Background/Aim: Few studies have examined the prognostic significance of histological subtypes in patients with soft tissue sarcoma (STS) and distant metastasis, and we evaluated the relationship between the histological subtypes and clinical outcomes. Patients and Methods: This retrospective study evaluated the histological subtypes of 105 patients with STS and distant metastasis. The STS histological subtypes were compared based on the clinical information. Results: The Kaplan-Meier curves for overall survival revealed that myxoid liposarcoma had a significantly better prognosis compared to Malignant Peripheral Nerve Sheath Tumor (MPNST) (p=0.0221). In the multivariate logistic regression analyses, the independent predictors of a poor prognosis were: i) large size, ii) advanced stage, and iii) non-surgical treatment for metastasis (p<0.05). The presence or absence of lung metastasis was not significantly associated with prognosis (p=0.4452). Conclusion: Myxoid liposarcoma had a better prognosis compared to MPNST in STS patients with distant metastasis. The surgical removal of distant metastatic lesions may improve the patient's prognosis. Soft tissue sarcoma (STS) is relatively rare, and mainly occurs in middle-aged or older individuals. Despite its low incidence, STS has various histological subtypes, such as liposarcoma and synovial sarcoma, which results in relatively small populations of patients with different histological types. Radical resection with adequate margins is important for treating STS, regardless of the histological subtype, in order to prevent local recurrence. However, recent developments in surgical treatment, radiotherapy, and chemotherapy have improved patient outcomes (1), and various studies have identified factors associated with prognosis among STS patients (2-10). There is consensus that distant metastasis, which mainly involves the lungs, has a strong influence on prognosis. However, the small sample sizes for each subtype typically prevent studies from identifying a clear relationship between subtype and prognosis, despite the fact that several reports have examined the prognostic significance of the STS histological subtypes (2-8, 11, 12). Moreover, only few studies have examined this topic among patients with STS and distant metastases. Therefore, the present study aimed to examine the association of clinical outcomes with the histological subtypes and distant metastasis sites in patients with STS and distant metastasis. 1975 This article is freely accessible online.