Comparison of clinicopathological features and outcomes in patients with primary leiomyosarcoma of bone and soft tissue

Abstract

Backgrounds and Objectives This investigation sought to describe the outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS). Methods This was a review of the Surveillance, Epidemiology, and End Results database from 1975 to 2016. Kaplan‐Meier methods were used to estimate disease‐specific survival (DSS), and a Cox regression model was used to identify prognostic factors. Results Of the 7502 identifiable cases, 1% (n = 74) were PLB and 99% (n = 7428) were SLMS. Survival was the same between PLB and SLMS (p = .209). On multivariable analysis for high‐grade SLMS, radiation (neoadjuvant: hazard ratio [HR], 0.56; 95% confidence interval [CI], 0.4–0.8; p = .003; adjuvant: HR, 0.75; 95% CI, 0.6–0.9; p = .008) and surgery (procedure specific) improved DSS. For PLB, wide resection/limb salvage (HR, 0.40; 95% CI, 0.3–0.5; p = .018) and amputation (HR, 0.69; 95% CI, 0.5–0.9; p < .001) were positive prognostic factors. Neither radiation nor chemotherapy were prognostic factors for survival in PLB. Conclusions For SLMS, radiation portends a survival advantage. For PLB, however, neither chemotherapy nor radiation were significant prognostic factors, which suggests the optimal treatment for PLB, similar to other primary soft tissue sarcomas originating in bone, remains an unmet medical need.

Case report and literature review: Primary leiomyosarcoma of the bone in the trochanteric region of the femur

“…However, in recent years, limb salvage has been conducted more often (12). Currently, clinicians commonly use limb salvage surgical methods, including prosthesis reconstruction with tumor resection and prosthesis replacement and conventional internal fixation with a series of surgical procedures including lesion resection, bone cement filling, and implant fixation (12,15,(32)(33)(34)(35)(36)(37)(38).…”

“…LMSB is a rare and highly-invasive leiomyosarcoma that is difficult to diagnose and treat ( 7 ). The incidence of LMSB is slightly lower in men than in women ( 12 ), and the 5-year disease-specific survival rate of limb LMSB is 55%, while the median disease-specific survival after diagnosis is 61 months (95% confidence interval: 36–85 months) ( 15 ). Patients with metastasis at the initial diagnosis have worse prognoses ( 8 ).…”

“…A previous study showed that the average age of patients with LMSB was 46 years (range: 9–88 years) ( 12 ). The optimal treatment for LMSB is unclear, and surgery, including tumor resection and limb salvage or hip amputation, is a widely-accepted treatment modality ( 15 , 16 ). After successful limb salvage, the patient in this report was monitored for more than 50 months and showed no local recurrence or metastasis and satisfactory joint function.…”

“…LMSB is often misdiagnosed as metastatic bone leiomyosarcoma, undifferentiated pleomorphic sarcoma (UPS), fibroblastic osteosarcoma, and metastatic sarcomatoid carcinoma ( 7 ). Metastatic bone leiomyosarcoma is ruled out by the absence of primary lesions in the gastrointestinal tract or uterus via imaging ( 7 , 15 ). The diagnosis of UPS, also known as high-grade malignant fibrous histiocytotumor, is typically exclusionary.…”

Comparison of clinical features between patients with bone and soft tissue fibrosarcomas

“…Descriptive epidemiological studies using large cancer databases have been reported in the field of rare cancers and are showing new directions in the treatment of patients. 7,[11][12][13][14] We, therefore, hypothesized that the use of large cancer databases could clarify the impact of fibrosarcoma developing in bone on clinical characteristics. The purpose of this study was to investigate the impact of different sites of fibrosarcoma development on clinical features and to clarify risk factors affecting survival in each site.…”