Primary sarcoma of the liver: A nationwide analysis of a rare mesenchymal tumor.

BACKGROUND AND OBJECTIVES: Primary liver sarcomas are rare malignancies. Prognostic factors associated with long-term survival remain poorly understood. The objective of this study is to determine factors associated with long-term survival. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with visceral sarcoma arising from the liver. Demographic factors, tumor characteristics, resection status, and survival were evaluated. Multivariate Cox regression analysis was performed to determine predictors of survival. RESULTS: A total of 743 patients with primary hepatic sarcoma were identified. The median tumor size was 10 cm. Only 30% (n = 221) of patients in the cohort underwent surgery. The 5-year overall survival rates were 47.9% for localized disease, 29.5% for regional disease, and 16.5% for distant disease, p < 0.001. Among patients who underwent surgical resection, patients with embryonal sarcoma had better 5-year survival compared with angiosarcoma and other histologic subtypes. On multivariate analysis, surgery was associated with improved survival, while older age, higher stage, and angiosarcoma histology were the strongest independent predictors of poor survival. CONCLUSIONS: Surgery remains the mainstay of treatment for this rare malignancy but is performed in less than one-third of patients. Angiosarcoma histology is associated with worse overall survival, while surgical resection remains the strongest predictor of improved overall survival.

Establishment of a staging system for visceral sarcoma.

BACKGROUND: Visceral sarcoma is a rare malignancy with a poor prognosis. However, there is no recommended prognostic staging system for the malignant disease. METHOD: We analyzed the data of patients diagnosed with primary soft tissue sarcoma (STS) of the abdomen and thoracic visceral organs between 2006 and 2017 at our hospital. Prognostic factors (size, tumor grade, and lymph node metastasis) were analyzed in our cohort (n = 203) and the SEER validation cohort (n = 5826). RESULTS: Tumor size, grade, and lymph node metastasis were important prognostic factors for visceral sarcoma in both our and the SEER cohorts. Based on these prognostic factors, we established a new staging system for visceral sarcoma, by which patients could be stratified into clinically meaningful and non-overlapping stages in both our cohort and the SEER validation series. Moreover, the area under the curve (AUC) value of the staging system for 5-year survival was 0.84 (95% CI: 0.78-0.89) in our series and 0.80 (95% CI: 0.79-0.81) in SEER series, respectively. In addition, compared with the widely used FIGO staging system for female genital sarcoma, the visceral sarcoma staging system could more effectively and reliably stratify patients into four different prognostic groups. CONCLUSIONS: The visceral sarcoma staging system is applicable for STS of the abdomen and thoracic visceral organs and is better than the current FIGO staging system for female genital sarcoma and should be incorporated into the AJCC Cancer Staging Manual.

Clinicopathologic features, prognostic factors, and outcomes of visceral sarcomas: A retrospective 12-year single-center study.

Background: Visceral sarcomas are a rare form of soft tissue sarcoma. This study aimed to evaluate the survival and prognostic factors and effective treatments for visceral sarcomas. Methods: All patients with visceral sarcoma referred to our center between January 2010 and December 2021 were retrospectively analyzed. The Kaplan-Meier method and a log-rank test were used for survival analysis. Results: A total of 53 patients with visceral sarcoma were analyzed in this study with the median age at diagnosis of 57 (range, 24-77) years. Among them, 37 (69.8%) and 16 (30.2%) patients had localized and metastatic diseases at the initial presentation, respectively, and 44 patients underwent surgical resection. The median follow-up, event-free survival (EFS) and overall survival (OS) were 63.0 (range, 2-130), 42.0 months (95% confidence interval [CI] 10.879-73.121) and 45.0 months (95% CI 9.938-80.062), respectively. The 5-year EFS and OS rates were 44% and 46%, respectively. Univariate analysis of prognostic indicators illustrated that metastasis at presentation, surgery, surgical margin and the types of surgery were significantly associated with OS and EFS. In this study, combined chemotherapy or radiotherapy had no effects on EFS and OS. Conclusion: Primary visceral sarcoma is an uncommon and aggressive malignant tumor with a higher rate of local recurrence. In the largest cohort of visceral sarcomas in China to date, we identified metastases at presentation, surgery, surgical margin, and the types of surgery as independent predictors of survival. The combination of chemotherapy and radiotherapy did not affect survival.

Surgical management of GIST and intra-abdominal visceral leiomyosarcomas.

Intra-abdominal visceral sarcomas are a rare group of mesenchymal tumors that require the use of distinct surgical principles and a multidisciplinary approach in order to optimize treatment. Surgery remains the only potentially curative treatment. Management and outcomes vary for the different sarcomas arising from viscera. This review will summarize the current treatment strategies for the two most common visceral sarcomas, leiomyosarcomas, and gastrointestinal stromal tumors.