Department of Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy.
Department of Pathology, Padua University Hospital, Padua, Italy.
Eur J Surg Oncol. 2020 Jul;46(7):1320-1326. doi: 10.1016/j.ejso.2020.03.215. Epub 2020 Apr 8.
This case-series is aimed to describe the natural history of epithelioid sarcoma (ES) and to provide insights into the differential clinical behaviour of its two variants ("classic-type" and "proximal-type"). The value of a subtype-adapted grading system based on pathological features is explored.
Data from consecutive, primary, localised, INI1-deleted ES operated at three Italian sarcoma reference centres (1995-2015) were included. Centralised pathological review was performed. Classic-type ES was broken down into "high-grade" and "low-grade", according to number of mitoses, evidence of necrosis and nuclear atypia. Five- and 10-year overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were estimated.
Fifty-two patients were included. 5- and 10-year OS estimates were 70% and 47% in the whole series, 57% and 37% in patients with proximal-type ES, 77% and 54% in patients with classic-type ES (P = 0.02). In classic-type ES, 5- and 10-year OS was higher for low-grade (95% and 72%, respectively) than high-grade tumours (P = 0.002). 5- and 10-year CCI estimates for LR were 21% and 33% in the whole series. 5- and 10-year CCI estimates for DM were 35% and 39% in the whole series, both 28% in classic-type ES, 47% and 59% in proximal-type ES (P = 0.03).
Suffering from a proximal- or a classic-type is the stronger predictor of outcome in patients with localised ES, with proximal-type ES patients having lower survival due to a higher tendency toward metastatic spreading. However, the "high-grade" classic-type ES was associated with outcomes close to proximal-type ES.
本病例系列旨在描述上皮样肉瘤（ES）的自然病史，并深入了解其两种变体（“经典型”和“近端型”）的不同临床行为。还探索了基于病理特征的亚型适应性分级系统的价值。
纳入了三家意大利肉瘤参考中心（1995-2015 年）连续收治的局部初发 INI1 缺失的原发性上皮样肉瘤患者的数据。进行了集中的病理审查。经典型 ES 根据有丝分裂数量、坏死证据和核异型性被细分为“高级别”和“低级别”。评估了整体生存率（OS）和局部复发（LR）及远处转移（DM）的粗累积发生率（CCI）的 5 年和 10 年估计值。
共纳入 52 例患者。全组 5 年和 10 年 OS 估计值分别为 70%和 47%，近端型 ES 患者为 57%和 37%，经典型 ES 患者为 77%和 54%（P=0.02）。在经典型 ES 中，低级别肿瘤的 5 年和 10 年 OS 分别为 95%和 72%，明显高于高级别肿瘤（P=0.002）。全组 LR 的 5 年和 10 年 CCI 估计值分别为 21%和 33%。全组 DM 的 5 年和 10 年 CCI 估计值分别为 35%和 39%，经典型 ES 患者分别为 28%，近端型 ES 患者分别为 47%和 59%（P=0.03）。
患有近端型或经典型 ES 的患者的预后更受局部 ES 影响，由于转移扩散的趋势更高，近端型 ES 患者的生存率更低。然而，“高级别”经典型 ES 的结局接近近端型 ES。
