Wang Ling-Ling, Wei Xue-Jing, Zhang Qiao-Chu, Li Feng, Chen Guang-Yong
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
Department of Pathology, Shanghai Pudong Gongli Hospital, China.
Ann Diagn Pathol. 2025 Dec;79:152525. doi: 10.1016/j.anndiagpath.2025.152525. Epub 2025 Jul 8.
This study aimed to analyze the expression of three immune markers, succinate dehydrogenase (SDHB), S-100, and alpha thalassemia retardation syndrome X-linked (ATRX), in pheochromocytoma and paraganglioma (PPGL) tumor tissues and to evaluate their correlation with histopathological parameters to predict the recurrence risk in PPGLs. A retrospective analysis was conducted using a cohort of 173 patients with PPGLs with definite pathological diagnoses and complete follow-up data. The expression of SDHB, S-100, and ATRX in the tumor tissues was detected using the EnVision immunohistochemical method. Histological grading of PPGLs was performed using the Compound Adrenal Pheochromocytoma and Paraganglioma Grading System (COPPS), and the correlation between the expression of the three immune markers and metastasis or recurrence of PPGLs was analyzed. Among 173 patients with PPGLs, 57 (32.9 %) had metastasis or recurrence. Of these 57 patients, 57.9 % (33/57) had tumors in the retroperitoneum. In 83 % (53/57) of the cases, the maximum diameter of the tumors was ≥5 cm. Vascular invasion was observed in 46 patients (80.7 %). SDHB, ATRX, and S-100 were negatively stained in 33 (57.9 %), 40(70.2 %), and 43(75.4 %) PPGL patients with metastasis or recurrence, respectively, which was significantly higher than that in those without metastasis or recurrence. These differences were statistically significant (P < 0.001). Multivariate analysis showed that tumor diameter ≥ 5 cm, negative ATRX and SDHB expressions, and vascular invasion were independent risk factors for tumor metastasis and recurrence. SDHB, ATRX and S-100 can be used as immunohistochemical indicators to predict the metastatic risk of PPGLs.
本研究旨在分析三种免疫标志物，即琥珀酸脱氢酶（SDHB）、S-100和X连锁α地中海贫血迟缓综合征（ATRX），在嗜铬细胞瘤和副神经节瘤（PPGL）肿瘤组织中的表达，并评估它们与组织病理学参数的相关性，以预测PPGL的复发风险。对173例经明确病理诊断且有完整随访数据的PPGL患者进行了回顾性分析。采用EnVision免疫组化方法检测肿瘤组织中SDHB、S-100和ATRX的表达。使用复合肾上腺嗜铬细胞瘤和副神经节瘤分级系统（COPPS）对PPGL进行组织学分级，并分析三种免疫标志物的表达与PPGL转移或复发之间的相关性。在173例PPGL患者中，57例（32.9%）发生了转移或复发。在这57例患者中，57.9%（33/57）的肿瘤位于腹膜后。在83%（53/57）的病例中，肿瘤最大直径≥5 cm。46例患者（80.7%）观察到血管侵犯。发生转移或复发的PPGL患者中，SDHB、ATRX和S-100分别有33例（57.9%）、40例（70.2%）和43例（75.4%）呈阴性染色，显著高于未发生转移或复发的患者。这些差异具有统计学意义（P<0.001）。多因素分析显示，肿瘤直径≥5 cm、ATRX和SDHB表达阴性以及血管侵犯是肿瘤转移和复发的独立危险因素。SDHB、ATRX和S-100可作为免疫组化指标来预测PPGL的转移风险。
