Comparing epidemiological and clinical data from RPS patients documented in a German cancer registry to a cohort from TARPSWG reference centres

Demographic data

Characteristics of the TARPSWG and CR cohort are shown in Table 1. While the gender distribution was comparable in both cohorts, strong age differences were observed with a median age of 64 years in the CR and 58 years in TARPS cohort (p < 0.0001). While 23.9% in the CR cohort were 75 years or older, the proportion was only 8.7% in the TAPRS cohort.

Table 1 Comparison of patient demographics, tumour characteristics and treatments received: comparison of CR and TARPSWG cohortsTumour data

The CR cohort had fewer Grade 1/2 than TARPSWG (51.9% vs. 72.6%), and a correspondingly greater proportion of Grade 3 (48.1% than 27.4%). This difference remained significant after adjustment for age and sex (p < 0.0001). In terms of histological subtypes, the proportion of DDLS (37.1% CR vs. 37% TARPSWG) and LMS (20.1% vs. 19.2%) was comparable between both cohorts. By contrast, there were differences in the proportion of WDLS (15.1% vs. 26.1%), MPNST (0.8% vs. 3.3%), SFT (0.5% vs. 5.9%) and the group of “other” histological subtypes (22.8% vs. 6.3%). Histological distributions remained different after adjustment (p < 0.0001). The resection margin status was similar in both cohorts (adjusted p = 0.7608).

Treatment data

There was no significant difference in the proportion of patients who received chemo- and/or radiation therapy between the two cohorts (adjusted comparison: p = 0.7175 for chemotherapy, p = 0.6208 for radiotherapy). With respect to the timing of therapy, planned preoperative (neoadjuvant) therapy was given less often in the CR cohort than the TARPSWG cohort (chemotherapy: 7.1% vs. 15.1%, p = 0.0001; radiotherapy: 14% vs. 21.7%, p = 0.0016). This pattern remained significant after adjustment for age and sex. Postoperative adjuvant chemotherapy was given more commonly in the CR cohort (9.3% vs. 3.9%, p = 0.0001). After adjustment, post-operative radiotherapy was also given more commonly in the CR cohort (p = 0.0087).

Overall survival

As shown in Fig. 2a, overall survival (OS) at 5 years following resection was lower in the CR than in the TARPSWG cohort (56.3% vs. 67.9%, p = 0.0015). After adjustment for age, sex, histotype and r-status the hazard ratio (HR) was 0.99 (0.77–1.29).

Fig. 2figure 2

Overall survival curves CR vs. TARPSWG cohorts

WDLS

Table 2 shows the comparison between patients with WDLS in CR vs. TARPSWG cohorts. In terms of grade, surgical margins, receipt of adjuvant RT and 5-year LR and DM rates the cohorts were comparable. In the CR cohort, age was on average 5 years older, and there was less frequent receipt of chemotherapy (1.8% vs. 5.7%) or radiotherapy (12.7% vs. 20.7%), in particular less neoadjuvant chemotherapy (0% vs. 5.4%) and/or neoadjuvant radiotherapy (7.3% vs. 15.7%). Age- and sex-adjusted analyses on treatment showed the same direction of the association but no significance and could sometimes not be conducted due to small case numbers. The 5-year OS for patients with WDLS was lower in the CR compared to the TARPSWG cohort (71.2 vs. 89.9%, HR 2.42).

Table 2 Patient, tumour, treatment, and outcome data for WDLS, DDLS and LMS, cancer registry vs. TARPSWG cohorts., CR vs. TARPSWG cohortsDDLS

Compared to the TARPSWG cohort, patients with DDLS in the CR cohort were less often female (31.1% vs. 42.4%, adjusted OR: 0.64), were on average six years older, had less often grade 2 (34.9% vs. 60.4%, OR: 0.33) but more often grade 3 and had less often an R2 status (2.9% vs. 6.8%, OR: 0.42, Table 2). While receipt of chemo- and radiotherapy were overall comparable in both cohorts, the setting was more often post-surgery in the CR cohort (chemotherapy: 10.4% vs. 3%, OR 4.38; radiotherapy: 15.6% vs. 10.3%; OR 2.05). After adjustment for sex and age, there was a tendency to higher 5-year OS (0.88) and CS (0.86) rates for patients in the CR. Figure 3 shows the crude cumulative incidence function of LR. The incidence of LR is higher in the CR cohort after 1 year, even after adjustment for sex and age (HR 1.69), but comparable after 5-years.

Fig. 3figure 3

Crude cumulative incidence of local recurrence in patients with DDLS, CR vs. TARPS cohorts

LMS

Table 2 shows the comparison between patients with LMS in the CR vs. TARPSWG cohorts. In terms of sex, grade, surgical margins and 5-year LR rate, the cohorts were comparable. In the CR cohort, age was on average 10 years older, and patients were less likely to receive chemotherapy (12.3% vs. 28.6%) and/or radiotherapy (24.7% vs. 37.5%), particularly neoadjuvant chemotherapy (6.8% vs. 23.4%) and/or radiotherapy (9.6% vs. 27.6%), than in the TARPSWG cohort. After adjustment for sex and age, 5-year OS, CS, and cumulative incidence of local recurrences were comparable. While there was overall no significant difference in the cumulative incidence of distant metastasis within 5 years (crude: p = 0.3084, Fig. 4, adjusted HR: 0.81), five to 58 months following resection the incidence was slightly lower in the CR than the TARPSWG cohort.

Fig. 4figure 4

Crude cumulative incidence of distant metastasis in patients with LMS

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