Abstract
Background
Leiomyosarcoma of the skin is a rare malignant mesenchymal neoplasm categorized as cutaneous or subcutaneous. This study identified demographic and pathological factors affecting the prognosis and treatment of leiomyosarcoma of the skin.
Methods
Data of patients with skin leiomyosarcoma was extracted from 2000 to 2020 using the Surveillance, Epidemiology, and End Results database. Propensity score matching and prognostic nomograms were used to analyze survival rate predictions.
Results
Most patients were >65 years of age (50.5%), male (78.8%), and White (88.5%). The overall 5-year survival was 79.9% (95% confidence interval [CI] = 77.4–82.6), and the 5-year cause-specific survival rate was 95.7% (95% CI = 94.2–97.2). Based on treatment, the 5-year cause-specific survival rate was 82.9% (95% CI = 80.3–85.6) for surgery only, 60.2% (95% CI = 47.7–76.0) for both radiation and surgery, and 68.2% (95% CI = 58.9–78.9) for no treatment. Multivariable analysis showed that head and neck tumors were associated with worse mortality.
Conclusion
The results provide valuable insights that could shape development of future clinical protocols for skin leiomyosarcoma and impact patient care and treatment efficacy.
Keywords: Leiomyosarcoma, mesenchymal neoplasm, SEER, skin cancers, survival outcomes
Leiomyosarcoma is the most common subtype of soft tissue sarcoma (STS), representing approximately 25% of all diagnosed STS.1,2 Primary leiomyosarcoma of the skin, a rare malignant mesenchymal neoplasm, originates from smooth muscle cells and accounts for 2% to 3% of all STS and 0.04% of all neoplasms.3,4 The age-adjusted incidence rate of leiomyosarcoma of the skin is 0.6 per 1,000,000 person-years.5 Although leiomyosarcoma can occur in the younger population, most cases are diagnosed in adults in their sixth to eighth decades of life.6 Leiomyosarcoma commonly arises in soft tissue locations such as the abdomen, large blood vessels, retroperitoneum, uterus, and limbs.1 Primary leiomyosarcoma has two subcategories: cutaneous and subcutaneous.7 Cutaneous leiomyosarcoma arises from the arrector pili smooth muscle and is limited to the superficial layer of the skin with no deeper infiltration. In contrast, subcutaneous leiomyosarcoma originates from vascular smooth muscle cells and invades the deeper tissue layers.3,7
The clinical presentation of leiomyosarcoma is nonspecific.1,6 The symptomatic presentation commonly seen is due to mass effect or compression of adjacent organs.1 Leiomyosarcoma can present grossly as irregular, asymmetrical, painful nodules that may have hyperpigmentation, bleeding, sclerosis, hyalinization, necrosis, or ulceration.3,6,7 Other cases have also described multilobulated plaques and pedunculated masses. Unlike cutaneous leiomyosarcoma, subcutaneous leiomyosarcoma is better described as a well-circumscribed lesion.3,6
The French Federation of Cancers Center Sarcoma Group guidelines for cancer grading are commonly used for leiomyosarcoma, focusing on three parameters: differentiation, mitotic rate, and tumor necrosis.7,8 The diagnosis relies on histological findings and immunohistochemical studies upon skin biopsy.1,6 Chemotherapy and radiation therapy can be used for treatment; however, the gold-standard treatment for leiomyosarcoma is surgical excision of the lesion with clear margins.1,3,6
Despite the progress in understanding leiomyosarcoma, existing literature lacks large-scale studies analyzing demographic trends, prognostic factors, and survival outcomes. The negative surgical margin for recurrence prevention and the role of adjuvant therapies remain poorly defined due to limited and inconsistent data. Additionally, much of the data is derived from small case series or institutional studies, which may not be generalizable to broader populations. This study is one of the most comprehensive and current database studies evaluating the demographics and pathological factors of patients with skin leiomyosarcoma. We aim to contribute insights into the clinical behavior, prognostic indicators, and potential treatment strategies for this rare malignancy by addressing some of these knowledge gaps.
