Rebrova D V, Loginova O I, Vorobyev S L, Vorokhobina N V, Kozorezova E S, Indeykin F A, Savelyeva T V, Sleptsov I V, Chernikov R A, Fedorov E A, Semenov A A, Chinchuk I K, Shikhmagomedov Sh Sh, Alekseev M A, Krasnov L M, Rusakov V F
Saint Petersburg State University, Saint Petersburg State University Hospital.
National Clinical Center of Morphological Diagnostic.
Probl Endokrinol (Mosk). 2023 Oct 4;70(2):37-45. doi: 10.14341/probl13331.
Currently, all pheochromocytoma/paraganglioma (PPGLs) are considered malignant due to metastatic potential. Consequently, PPGLs are divided into «metastatic» and «non-metastatic». Metastatic PPGLs can be with synchronous metastasis (metastases appear simultaneously with the identified primary tumor) or metachronous (metastases develop after removal of the primary tumor). The term metastatic PPGLs is not used in the presence of tumor invasion into surrounding organs and tissues, without the presence of distant metastases of lymphogenic or hematogenic origin. It is generally believed that about 10% of pheochromocytomas and about 40% of sympathetic paragangliomas have metastatic potential. On average, the prevalence of PPGLs with the presence of metastases is 15-20%. Risk factors for metastatic PPGLs are widely discussed in the literature, the most significant of which are groups of clinical, morphological and genetic characteristics. The review presents a discussion of such risk factors for metastatic PPGLs as age, localization and type of hormonal secretion of the tumor, the size and growth pattern of the adrenal lesion, the presence of necrosis and invasion into the vessels, the tumor capsule surrounding adipose tissue, high cellular and mitotic activity, Ki-67 index, expression of chromogranin B and S100 protein, the presence of genetic mutations of three main clusters (pseudohypoxia, kinase signaling and Wnt signaling).Over the past two decades, a number of authors have proposed various predictor factors and scales for assessing a probability of metastatic PPGLs. The review contains detailed description and comparison of sensitivity and specificity of such predictor scales as PASS, GAPP, M-GAPP, ASES and COPPS.
目前，由于具有转移潜能，所有嗜铬细胞瘤/副神经节瘤（PPGLs）均被视为恶性肿瘤。因此，PPGLs被分为“转移性”和“非转移性”。转移性PPGLs可出现同步转移（转移灶与已确诊的原发肿瘤同时出现）或异时转移（转移灶在原发肿瘤切除后出现）。当肿瘤侵犯周围器官和组织但不存在淋巴源性或血源性远处转移时，不使用“转移性PPGLs”这一术语。一般认为，约10%的嗜铬细胞瘤和约40%的交感神经副神经节瘤具有转移潜能。平均而言，存在转移的PPGLs患病率为15% - 20%。转移性PPGLs的危险因素在文献中已有广泛讨论，其中最重要的是临床、形态学和遗传学特征组。本文综述讨论了转移性PPGLs的此类危险因素，如年龄、肿瘤的定位和激素分泌类型、肾上腺病变的大小和生长模式、坏死和血管侵犯的存在、肿瘤包膜周围脂肪组织、高细胞活性和有丝分裂活性、Ki-67指数、嗜铬粒蛋白B和S100蛋白的表达、三个主要基因簇（假性低氧、激酶信号传导和Wnt信号传导）的基因突变的存在。在过去二十年中，许多作者提出了各种预测因素和量表来评估转移性PPGLs的可能性。本文综述详细描述并比较了PASS、GAPP、M-GAPP、ASES和COPPS等预测量表的敏感性和特异性。
