Department of Pathology and Medical Research Center, Beijing Institute of Respiratory Medicine and Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China; Department of Pathology, Beijing Shijitan Hospital, Capital Medical University, Beijing, 100038, China.
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100069, China.
Ann Diagn Pathol. 2022 Oct;60:151981. doi: 10.1016/j.anndiagpath.2022.151981. Epub 2022 May 28.
This study aimed to evaluate immunohistochemical markers of pheochromocytoma/paraganglioma (PPGL) and the relationships between the grading system for adrenal pheochromocytoma and paraganglioma (GAPP) and the prognosis of PPGL in a Chinese population. A retrospective analysis was conducted on a cohort of 102 PPGL cases, from January 2012 to December 2019, with complete clinicopathological and follow-up data. Surgical pathology slides were re-reviewed. All histological parameters involved in GAPP were summarized. The relationship between clinical characteristics, expression of SDHB (succinate dehydrogenase), S-100 and Ki-67 as well as GAPP classifications and prognosis of PPGL was statistically analyzed. The 102 cases included 51 males (50%) and 51 females (50%) with a median age of 48.7 years. The median tumor size was 6.8 cm. Metastases or relapse developed in 23 (22.5%) cases. Larger tumor size, extra-adrenal location, and poorly differentiated PPGL according to GAPP were associated with metastases or relapse (P < 0.05). Histological parameters, including the appearance of large or fused cell nests, necrosis, vascular invasion, and capsular invasion, were more common in the cases with metastases or relapse (P < 0.01). Loss of SDHB or S-100 expression was more common in poorly differentiated PPGL and associated with metastases or relapse (P < 0.01). However, no significant difference in the Ki-67 index between the clinically malignant and benign group was observed. GAPP is thus helpful for evaluations of the biological behavior of PPGL.
这项研究旨在评估嗜铬细胞瘤/副神经节瘤（PPGL）的免疫组织化学标志物，以及肾上腺嗜铬细胞瘤和副神经节瘤分级系统（GAPP）与中国人群 PPGL 预后之间的关系。对 2012 年 1 月至 2019 年 12 月期间具有完整临床病理和随访资料的 102 例 PPGL 病例进行了回顾性分析。重新审查了手术病理切片。总结了 GAPP 中涉及的所有组织学参数。对临床特征、SDHB（琥珀酸脱氢酶）、S-100 和 Ki-67 的表达以及 GAPP 分类与 PPGL 预后之间的关系进行了统计学分析。102 例患者中，男性 51 例（50%），女性 51 例（50%），中位年龄为 48.7 岁。肿瘤大小中位数为 6.8cm。23 例（22.5%）发生转移或复发。根据 GAPP，肿瘤较大、肾上腺外位置和分化不良的 PPGL 与转移或复发相关（P<0.05）。在发生转移或复发的病例中，更常见大细胞巢或融合细胞巢、坏死、血管侵犯和包膜侵犯等组织学参数（P<0.01）。SDHB 或 S-100 表达缺失在分化不良的 PPGL 中更为常见，与转移或复发相关（P<0.01）。然而，Ki-67 指数在临床上恶性和良性组之间无显著差异。因此，GAPP 有助于评估 PPGL 的生物学行为。
