Department of Pathology and Medical Research Center, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.
Department of Pathology, The Fourth Affiliated Hospital of Shihezi University, Akesu, China.
J Surg Oncol. 2024 Jul;130(1):47-55. doi: 10.1002/jso.27681. Epub 2024 Jun 12.
The aim of this study is to explore the long-term prognostic risk factors associated with patients diagnosed with retroperitoneal paraganglioma (RPGL) and examine their clinical and pathological characteristics.
Expressions of biomarkers were identified using immunohistochemistry (IHC) and case databases were retrospectively searched. Survival analysis was performed using Kaplan-Meier and Cox risk regression to identify the factors that influence the postoperative progression-free survival of patients with RPGL.
A total of 105 patients, most of whom had tumors situated in the paraaortic region, and whose average tumor size was 8.6 cm, were enrolled in this study. The average follow-up duration was 51 months, with a mortality rate of 19% and a recurrence and metastasis rate of 41.9%. Tumors were assessed using the modified Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP), and SDHB, S-100, and Ki-67 were stained using IHC in all cases. Out of the total cases examined, negative in SDHB expression were observed in 18.1% of cases, S-100 expression was negative in 36.2% of cases, and endovascular tumor enboluswas present in approximately 25.7% of cases. The results of the univariate analysis indicated that several factors significantly influenced the progression-free survival of patients with PGL as follow: maximum tumor diameter (>5.5 cm), tumor morphological features, tumor grading (modified GAPP score > 6), SDHB negative, S-100 negative, and expression of proliferation index Ki-67 (>3%) (X = 4.217-27.420, p < 0.05). The results of the multivariate analysis indicated that negative of S-100 (p = 0.021) and SDHB (p = 0.038), as well as intravascular tumor thrombus (p = 0.047) expression were independent risk factors for progression-free survival in patients.
RPGL is characterized by diverse biological features and an elevated susceptibility to both recurrence and metastasis. Both SDHB and S-100 can be employed as traditional IHC indicators to predict the metastatic risk of PGL, whereas the tumor histomorphology-endovascular tumor enbolus assists in determining the metastasis risk of RPGL.
本研究旨在探讨与腹膜后副神经节瘤（RPGL）患者相关的长期预后危险因素，并分析其临床病理特征。
采用免疫组织化学（IHC）方法鉴定生物标志物的表达，并对病例数据库进行回顾性检索。采用 Kaplan-Meier 和 Cox 风险回归分析对影响 RPGL 患者术后无进展生存率的因素进行生存分析。
共纳入 105 例患者，肿瘤多位于腹主动脉旁，平均肿瘤大小为 8.6cm。平均随访时间为 51 个月，死亡率为 19%，复发转移率为 41.9%。采用改良的肾上腺嗜铬细胞瘤和副神经节瘤分级系统（GAPP）对肿瘤进行评估，所有病例均采用 IHC 染色检测 SDHB、S-100 和 Ki-67。在所有检查的病例中，SDHB 表达阴性者占 18.1%，S-100 表达阴性者占 36.2%，血管内肿瘤栓子者占 25.7%左右。单因素分析结果表明，以下因素显著影响 PGL 患者的无进展生存率：最大肿瘤直径（>5.5cm）、肿瘤形态学特征、肿瘤分级（改良 GAPP 评分>6）、SDHB 阴性、S-100 阴性、增殖指数 Ki-67 表达（>3%）（X=4.217-27.420，p<0.05）。多因素分析结果表明，S-100 阴性（p=0.021）和 SDHB 阴性（p=0.038）以及血管内肿瘤栓子（p=0.047）是影响患者无进展生存率的独立危险因素。
RPGL 具有多种生物学特征，易复发和转移。SDHB 和 S-100 均可作为传统的 IHC 指标预测 PGL 的转移风险，而肿瘤组织形态-血管内肿瘤栓子有助于确定 RPGL 的转移风险。