METHODS
The National Cancer Institute started the Surveillance, Epidemiology, and End Results (SEER) database in 1973, centralizing cancer statistics in approximately 26.5% of the US population. Patient data in the SEER database are collected from hospital-based and regional cancer registries across the United States. When a patient is diagnosed with cancer, trained tumor registrars abstract key information—including demographics, tumor characteristics, stage at diagnosis, and treatment details—from pathology reports, surgical records, and clinical notes. These data are then submitted to regional registries, which undergo quality control checks before reporting to SEER.
SEER captures a large, population-based sample of cancer cases but does not cover the entire US population. Given its reliance on hospital and pathology reports, underreporting may occur, particularly for rare skin lesions diagnosed in outpatient settings or not undergoing histopathological confirmation. Despite this limitation, SEER remains one of the most comprehensive sources for analyzing cancer incidence, trends, and outcomes.9
Data from 2000 to 2020 were collected using the SEER*Stat software (Version 8.4.0) with International Classification of Diseases version 3 (ICD-O-3). The data collected from SEER registries included patient demographic characteristics, tumor characteristics, lymph node and metastatic status, treatment modalities, and survival outcomes. The inclusion criteria for our study included cases that had positive histology, immunophenotyping, genetic studies, or another unspecified method (Figure 1).
Figure 1.
Exclusion flowchart describing the filtering of primary cutaneous leiomyosarcoma in the US population.
R version 4.2.3 (Shortstop Beagle) was used for statistical analysis. Univariate analysis was conducted to identify significant factors for multivariate analysis. The Cox regression method was used to calculate hazard ratios (HRs) and to identify independent factors affecting survival outcomes. Unidentified or missing data were removed from multivariate analysis. The data were analyzed using multivariate Cox regression with statistical significance defined as P < 0.05.
RESULTS
In total, 1067 cases of primary cutaneous leiomyosarcoma of the skin were identified from 2000 to 2020. The median age was found to be 66 years. By ethnicity, most cases were White (88.5%). There was a relatively even split when analyzing the median household income of the patients, with 52.8% of the patients earning more than $70,000 per year. In addition, a majority (86.7%) of the patients resided in urban/metropolitan areas. In known cases of grading, 43.7% were moderately differentiated (grade II) (Table 1).
Table 1.
Demographic profiles and tumor characteristics of 1067 patients with leiomyosarcoma of the skin from the Surveillance, Epidemiology, and End Results (SEER) database, 2000 to 2020
| Category | Variable | Frequency (%) |
|---|---|---|
| Age (years) | ≤65 | 528 (49.5%) |
| >65 | 539 (50.5%) | |
| Gender | Female | 226 (21.2%) |
| Male | 841 (78.8%) | |
| Race/ethnicity | White | 944 (88.5%) |
| Black | 20 (1.9%) | |
| Asian or Pacific Islander | 4 (0.4%) | |
| American Indian or Alaska Native | 5 (0.5%) | |
| Hispanic (all races) | 55 (5.2%) | |
| Unknown | 39 (3.7%) | |
| Income | <$70,000 | 503 (47.2%) |
| $70,000+ | 563 (52.8%) | |
| Housing location | Urban | 925 (86.7%) |
| Rural | 142 (13.2%) | |
| Tumor site | Head and neck | 290 (27.4%) |
| Trunk and limbs | 770 (72.6%) | |
| Grade (n = 1067) | Known | 323 (30.3%) |
| Unknown | 744 (69.7%) | |
| Grade where known (n = 323) | Well differentiated – Grade I | 111 (34.4%) |
| Moderately differentiated – Grade II | 141 (43.7%) | |
| Poorly differentiated – Grade III | 71 (22.0%) | |
| Size (n = 764) | Known | 742 (69.5%) |
| Unknown | 325 (30.5%) | |
| Size where known (n = 325) | <2 cm | 235 (72.6%) |
| 2–4 cm | 74 (22.8%) | |
| >4 cm | 15 (4.6%) | |
| No tumor found/microscopic (0 cm) | 1 (0.3%) | |
| Lymph node status | Known | 21 (2.0%) |
| Unknown | 1046 (98.0%) | |
| Status where known (n = 21) | Positive | 1 (4.8%) |
| Negative | 20 (95.2%) | |
| Systemic therapy | Known | 766 (71.8%) |
| Unknown | 301 (28.2%) | |
| Systemic therapy when known (n = 766) |
Positive | 7 (0.9%) |
| Negative | 759 (99.1%) |
Treatment characteristics
The most common treatment modality was surgery only (84.2%); 5% of the patients received surgery with radiation. Multimodal therapy (surgery with adjuvant chemoradiation) was received by only 0.8% of patients.
