Analysis of clinicopathological and immunohistochemical features of pheochromocytoma/paraganglioma

Ling-Ling Wang; Xue-Jing Wei; Qiao-Chu Zhang; Feng Li; Guang-Yong Chen

doi:10.1016/j.anndiagpath.2025.152525

Analysis of clinicopathological and immunohistochemical features of pheochromocytoma/paraganglioma

Ann Diagn Pathol. 2025 Jul 8:79:152525. doi: 10.1016/j.anndiagpath.2025.152525. Online ahead of print.

Authors

Ling-Ling Wang¹, Xue-Jing Wei², Qiao-Chu Zhang³, Feng Li⁴, Guang-Yong Chen⁵

Affiliations

¹ Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China. Electronic address: sun2000_2005@sina.com.
² Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China. Electronic address: weixuejing@ccmu.edu.cn.
³ Department of Pathology, Shanghai Pudong Gongli Hospital, China.
⁴ Department of Pathology and Medical Research Center, Beijing Institute of Respiratory Medicine and Beijing Chaoyang Hospital, Capital Medical University, Beijing 100020, China. Electronic address: lifeng7855@126.com.
⁵ Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China. Electronic address: chenguangyong@ccmu.cn.

PMID: 40652840
DOI: 10.1016/j.anndiagpath.2025.152525

Abstract

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.

Keywords: Alpha thalassemia retardation syndrome X-linked; COPPS; Metastasis and recurrence; Paraganglioma; S-100; Succinate dehydrogenase.