根据恶性肿瘤被诊断的时间（在诊断结节病之前、同时或相继发生）来调查结节病的表型，并在一大群结节病患者中确定与恶性肿瘤相关的预后因素。 SARCOGEAS 队列是一个多中心全国数据库，包含根据 ATS/ESC/WASOG 标准诊断为结节病的连续患者。实体恶性肿瘤使用国际疾病和相关健康问题统计分类第十修订版 (ICD-10) 命名法进行分类，血液恶性肿瘤使用 2016 年 WHO 分类。我们排除了经活检证实结节病诊断的患者，该诊断完全基于证明组织中也有恶性细胞所累及的肉芽肿。在 1942 名结节病患者中，233 名 (12%) 患有 250 种恶性肿瘤，包括实体瘤 (n = 173)、血液学 (n = 173) n = 57)，以及两种类型的恶性肿瘤 (n = 3)。关于这两种疾病诊断之间的时间间隔，83 名患者（36%）在结节病诊断前至少 1 年被诊断出患有恶性肿瘤，22 名患者（9%）同时诊断出两种疾病，118 名患者（51%）患有恶性肿瘤诊断结节病后至少 1 年（其余病例在不同时间间隔出现恶性肿瘤）。多变量调整模型显示，患有恶性肿瘤的结节病患者在诊断结节病时无症状临床表型且脾脏的风险比 (HR) 为 2.27 [95% 置信区间 (CI)，1.62-3.17]。存在与不存在：HR = 2.06；95% CI，1.21-3.51）和骨髓（存在与不存在：HR = 3.04；95% CI，1.77-5.24）受累是所有类型恶性肿瘤发生的独立预测因素。当分析仅限于实体恶性肿瘤的发展时，没有发现预测因素。仅限于血液恶性肿瘤发展的分析证实了脾脏（HR = 3.73；95% CI，1.38-10.06）和骨髓（存在与不存在：HR = 8.00；95% CI，3.15-20.35）的存在）在结节病诊断时作为预测因素。必须考虑结节病患者恶性肿瘤诊断的同步或异时时机。我们发现，一半的恶性肿瘤是在诊断出结节病后确诊的，脾脏和骨髓受累与患血液恶性肿瘤的风险增加四到八倍相关。关键信息 关于此主题的已知信息 恶性肿瘤是结节病患者更常遇到的合并症之一 这项研究补充的内容 12% 的结节病患者会发生恶性肿瘤 恶性肿瘤可能先于结节病诊断、同时发生或继发于结节病诊断之后三分之一是在结节病之前确诊的，一半是在结节病之后诊断的接受早期发现教育。那些脾脏或骨髓受累的患者必须密切关注。© 作者 2024。由牛津大学出版社代表研究生医学会出版。版权所有。如需权限，请发送电子邮件至：journals.permissions@oup.com。

To investigate the phenotype of sarcoidosis according to the time when a malignancy is diagnosed (preexisting to the diagnosis of sarcoidosis, concomitant, or sequential) and to identify prognostic factors associated with malignancies in a large cohort of patients with sarcoidosis.We searched for malignancies in the SARCOGEAS cohort, a multicenter nationwide database of consecutive patients diagnosed with sarcoidosis according to the ATS/ESC/WASOG criteria. Solid malignancies were classified using the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) nomenclature, and hematological malignancies using the 2016 WHO classification. We excluded patients with a biopsy-proven diagnosis of sarcoidosis based exclusively on demonstrating granulomas in tissues also involved by malignant cells.Out of 1942 patients with sarcoidosis, 233 (12%) developed 250 malignancies, including solid (n = 173), hematological (n = 57), and both types of malignancies (n = 3). Concerning the time interval between the diagnoses of both conditions, 83 (36%) patients were diagnosed with malignancy at least 1 year before sarcoidosis diagnosis, 22 (9%) had s synchronous diagnosis of both diseases, and 118 (51%) developed malignancies at least 1 year after the diagnosis of sarcoidosis (the remaining cases developed malignancies in different time intervals). The multivariate-adjusted model showed that individuals with sarcoidosis who developed a malignancy had an hazard ratio (HR) of 2.27 [95% confidence interval (CI), 1.62-3.17] for having an asymptomatic clinical phenotype at diagnosis of sarcoidosis and that spleen (presence vs. absence: HR = 2.06; 95% CI, 1.21-3.51) and bone marrow (presence vs. absence: HR = 3.04; 95% CI, 1.77-5.24) involvements were independent predictors for the development of all-type malignancies. No predictive factors were identified when the analysis was restricted to the development of solid malignancies. The analysis limited to the development of hematological malignancies confirmed the presence of involvement in the spleen (HR = 3.73; 95% CI, 1.38-10.06) and bone marrow (presence vs. absence: HR = 8.00; 95% CI, 3.15-20.35) at the time of sarcoidosis diagnosis as predictive factors.It is essential to consider the synchronous or metachronous timing of the diagnosis of malignancies in people with sarcoidosis. We found that half of the malignancies were diagnosed after a diagnosis of sarcoidosis, with spleen and bone marrow involvement associated with a four to eight times higher risk of developing hematological malignancies. Key messages What is already known on this topic Malignancies are one of the comorbidities more frequently encountered in people with sarcoidosis What this study adds Malignancies occur in 12% of patients with sarcoidosis Malignancy may precede, coincide with, or follow the diagnosis of sarcoidosis One-third were identified before sarcoidosis, and half were diagnosed after Spleen and bone marrow involvement are risk factors for developing hematological malignancies How this study might affect research, practice or policy Patients with sarcoidosis should be regularly monitored for neoplasms, informed of the increased risk, and educated on early detection. Those with spleen or bone marrow involvement must be closely followed.© The Author(s) 2024. Published by Oxford University Press on behalf of Fellowship of Postgraduate Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.