Division of Rheumatology, School of Medicine, Kyungpook National University, 130 Dongdeok-ro, Jung-gu, Daegu, 41944, Republic of Korea.
Department of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology, and College of Medicine, Medical Research Institute, Seoul National University, Seoul, Republic of Korea.
Arthritis Res Ther. 2021 Sep 26;23(1):249. doi: 10.1186/s13075-021-02627-3.
Retroperitoneal fibrosis (RPF) is characterized by a highly fibrotic retroperitoneal mass and encompasses the idiopathic form and secondary to malignancies. Because we have limited knowledge whether RPF is associated with malignancy, we aimed to investigate the relationship between RPF and malignancy and to compare the characteristics and prognosis of cancers among patients with RPF.
Medical records of 111 patients diagnosed as having RPF were reviewed and 38 cases of cancer, confirmed by biopsy, were identified. Standardized incidence ratios (SIRs) were calculated for cancers and stratified according to cancer type and RPF-cancer diagnosis interval. Cancer characteristics and outcomes were compared between RPF-cancer diagnosis intervals.
The average age at RPF diagnosis was 59.2 ± 15.0 years, and 69.4% of the patients were male. The cancer SIRs in patients with RPF relative to age- and sex-matched individuals in the general population was 2.2 (1.6-3.1). SIRs of renal pelvis cancer and multiple myeloma were significantly higher than in the general population. When stratified by RPF-cancer intervals, the SIR for cancer was 9.9 within 1 year of RPF diagnosis, while no significant increase in the SIR was found after 1 year from RPF diagnosis. Cancer stage was more advanced at the time of diagnosis in patients within a 1-year interval for RPF than those with cancer within a >5-year interval, with a correspondingly increased mortality in the former patients.
RPF was significantly associated with malignancy, particularly those diagnosed within 1 year of RPF diagnosis. Cancer stages at diagnosis were more advanced and the mortality rate was higher in patients within a 1-year interval between RPF and cancer diagnosis than in those with a >5-year interval between diagnoses.
腹膜后纤维化（RPF）的特征是腹膜后有高度纤维化的肿块，包括特发性和恶性肿瘤继发的两种类型。由于我们对 RPF 是否与恶性肿瘤有关知之甚少，因此我们旨在研究 RPF 与恶性肿瘤之间的关系，并比较 RPF 患者中癌症的特征和预后。
回顾了 111 例被诊断为 RPF 的患者的病历，并通过活检确定了 38 例癌症病例。计算了癌症的标准化发病比（SIR），并根据癌症类型和 RPF-癌症诊断间隔进行分层。比较了 RPF-癌症诊断间隔内癌症的特征和结局。
RPF 诊断时的平均年龄为 59.2±15.0 岁，69.4%的患者为男性。与普通人群中年龄和性别匹配的个体相比，RPF 患者的癌症 SIR 为 2.2（1.6-3.1）。肾盂癌和多发性骨髓瘤的 SIR 明显高于普通人群。按 RPF-癌症间隔分层时，RPF 诊断后 1 年内癌症的 SIR 为 9.9，而 RPF 诊断后 1 年以上，SIR 无明显增加。在 RPF 诊断后 1 年内诊断出癌症的患者，其癌症分期在诊断时更为晚期，死亡率也相应较高。
RPF 与恶性肿瘤显著相关，特别是在 RPF 诊断后 1 年内诊断出的恶性肿瘤。在 RPF 和癌症诊断之间间隔 1 年以内的患者，其诊断时的癌症分期更为晚期，死亡率也更高。
