预测嗜铬细胞瘤和副神经节瘤的转移潜能：PASS 和 GAPP 评分系统的比较。
Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.
机构信息
Hospital of the University of Pennsylvania, Department of Surgery, Division of Endocrine and Oncologic, Surgery, Philadelphia, Pennsylvania.
The Abramson Cancer Center, Philadelphia, Pennsylvania.
出版信息
J Clin Endocrinol Metab. 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608.
PURPOSE
The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP) are scoring systems to predict metastatic potential in pheochromocytomas (PCC) and paragangliomas (PGLs). The goal of this study is to assess PASS and GAPP as metastatic predictors and to correlate with survival outcomes.
METHODS
The cohort included PCC/PGL with ≥5 years of follow-up or known metastases. Surgical pathology slides were rereviewed. PASS and GAPP scores were assigned. Univariable and multivariable logistic regression, Kaplan-Meier survival analysis, and Cox proportional hazards were performed to assess recurrence-free survival (RFS) and disease-specific survival (DSS).
RESULTS
From 143 subjects, 106 tumors were PCC and 37 were PGL. Metastases developed in 24%. The median PASS score was 6.5 (interquartile range [IQR]: 4.0-8.0) and median GAPP score was 3.0 (IQR: 2.0-4.0). Interrater reliability was low-moderate for PASS (intraclass correlation coefficient [ICC]: 0.6082) and good for GAPP (ICC 0.7921). Older age (OR: 0.969, P = .0170) was associated with longer RFS. SDHB germline pathogenic variant (OR: 8.205, P = .0049), extra-adrenal tumor (OR: 6.357, P < .0001), Ki-67 index 1% to 3% (OR: 4.810, P = .0477), and higher GAPP score (OR: 1.537, P = .0047) were associated with shorter RFS. PASS score was not associated with RFS (P = .1779). On Cox regression, a GAPP score in the moderately differentiated range was significantly associated with disease recurrence (HR: 3.367, P = .0184) compared with well-differentiated score.
CONCLUSION
Higher GAPP scores were associated with aggressive PCC/PGL. PASS score was not associated with metastases and demonstrated significant interobserver variability. Scoring systems for predicting metastatic PCC/PGL may be improved by incorporation of histopathology, clinical data, and germline and somatic tumor markers.
目的
肾上腺嗜铬细胞瘤评分（PASS）和肾上腺嗜铬细胞瘤和副神经节瘤分级系统（GAPP）是预测嗜铬细胞瘤（PCC）和副神经节瘤（PGL）转移潜能的评分系统。本研究的目的是评估 PASS 和 GAPP 作为转移预测因子，并与生存结果相关联。
方法
该队列包括至少 5 年随访或已知转移的 PCC/PGL。重新审查手术病理切片。分配 PASS 和 GAPP 评分。进行单变量和多变量逻辑回归、Kaplan-Meier 生存分析和 Cox 比例风险分析，以评估无复发生存率（RFS）和疾病特异性生存率（DSS）。
结果
从 143 名受试者中，106 例肿瘤为 PCC，37 例为 PGL。24%的患者发生转移。PASS 评分中位数为 6.5（四分位距[IQR]：4.0-8.0），GAPP 评分中位数为 3.0（IQR：2.0-4.0）。PASS 的观察者间可靠性为低-中度（组内相关系数[ICC]：0.6082），GAPP 的可靠性为良好（ICC 0.7921）。年龄较大（OR：0.969，P=0.0170）与 RFS 较长相关。SDHB 种系致病性变异（OR：8.205，P=0.0049）、肾上腺外肿瘤（OR：6.357，P<.0001）、Ki-67 指数 1%-3%（OR：4.810，P=0.0477）和较高的 GAPP 评分（OR：1.537，P=0.0047）与 RFS 较短相关。PASS 评分与 RFS 无关（P=0.1779）。在 Cox 回归中，中度分化范围的 GAPP 评分与疾病复发显著相关（HR：3.367，P=0.0184），而与分化良好的评分相比。
结论
较高的 GAPP 评分与侵袭性 PCC/PGL 相关。PASS 评分与转移无关，且具有显著的观察者间变异性。预测转移性 PCC/PGL 的评分系统可以通过纳入组织病理学、临床数据以及种系和体细胞标志物来改进。
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