PEComas is a family of rare mesenchymal tumors. This systematic review aims to better understand the natural history of advanced PEComas. After a search on the PubMed database and main oncology meeting libraries according to the PRISMA guidelines, 88 articles reported in the English literature were included. Data on clinical and histological features, treatments and outcomes were collected. To identify risk factors, univariate and multivariate analyses were performed. Seven cohorts of patients and 124 individual patients were identified. Focusing on case reports, most patients were metastatic, and the median overall survival (OS) of the entire cohort was 60 months (95%CI 33; NA). Risk factors significantly associated with OS in the multivariate analysis were the presence of metastasis at diagnosis (HR: 2.59, 95%CI 1.06;6.33, p = 0.036) and the grouped-Bleeker’s risk category (HR: 4.66; 95%CI 1.07;20.19; p = 0.039). In the metastatic population, only the presence of lymph node metastasis was associated with OS (HR: 3.11; 95%CI 1.13;8.60, p < 0.05). Due to a lack of events, it was not possible to conclude on other factors. This review of the literature highlights the heterogeneity of literature data and shows the great diversity of clinical management strategies.
Gastric cancer is one of the most aggressive cancers, with a median survival of 12 months. This illustrates its complexity and the lack of therapeutic options, such as personalized therapy, because predictive markers do not exist. Thus, gastric cancer remains mostly treated with cytotoxic chemotherapies. In addition, less than 20% of patients respond to immunotherapy. TP53 mutations are particularly frequent in gastric cancer (±50% and up to 70% in metastatic) and are considered an early event in the tumorigenic process. Alterations in the expression of other members of the p53 family, i.e., p63 and p73, have also been described. In this context, the role of the members of the p53 family and their isoforms have been investigated over the years, resulting in conflicting data. For instance, whether mutations of TP53 or the dysregulation of its homologs may represent biomarkers for aggressivity or response to therapy still remains a matter of debate. This uncertainty illustrates the lack of information on the molecular pathways involving the p53 family in gastric cancer. In this review, we summarize and discuss the most relevant molecular and clinical data on the role of the p53 family in gastric cancer and enumerate potential therapeutic innovative strategies.
11571 Background: Perivascular Epithelioid Cell Neoplasms (PEComas) encompass a heterogeneous family of mesenchymal tumors. The current understanding of their natural history is limited. Previously described clinicopathological factors aimed to define benign or malignant variants, but there is a lack of prognostic factors associated with recurrence of surgically resected tumors, preventing the development of a prognostic score to better optimize patient’s management. Methods: This is a retrospective analysis of clinicopathological features from patients diagnosed with a localized PEComa, within all centers from the French Sarcoma network and one center in Belgium. We analyzed 12 clinicopathological factors in a Cox proportional hazard framework to derive a multivariate prognostic risk model for progression-free survival (PFS). We built the PEComa PROgnostic score (PEC-PRO) ranging from 0 to 5, based on the coefficients of the multivariate model. Three different prognostic groups were identified: low risk (score = 0), intermediate risk (score = 1) and high risk (score ≥2). Results: Ninety-three patients were analyzed with a median follow-up of 46 months (range, 3-253). At diagnosis, the median age was 54 years (range, 13-84), with female predominance (72%). Most common primary locations were uterus (n = 15;16%) and kidney (n = 15;16%). Median tumor size was 6.2 cm (range, 0.8-30). Among patients with reported surgical margins, 64 (73%) and 23 (27%) had R0 and R1-2 margins, respectively. The median PFS was 26 months (IC95, 2.9-124.4), with 1- and 5-year overall survival (OS) rates of 95.7% and 69.9%, respectively, while the median OS was not reached. Using univariate analyses, male gender, primary tumor size > 5 cm, high nuclear grade and cellularity, high mitotic rate > 1/50 HPF, necrosis, vascular invasion, nodal invasion, and R1-2 margins were associated with a shorter PFS. Among those, male gender (HR = 2.88; IC95 1.12-7.411, p = 0.03), vascular invasion (HR = 3.14; IC95 1.10-8.96, p = 0.034), necrosis (HR = 3.93; IC95 1.35-11.47, p = 0.015), and R1-2 margins (HR 4.47; IC95 1.60-12.46, p = 0.007) remained associated with PFS in the multivariate analysis and were included in the multivariate model. Median PFS in patients with high PEC-PRO score was 16 months as compared to 104 months and not reached for patients with intermediate and low PEC-PRO scores, respectively (p < 0.001). We also confirmed the prognostic relevance of the PEC-PRO score in terms of OS. Conclusions: Using a weighted combination of clinicopathological features, the PEC-PRO score reliably predicts the post-operative recurrence risk in patients with localized PEComas. It has the potential to better improve follow-up strategies and personalize adjuvant treatments. The findings of this retrospective analysis require validation in a prospective trial.
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