Iguchi Daniela Yone Veiga, Martins Filho Sebastião Nunes, Soares Iberê Cauduro, Siqueira Sheila Aparecida Coelho, Alves Venâncio Avancini Ferreira, Assato Aline Kawassaki, Yang Ji Hoon, Almeida Madson Q, Villares Fragoso Maria Candida Barisson, Fagundes Gustavo Freitas Cardoso, Mendonca Berenice B, Lourenço Junior Delmar Muniz, Hoff Ana O, Castroneves Luciana Audi, Ferraz-de-Souza Bruno, Giannella Maria Lucia Cardillo Correa, Pereira Maria Adelaide Albergaria
Divisão de Endocrinologia e Metabologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo 05403-000, Brazil.
Department of Laboratory Medicine and Pathology, University of Alberta, Alberta T6G 1C9, Canada.
J Endocr Soc. 2024 May 7;8(7):bvae093. doi: 10.1210/jendso/bvae093. eCollection 2024 May 23.
Paragangliomas (PGLs) are rare tumors in adrenal and extra-adrenal locations. Metastasis are found in approximately 5% to 35% of PGLs, and there are no reliable predictors of metastatic disease.
This work aimed to develop a prognostic score of metastatic potential in PGLs.
A retrospective analysis was conducted of clinical data from a cohort with PGLs and tumor histological assessment. Patients were divided into metastatic PGL (presence of metastasis) and nonmetastatic PGL (absence of metastasis ≥96 months of follow-up) groups. Univariate and multivariable analysis were performed to identify predictors of metastatic potential. A prognostic score was developed based on coefficients of multivariable analysis. Kaplan-Meier curves were generated to estimate disease-specific survival (DSS).
Out of 263 patients, 35 patients had metastatic PGL and 110 patients had nonmetastatic PGL. In multivariable analysis, 4 features were independently related to metastatic disease and composed the Prognostic Score of Paragangliomas (PSPGL): presence of central or confluent necrosis (33 points), more than 3 mitosis/10 high-power field (HPF) (28 points), extension into adipose tissue (20 points), and extra-adrenal location (19 points). A PSPGL of 24 or greater showed similar sensitivity with higher specificity than the Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP). PSPGL less than or equal to 20 was associated with a risk of metastasis of approximately 10%, whereas a PSPGL of 40 or greater was associated with approximately 80%. The presence of metastasis and Ki-67 of 3% or greater were related to lower DSS.
The PSPGL, composed of 4 easy-to-assess parameters, demonstrated good performance in predicting metastatic potential and good ability in estimating metastasis risk.
副神经节瘤（PGLs）是肾上腺及肾上腺外部位的罕见肿瘤。约5%至35%的PGLs会发生转移，且尚无转移性疾病的可靠预测指标。
本研究旨在建立一种PGLs转移潜能的预后评分系统。
对一组PGLs患者的临床资料及肿瘤组织学评估进行回顾性分析。将患者分为转移性PGL（存在转移）和非转移性PGL（随访≥96个月无转移）两组。进行单因素和多因素分析以确定转移潜能的预测指标。基于多因素分析系数建立预后评分系统。绘制Kaplan-Meier曲线以评估疾病特异性生存率（DSS）。
263例患者中，35例为转移性PGL，110例为非转移性PGL。多因素分析显示，4个特征与转移性疾病独立相关，构成副神经节瘤预后评分（PSPGL）：存在中央或融合性坏死（33分）、每10个高倍视野（HPF）有超过3个有丝分裂（28分）、侵犯脂肪组织（20分）及肾上腺外部位（19分）。PSPGL为24分及以上时，其敏感性与肾上腺嗜铬细胞瘤分级评分（PASS）和肾上腺嗜铬细胞瘤及副神经节瘤分级系统（GAPP）相似，但特异性更高。PSPGL小于或等于20分与转移风险约10%相关，而PSPGL为40分及以上与约80%的转移风险相关。转移的存在及Ki-67≥3%与较低的DSS相关。
由4个易于评估的参数组成的PSPGL在预测转移潜能方面表现良好，在估计转移风险方面能力较强。
