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Background: Perivascular epithelioid cell neoplasms (PEComas) encompass a heterogeneous family of mesenchymal tumors. Previously described clinicopathologic features aimed at distinguishing benign from malignant variants but lacked prognostic value. Methods: This retrospective analysis examined clinicopathologic data from patients who had localized PEComa across French Sarcoma Network centers. The authors analyzed 12 clinicopathologic features in a Cox proportional hazard framework to derive a multivariate prognostic risk model for event‐free survival (EFS). They built the PEComa prognostic score (PEC‐PRO), in which scores ranged from 0 to 5, based on the coefficients of the multivariate model. Three groups were identified: low risk (score = 0), intermediate risk (score = 1), and high risk (score ≥ 2). Results: Analyzing 87 patients who had a median 46‐month follow‐up (interquartile range, 20–74 months), the median EFS was 96.5 months (95% confidence interval [CI], 47.1 months to not applicable), with 2‐year and 5‐year EFS rates of 64.7% and 58%, respectively. The median overall survival was unreached, with 2‐year and 5‐year overall survival rates of 82.3% and 69.3%, respectively. The simplified Folpe classification did not correlate with EFS. Multivariate analysis identified three factors affecting EFS: positive surgical margins (hazard ratio [HR], 5.17; 95% CI, 1.65–16.24; p =.008), necrosis (HR, 3.94; 95% CI, 1.16–13.43; p =.030), and male sex (HR, 3.13; 95% CI, 1.19–8.27; p = 0.023). Four variables were retained in the prognostic model. Patients with low‐risk PEC‐PRO scores had a 2‐year EFS rate of 93.7% (95% CI, 83.8%–100.0%), those with intermediate‐risk PEC‐PRO scores had a 2‐year EFS rate of 67.4% (95% CI, 53.9%–80.9%), and those with high‐risk PEC‐PRO scores had a 2‐year EFS rate of 2.3% (95% CI, 0.0%–18.3%). Conclusions: The PEC‐PRO score reliably predicts the risk of postoperative recurrence in patients with localized PEComa. It has the potential to improve follow‐up strategies but requires validation in a prospective trial. By using a weighted combination of clinicopathologic features, the perivascular epithelioid cell neoplasm prognostic (PEC‐PRO) score identified three different prognostic groups, including a low risk (score = 0), an intermediate risk (score = 1), and a high risk (score ≥2) of recurrence. The PEC‐PRO score reliably predicts the recurrence risk in patients with localized perivascular epithelioid cell neoplasms and has the potential to improve follow‐up strategies and personalize adjuvant treatments. [ABSTRACT FROM AUTHOR]

Background: CIC‐rearranged sarcomas (CIC‐RS) represent the most frequent subset of "Ewing‐like" undifferentiated small round cell sarcomas. These tumors tend to be more aggressive than Ewing sarcomas. Moreover, treatment strategy can differ according to teams. The primary aim of this retrospective study was to describe the characteristics, treatments, and outcome for patients with CIC‐RS included in the French NETSARC+ database. Methods: Pediatric and adult patients from 13 French centers with a diagnosis of CIC‐RS were registered from October 2008 to March 2021. Patients and tumors characteristics were collected from the national network NETSARC+ database (http://netsarc.sarcomabcb.org). CIC‐RS diagnosis was pathologically and molecularly confirmed with a central review by expert pathologists. Two groups of patients were studied: those treated as classical Ewing sarcomas (cohort EwS) and those treated as high‐grade soft tissue sarcomas (cohort STS) according to ESMO and/or EpSSG guidelines. Survival was calculated using the Kaplan–Meier method and the log‐rank test was used to compare survival. Results: Among 79 patients, the male/female sex ratio was 0.7 and the median age at diagnosis was 27 years (range 2–87). With a median follow‐up of 37 months, 39 patients died of the disease. Median overall survival from diagnosis was 18 months, with no significant difference between both cohorts (p = 0.9). Nevertheless, when focusing on patients with metastatic disease at diagnosis (N = 21), all patients from cohort STS died of disease while some patients from cohort EwS were still alive and in complete remission. Conclusion: FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen. [ABSTRACT FROM AUTHOR]