Overall and cause-specific survival
The 5-year overall survival was 79.9% (95% confidence interval [CI] = 77.4–82.6). SEER calls disease-specific survival “cause-specific survival.” The 5-year cause-specific survival of the study group was 95.7% (95% CI = 94.2–97.2) (Supplemental Table 1). A Kaplan-Meier graph visualizing the 5-year survival is shown in Figure 2.
Figure 2.
Five-year survival for primary cutaneous leiomyosarcoma: (a) cause-specific survival and (b) overall survival.
Survival analysis based on demographic factors
The 5-year survival outcome for gender, specifically females, was 75.1% (95% CI = 69.2–81.4). Univariate analysis determined that sex was not a prognostic factor for survival (Figure 3a, Supplemental Table 2). The 5-year survival outcome for patients ≤60 years of age was 93.7% (95% CI = 91.5–96.0), compared with 66.6% (95% CI = 62.4–71.0) for those >60 years. Univariate analysis determined increased age to be a negative predictor for survival (HR = 6.9, 95% CI = 5.2–9.15, P < 0.001) (Figure 3b, Supplemental Table 3).
Figure 3.
Survival analysis of primary cutaneous leiomyosarcoma by (a) gender, (b) age, (c) race, and (d) median annual household income.
The 5-year survival outcomes for White patients, Black patients, and Hispanic patients were 78.9% (95% CI = 76.2–81.7), 82.3% (95% CI = 66.9–100), and 87.6% (95% CI = 77.8–98.6), respectively (Supplemental Table 5). Univariate analysis found race to be a prognostic factor for survival, with White patients having a worse survival outcome (HR = 1.91, 95% CI = 1.01–3.71, P = 0.05). Asian and Native American patients were not included in the analysis due to low power (Figure 3c).
The 5-year survival for patients with a median household income ≤$70,000 was 77.5% (95% CI = 73.8–81.4), compared with 82.1% (95% CI = 78.7–85.7) for those with a median household income >$70,000. Median household income classification was not included in multivariate analysis since the P value was lower than the screening threshold (P = 0.08). Housing location was not determined to be a prognostic factor for survival (Figure 3d, Table 2, Supplemental Table 4).
Table 2.
Multivariate analysis of independent factors influencing mortality of primary cutaneous leiomyosarcoma
| Variables | Univariate |
Multivariate |
|||
|---|---|---|---|---|---|
| HR (95% CI) | P value | HR (95% CI) | P value | ||
| Age | ≤60 years | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| >60 years | 6.91 (5.22–9.15) | <0.001 * | 3.74 (1.74–8.04) | <0.001 | |
| Race | Hispanic | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| White | 1.91 (1.01–3.71) | 0.05 * | 0.85 (0.24–2.96) | 0.80 | |
| Median household income | ≤$70,000/year | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| >$70,000/year | 0.83 (0.67–1.02) | 0.08 | 1.66 (0.86–3.21) | 0.13 | |
| Tumor site | Head and neck | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| Trunk and limbs | 0.30 (0.24–0.38) | <0.001 | 0.49 (0.24–0.99) | 0.04 | |
| Grade | Moderately differentiated (Grade II) | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| Poorly differentiated (Grade III) | 2.23 (1.42–3.50) | <0.001 | 1.43 (0.63–3.28) | 0.39 | |
| Tumor size | <2.0 cm | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| 2.0–4.0 cm | 1.42 (0.93–2.19) | 0.11 | 1.32 (0.61–2.87) | 0.48 | |
| >4.0 cm | 2.81 (1.35–5.84) | 0.006 | 6.86 (1.96–24.06) | 0.003 | |
| Treatment modality | None | 1 (reference) | 1 (reference) | 1 (reference) | 1 (reference) |
| Surgery only | 0.52 (0.37–0.73) | <0.001 | 0.80 (0.07–9.82)c | 0.86 | |
CI indicates confidence interval; HR, hazard ratio.
