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. 2021 Jan 1;18(4):911-920.
doi: 10.7150/ijms.51186. eCollection 2021.

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

Wen Cai  1   2 , Xue Liu  3 , Weiting Ge  2 , Dehao Wu  2 , Junxi Xu  3 , Rui Bai  3 , Hanguang Hu  3
Affiliations

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

Wen Cai et al. Int J Med Sci. .

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: Epidemiology; Malignant rhabdoid tumor; Risk factors; SEER.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interest exists.

Figures

Figure 1
Figure 1
Flow chart of selecting patients.
Figure 2
Figure 2
Incident of different age groups.
Figure 3
Figure 3
A: Survival of different age groups. B: Different primary site composition of different age groups.
Figure 4
Figure 4
The treatment choices of different primary sites.
Figure 5
Figure 5
The relationship between treatment choices and overall survival in different primary sites. A: Treatment choices and different survival of all MRTs. B: Treatment choices and different survival of renal MRTs. C: Treatment choices and different survival of CNS MRTs. D: Treatment choices and different survival of bone and soft tissue MRTs. E: Treatment choices and different survival of digestive tract MRTs. F: Treatment choices and different survival of other uncommon sites MRTs.
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