Background: The incidence of different soft tissue sarcoma (STS) histotypes among ethnic and geographic populations has not been comprehensively investigated.Methods: Data from 2013 to 2016 were obtained from national cancer registry databases in France and Taiwan. Liposarcoma (LPS), leiomyosarcoma (LMS), angiosarcoma (AS), synovial sarcoma (SS), and malignant peripheral nerve sheath tumor (MPNST) were selected as index STSs to estimate the age-standardized incidence rates (ASRs) and other clinical features between patients.Results: In total, 9398 patients (7148 from France and 2250 from Taiwan) were included. The ASRs of AS (5.4 vs. 2.8) and MPNST (2.0 vs. 1.0) were significantly higher in Taiwan; France had significantly higher ASRs for LPS (12.0 vs. 10.0), LMS (9.7 vs. 7.6), and SS (1.7 vs. 1.2). Patients in Taiwan with LMS or LPS were younger than their French counterparts. With regard to the distribution according to primary anatomic site, French patients had higher odds for extremity and truncal LMS (odds ratio [OR], 2.84; p < .001), AS (OR, 2.67; p < .001), MPNST (OR, 1.55; p = .027), and LPS (OR, 1.38; p < .001) and for breast AS (OR, 10.58; p < .001). Taiwanese patients had higher odds for liver AS (OR, 10.72; p < .001) and uterine LMS (OR, 3.21; p < .001). SS age and distribution according to primary anatomic site did not differ significantly between the French and Taiwanese populations.Conclusions: Significant differences in the incidence and clinical characteristics of index STS suggested that geographic (environmental) and ethnicity factors likely play a vital role in the pathogenesis of STS. [ABSTRACT FROM AUTHOR]

The benefit of chemotherapy (CT) in rare bone sarcomas is not documented in prospective studies. Our retrospective study from the French sarcoma network for bone tumors ResOs was performed in adult patients (pts) from 1976 to 2014, with histologically verified diagnosis of leiomyosarcomas (LMS), undifferentiated pleomorphic sarcoma (UPS) or radiation‐associated sarcomas of bone. The median follow‐up was 4.7 years (95% CI: 3.7‐6.5). Clinical features, treatment modalities and outcomes were recorded and analyzed from 145 pts (median age 53 years [range 20‐87]). Site of disease was extremities (66%) or axial skeleton (34%), 111 (77%) presented with localized and potentially resectable disease. The most common histological subtypes were UPS (58%) and LMS (33%); 58% were high‐grade tumors. Surgery was performed in 127 pts. In the 111 localized pts, 28 pts (25%) underwent upfront surgery or exclusive radiotherapy (RT; >50 Gy) without CT, whereas 83 pts (75%) received either neoadjuvant (n = 26) or adjuvant CT (n = 13) or both (n = 44). Neoadjuvant and adjuvant CT was mostly doxorubicin‐based (95%/86%) and cisplatin‐based (67%/63%). R0 resection was achieved in 59 pts, and a good histological response in 15 patients (25%). Adjuvant RT was performed in 24 (22%) pts. For the whole cohort (n = 145), the 5‐year overall survival (OS) rate was 53% [42; 62]. In univariate analysis, age ≤ 60 was associated with a longer disease‐free survival (DFS) (P =.0436). Neoadjuvant and adjuvant CT tended to be associated with better DFS (P =.056) with no significant impact on OS in this retrospective series. What's new? Rarer types of primary bone sarcoma (non‐osteosarcoma, non‐chondrosarcoma, and non‐Ewing sarcoma) present a major diagnostic challenge, and are often excluded from clinical trials. In this study, the authors found that patients who had localized disease and were treated with both neo‐adjuvant and adjuvant chemotherapy tended to have better disease‐free survival (DFS). However, there was no significant impact on overall survival (OS). The role of adjuvant chemotherapy and radiation therapy remains unclear, which highlights the need for prospective randomised trials in these uncommon bone malignancies. [ABSTRACT FROM AUTHOR]

Treatment options for metastatic osteosarcomas are scarce. Following failure of standard first line therapy, patients who relapse present a challenging treatment dilemma, and have a poor prognosis. Surgical removal of all metastases is essential. A retrospective analysis of patients with metastatic osteosarcomas was conducted in 15 French Sarcoma Group centers. From January 2009 to December 2018, we identified 120 adult patients; 36 with synchronous and 84 with metachronous metastases with 74 males and 46 females. Mean age was 30 years (18‐53). Metastatic sites were lung, bone and other in 91, 11 and 24 patients, respectively. Mean time to first metachronous metastases was 22 months (4‐97). All patients except 13 (10.8%) with metachronous metastases received a first line systemic treatment for relapse, and 39 patients (32.5%) were included in a clinical trial. Eighty‐one patients (67.5%) had local treatment of distant metastases. Median progression free survival (PFS) and overall survival (OS) were 5.5 (95% CI 4.6‐6.4) and 20.5 months (95% CI 13.2‐27.7) respectively for the overall group. In multivariate analysis, more than five metastases, time to first metastases <24 months, were statistically significant negative prognostic factors for OS and PFS (P =.002, ≤.001 and P =.006, ≤.001, respectively). Surgery of metastases was associated with better prognosis on OS and PFS (P =.001 and.037, respectively). The presence of bone metastases was a negative prognostic factor on OS but not on PFS (P =.021). In reference sarcoma centers, relapsed osteosarcoma patients with more than one metastasis commonly receive more than one line of systemic therapy, and are included in clinical trial if available. What's new? About a third of osteosarcoma patients will develop metastases, usually with a poor prognosis. In this French study of adult osteosarcoma patients, the authors compared different treatment modalities, both systemic and localized. They also sought to define prognostic factors, both for patients who developed metastases after their initial treatment, and also for those with metastatic disease at the time of diagnosis. They found no significant difference in overall survival between these two groups. They also conclude that radical focal treatment of metastases is the only modality that allows long‐lasting survival. [ABSTRACT FROM AUTHOR]