Tumor characteristic and treatment survival
The 5-year survival outcome of primary cutaneous leiomyosarcoma located on the head and neck was 63.1% (95% CI = 57.5–69.2). Survival for primary cutaneous leiomyosarcoma located on the trunk and limbs was 86.9% (95% CI = 84.4–89.6). Univariate analysis revealed that cutaneous leiomyosarcoma found on the trunk and limbs had a significantly better prognosis and survival outcome (HR = 0.30, 95% CI = 0.24–0.38), P < 0.001) (Figure 4a).
Figure 4.
Survival analysis of primary cutaneous leiomyosarcoma by (a) tumor site, (b) grade, (c) tumor size, and (d) treatment modality.
The 5-year survival outcome was 81.1% (95% CI = 74.0–88.9) for tumors that were well differentiated (Grade I), 84.1% (95% CI = 78.2–90.4) for moderately differentiated (Grade II), and 73.9% (95% CI = 64.3–85.1) for poorly differentiated (Grade III). Univariate analysis found that poorly differentiated/grade III tumors had a worse survival outcome (HR = 2.23, 95% CI = 1.42–3.50, P < 0.001) (Figure 4b, Supplemental Table 8).
The 5-year survival outcome of tumors <2.0 cm in size was 85.0% (95% CI = 80.6–89.7); 2.0 to 4.0 cm in size, 74.2% (95% CI = 64.9–84.9); and >4.0 cm in size, 50.9% (95% CI = 30.5–85.1). Univariate analysis found that tumors >4.0 cm had a worse survival outcome (HR = 2.81, 95% CI = 1.35–5.84; P = 0.006) (Figure 4c, Supplemental Table 6).
The 5-year survival of patients who underwent surgery only for treatment was 82.9% (95% CI = 80.3–85.6), compared with 60.2% (95% CI = 47.7–76.0) for those who underwent radiation therapy and surgery. The 5-year survival of patients who did not undergo any documented treatment was 68.2% (95% CI = 58.9–78.9) (Supplemental Table 7). Other treatment modalities were not included in analysis because of low power. Univariate analysis found that patients who underwent surgery only had a significantly higher survival outcome (HR = 0.52, 95% CI = 0.37–0.73, P < 0.001) (Figure 4d).
Multivariate analysis and propensity score matching
On multivariable analysis, independent factors associated with increased mortality were identified as age >60 years (HR = 3.74, 95% CI = 1.74–8.04; P < 0.001) and tumor size >4.0 cm (HR 6.86, 95% CI = 1.96–24.06; P = 0.003). A factor associated with decreased mortality was tumors of the trunk and limbs (HR = 0.49, 95% CI = 0.24–0.99; P = 0.04) (Table 2). Propensity score matching showed that increased age and tumors of the head and neck were correlated with increased mortality (P = 0.007 and P = 0.002, respectively).
A prognostic nomogram was created to measure patient survivability and showed increased mortality for patients with an older age, tumors of the head and neck, and tumors >40 mm (4.0 cm) (Figure 5).
Figure 5.
Prognostic nomogram predicting mortality of patients with primary cutaneous leiomyosarcoma.
DISCUSSION
To the best of our knowledge, this study includes the largest cohort of patients with leiomyosarcoma of the skin to be published. We found that men had a higher overall 5-year survival rate than women (96.6% vs 92.7%). However, previous studies have demonstrated that leiomyosarcoma of the skin predominantly affects men more than women.8,10 In our study, 78.4% of patients were men. In another analysis of 82 cases of superficial leiomyosarcoma, 59% were male.11 We also found that the Black population had a higher 5-year survival rate of 100% versus their White counterparts at 95.8%. However, studies have noted that leiomyosarcoma of the skin affects the White race more frequently than other races.7,12 Likewise, in our study 93.6% of patients identified as White and only 2% as Black, leading to a potential discrepancy in our overall 5-year survival rate between White and Black patients. The overall 5-year survival rate of our data was 83.9%, which is lower than a Minnesota study that found a 98% survival rate and a French study that found an 86% survival rate.11,13 Although our observed overall 5-year survival rate was lower than that described in existing studies, leiomyosarcoma of the skin still has an overall good prognosis.
