Int J Med Sci 2021; 18(4):911-920. doi:10.7150/ijms.51186 This issue

Research Paper

Factors Affecting the Outcomes of Patients with Malignant Rhabdoid Tumors: A Population-Based Study

Wen Cai1,3, Xue Liu2, Weiting Ge3, Dehao Wu3, Junxi Xu2, Rui Bai2, Hanguang Hu2✉

1. Departments of Gastroenterology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
2. Departments of Medical Oncology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China
3. Cancer Institute (Key Laboratory of Cancer Prevention and Intervention, China National Ministry of Education), The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310009, P.R. China

This is an open access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/). See http://ivyspring.com/terms for full terms and conditions.
Citation:
Cai W, Liu X, Ge W, Wu D, Xu J, Bai R, Hu H. Factors Affecting the Outcomes of Patients with Malignant Rhabdoid Tumors: A Population-Based Study. Int J Med Sci 2021; 18(4):911-920. doi:10.7150/ijms.51186. Available from https://www.medsci.org/v18p0911.htm

File import instruction

Abstract

Objective: Malignant rhabdoid tumor (MRT) is a rare but aggressive malignancy. It has been a long time since data on this tumor have been updated.

Methods: We retrospectively reviewed patients from the SEER database who were pathologically diagnosed with MRT and analyzed incidence rates, clinical features and survival using Stata 12.0.

Results: In total, 544 patients were included in the epidemiological analysis. There were two peak periods of MRT incidence: patients younger than 4 years and those older than 70 years. Further survival analysis showed that the survival of children (especially younger than 1 year) was markedly worse than that of adults (P<0.01), and different primary sites were associated with different age groups and survival outcomes. The central nervous system (CNS) was the most common primary site (50.00%), followed by the kidney (15.66%). Patients with MRTs that originated from the digestive system experienced worse survival outcomes than those with MRTs originating from other locations. Primary site surgery conferred survival benefits to patients with renal and digestive system MRTs (HR = 0.06, CI: 0.02-0.23, P<0.01; HR=0.10, CI: 0.02-0.48, P<0.01), whereas radiotherapy conferred benefits to patients with CNS, bone and soft tissue MRTs (HR=0.22, CI: 0.15-0.34, P<0.01; HR=0.44, CI: 0.21-0.90 P=0.03).

Conclusions: Our results indicate that age and the primary site of MRT are critical clinical factors that affect patient survival and treatment choices. Primary site tumor resection should be considered for renal and digestive system MRTs, and systematic therapy, including surgery and radiotherapy, should be recommended for the treatment of CNS, bone and soft tissue MRTs.

Keywords: Malignant rhabdoid tumor, SEER, Risk factors, Epidemiology