Soft tissue sarcomas (STS) are rare tumors accounting for less than 1% of human cancers. While the highest incidence of sarcomas is observed in elderly, this population is often excluded or poorly represented in clinical trials. The present study reports on clinicopathological presentation, and outcome of sarcoma patients over 90 recorded in the Netsarc.org French national database. NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor board (MDTB), funded by the French National Cancer Institute to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB, second pathological review, and collection of sarcoma patient characteristics and follow‐up are collected in a database Information of patients registered from January 1, 2010, to December 31, 2016, in NETSARC were collected, analyzed and compared to the younger population. Patients with sarcomas aged >90 have almost exclusively sarcomas with complex genomics (92.0% vs. 66.3%), are less frequently metastatic (5.3% vs. 14·7%) at diagnosis, have more often superficial tumors (39.8% vs. 14.7%), as well as limbs and head and neck sites (75.2% vs. 38.7%) (all p < 0.001). Optimal diagnostic procedures and surgery were less frequently performed in patients over 90 (p < 0.001). These patients were less frequently operated in NETSARC centers, as compared to those of younger age groups including aged 80–90. However, local relapse‐free survival, metastatic relapse‐free survival and relapse‐free survival were not significantly different from those of younger patients, in the whole cohort, as well as in the subgroup of operated patients. As expected overall survival was worse in patients over 90 (p < 0.001). Patients over 90 who were not operated had worse overall survival than younger patients (9.9 vs. 27.3 months, p < 0.001). Patients with STS diagnosed after 90 have distinct clinicopathological features, but comparable relapse‐free survival, unless clinical practice guidelines recommendations are not applied. Standard management should be proposed to these patients if oncogeriatric status allows. What's new? While the highest incidence of soft‐tissue sarcoma (STS) is observed in the elderly, this population is often excluded or poorly represented in clinical trials. Therefore, little is known about the characteristics, treatment, and outcomes of STS in these patients. In this study, the authors analyzed numerous clinical characteristics of patients with sarcoma diagnosed at age 91 or older. They conclude that standard STS management and clinical practice guidelines should be followed for these patients if possible. [ABSTRACT FROM AUTHOR]

Breast cancer (BC) is the second most common cause of brain metastases (BM). Predictive factors for BM have been widely studied in metastatic BC; however, there is no known serum tumor marker to accurately predict BM. Elevated serum S100ß protein and neuron-specific enolase (NSE) could reflect the brain damages induced by BM. Matrix metalloproteinase 9 (MMP-9) is involved in tumor invasion and metastatic dissemination, including BM. Also, HER2-amplified BC were shown to have a particular tropism for central nervous system (CNS). This study evaluated the correlation of these biomarkers with the presence of BM in metastatic BC patients. In this case-control study, 88 consecutive metastatic BC patients with BM (BM group) treated in our institution (2008-2015) were retrospectively selected, based on the availability of frozen serum samples for tumor marker determination. Patients were matched by age, tumor biology and number of previous metastatic chemotherapy lines to 162 metastatic BC patients without CNS involvement (control group). Serum NSE, MMP-9 and HER2 extracellular domain (ECD) levels were significantly higher in the BM group ( p = 0.0051, p = 0.0062 and p = 0.0057, respectively). In multivariate analysis, serum HER2 and MMP-9 levels accurately discriminated patients with BM: odds ratios 4.4 ( p < 0.001; 95%CI: 1.9-9.6) for HER2 ECD and 3.5 ( p = 0.005; 95%CI: 1.5-8.4) for MMP-9. In multivariate analysis, HER2 ECD and NSE serum levels were independent prognostic factors in patients with BM. Serum HER2 ECD and MMP-9 appear to be associated with BM in metastatic BC patients. Their predictive value for BM still needs to be evaluated in further prospective studies. [ABSTRACT FROM AUTHOR]