Studies with smaller population samples did not find significantly different disease-specific mortality based on age.11,13 This contrasts with our study, as we found that age >60 years was associated with increased mortality (P < 0.05). The Minnesota study found all disease recurrences occurred in subcutaneous leiomyosarcoma (17%) versus none in cutaneous leiomyosarcoma (P = 0.02).14 Similarly, in our study of skin leiomyosarcoma, metastatic disease was absent except for one lung metastasis (0.2%) and one liver and lung metastasis (0.2%). Metastasis, high-grade lesions, subcutaneous localization, and size >5 cm are parameters that may classify the neoplasm as a high-risk leiomyosarcoma.7
The gold-standard treatment for skin leiomyosarcoma is surgical resection, as it has been proven successful in decreasing the risk of local recurrence.6,7,15 Correspondingly, of our patients with known treatment status, 85% had only surgical intervention. Although surgical resection is considered the first-line treatment for leiomyosarcoma, there is no established consensus on the width of safety margins. Recommendations vary from at least 1 cm to 2 to 5 cm. However, if wide excision is not feasible, there is limited evidence that Mohs surgery may be recommended; previous studies showed recurrence rates from 0% to 14% after Mohs surgery.6,14,16,17
Furthermore, adjuvant therapy in the treatment of skin leiomyosarcoma remains unstandardized and controversial, with limited known survival benefits.7,12,16,18 In our study, only 5.88% of individuals with known treatment status underwent adjuvant therapy, including surgery with chemotherapy, surgery with radiation, chemotherapy with radiation, and surgery with chemotherapy and radiation. Some studies have not recommended adjuvant radiotherapy or chemotherapy due to the indolent course of skin leiomyosarcoma, but it may be recommended for patients with higher-risk leiomyosarcoma.7,11,18 Interestingly, the standard treatment for leiomyosarcoma in Scandinavia is surgery with radiotherapy.8 From the SEER database, of all the treatment modalities, the combination of surgery and adjuvant radiation (5%) was the second most used treatment for skin. Therefore, at this time, the decision to include adjuvant therapy in the management of skin leiomyosarcoma should be individualized to each patient to minimize the likelihood of recurrence.12
Due to the low incidence of STS along with the lack of specific molecular markers for the disease and its many subtypes, most clinical trials investigating potential therapeutics include a wide array of applicable smooth muscle neoplasms.19 Small molecule inhibitors are currently being investigated in STS and its subtypes.2,20–24 Sorafenib, an angiogenesis inhibitor in phase II trials, showed modest activity in various STS subtypes but had hematologic toxicity when combined with other chemotherapeutic agents.20,21 Regorafenib, an oral multikinase inhibitor, demonstrated no difference in overall survival with refractory STS but increased progression-free survival relative to the placebo group.22,23 Other studies have identified the role that tyrosine kinase-like orphan receptor 2 (ROR2) plays in the invasiveness of leiomyosarcoma, establishing its potential as a prognostic marker and novel target for future personalized therapies in patients with leiomyosarcoma.2,24 PD-L1 expression is a common therapeutic target in many cancers and is identified in 11% of leiomyosarcoma cases.25,26 In a soft tissue leiomyosarcoma case report, the patient with PD-L1 expression had a complete response to pembrolizumab; however, another study found conflicting evidence, in that in their leiomyosarcoma population subset, their 10 patients showed no response to pembrolizumab.27,28
Despite the informative insights from this study, it is important to acknowledge the inherent limitations associated with the use of the SEER database. The generalizability of findings may be limited by the database’s demographic and geographic representation. Additionally, certain diagnoses, such as cancer familial syndromes, are not captured within SEER, limiting the completeness of the data. The rarity of skin leiomyosarcoma further complicates analysis, as specific recurrence rates following Mohs surgery and their impact on survival remain unclear. Moreover, the complexity of surgical interventions and their outcomes can vary widely, and factors outside of those captured by the SEER database, such as surgical technique, surgeon experience, and institutional protocols, may significantly influence patient prognosis.
In conclusion, our study demonstrated that age >60 years old, head and neck tumors, and tumors >4.0 cm in size had the highest impact on mortality in patients with leiomyosarcoma of the skin. Surgical intervention was associated with improved 5-year survival. Due to the rarity and heterogenous nature of cancer, very little is understood about the genetic alterations of skin leiomyosarcomas.
Supplementary Material
Disclosure statement
The authors report no funding or conflicts of interest.
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