Pathology Unit Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, via A. Gabelli 61, 35121, Padova, Italy.
Department of Interdisciplinary Medicine, Occupational Health Division, University of Bari, 1 Umberto I Sq., 70121, Bari, Italy.
Diagn Pathol. 2021 Jul 22;16(1):64. doi: 10.1186/s13000-021-01125-z.
Diffuse malignant peritoneal mesothelioma (DMPM) is a rare malignant neoplasm with poor survival that shares some similarities with the best-known pleural variant, pleural mesothelioma. The recent European Reference Network on Rare Adult Cancers (EURACAN)/International Association for the Study of Lung Cancer (IASLC) proposals attempted to improve the histological diagnosis and patient risk stratification. Herein, we investigated whether the pathology recommendations and suggestions of the pleural proposals were applicable to diffuse malignant peritoneal mesothelioma.
Fifty multiple laparoscopic biopsies of DMPM were consecutively collected at the Pathology Unit of the University of Bari. A two-tier system, i.e., low, and high grade, was used to categorize 34 epithelioid DMPMs. Architectural patterns, cytological features and stromal changes were also reported. Immunohistochemistry was performed for BRCA1-associated protein 1 (BAP1), programmed death-ligand 1 (PD-L1), and Ki67, while fluorescence in situ hybridization (FISH) was performed for p16/cyclin-dependent kinase inhibitor 2A (CDKN2A).
High-grade epithelioid mesothelioma, high Ki67, and p16/CDKN2A deletion were significantly associated with short survival (p = 0.004, p < 0.0001, and p = 0.002, respectively). BAP1 loss and PD-L1 negativity were the most common findings. Multivariate analysis revealed that the nuclear grading system and p16 deletion significantly correlated with survival (p = 0.003 each).
The present study examined the prognostic significance of several factors proposed for pleural mesothelioma in an extra pleural site. Notably, the introduction of a grading system may provide better risk stratification in epithelioid DMPM. Ki67, BAP1 and p16/CDKN2A should also be measured whenever possible. A detailed report with all supportive data would allow us to collect sufficient information for use in further studies on larger case series.
弥漫性恶性腹膜间皮瘤(DMPM)是一种罕见的恶性肿瘤,生存预后较差,与更为人熟知的胸膜变体胸膜间皮瘤有一些相似之处。最近的欧洲罕见成人癌症参考网络(EURACAN)/国际肺癌研究协会(IASLC)提议试图改善组织学诊断和患者风险分层。在此,我们研究了胸膜提议的病理学建议和意见是否适用于弥漫性恶性腹膜间皮瘤。
在巴里大学病理学系连续收集了 50 例 DMPM 的腹腔镜多部位活检。使用低级别和高级别两级系统对 34 例上皮样 DMPM 进行分类。还报告了结构模式、细胞学特征和基质变化。进行了 BRCA1 相关蛋白 1(BAP1)、程序性死亡配体 1(PD-L1)和 Ki67 的免疫组织化学检查,同时进行了荧光原位杂交(FISH)以检测 p16/细胞周期蛋白依赖性激酶抑制剂 2A(CDKN2A)。
高级别上皮样间皮瘤、高 Ki67 和 p16/CDKN2A 缺失与短生存期显著相关(p=0.004、p<0.0001 和 p=0.002)。BAP1 缺失和 PD-L1 阴性是最常见的发现。多变量分析显示核分级系统和 p16 缺失与生存显著相关(p=0.003 各)。
本研究在胸膜外部位检查了几个胸膜间皮瘤提议的预后因素的意义。值得注意的是,引入分级系统可能会为上皮样 DMPM 提供更好的风险分层。如果可能,还应测量 Ki67、BAP1 和 p16/CDKN2A。详细报告所有支持数据将使我们能够收集足够的信息,用于进一步研究更大的病例系列。