后不良。
病例报告
我们报告1例65岁白种男性的结肠“纯”恶性肾外横纹肌样瘤伴转移淋巴结。他接受了手术治疗，无复发，未进行辅助化疗，在本文提交时已存活4年且无疾病。
结论
我们展示了1例因广泛手术治疗而长期存活的特殊病例。我们认为，尽管病理检查在显微镜下证实有淋巴结受累（38个中有13个阳性），但就诊时无器官转移是一个积极的预后因素。
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