Urology Department, Egas Moniz Hospital, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.
Pathology Department, Champalimaud Clinical Centre, Lisbon, Portugal.
Virchows Arch. 2023 Mar;482(3):493-505. doi: 10.1007/s00428-022-03481-7. Epub 2023 Jan 5.
We report on the clinicopathologic features of 27 pleomorphic giant cell carcinoma (PGCC) cases of the prostate identified in 20 patients with an age range of 51 to 84 years (68 ± 9; median 71 years). Charlson comorbidity index ranged from 3 to 12. Serum PSA ranged from 4.30 to 662 ng/mL (median 13 ng/mL). On histologic examination, bizarre giant cells with pleomorphic nuclei characterized pleomorphic giant cell carcinoma of the prostate. PGCC component was present in 5% to 100%, with half of the patients presenting with ≥ 20%. Half of the patients initially presented with T4 and 26% with T3 disease. All patients were considered Gleason scores of 9 to 10 (ISUP grade 5). A combination of hormone therapy with chemotherapy with or without radiation therapy was applied in 68% of patients. On follow-up, 14 patients (52%) were alive with disease (1-69 months) or dead of disease (1-38 months). Patients diagnosed earlier with lower TNM stage had longer survival than those diagnosed at a later T-stage or with metastatic disease (p = 0.02). The percentage of PGCC was not related to survival in the current study. Molecular alterations in 3 samples showed a microsatellite-stable disease with low tumor mutation burden and variable PTEN, PTCH1, KDM6A, ARv7, and PIK3CA loss/alteration, TP53 mutation, TMPRSS2-ERG fusion, and MYC, PIK3CB, RICTOR, or IRS2 amplification. Our findings suggest that PGCC is a rare and aggressive subtype of prostate carcinoma whose recognition may steer clinicians to adopt more aggressive treatments and investigate new therapeutic strategies.
我们报告了 27 例前列腺多形性巨细胞癌(PGCC)病例的临床病理特征,这些病例来自 20 名年龄在 51 至 84 岁之间的患者(68±9;中位年龄 71 岁)。Charlson 合并症指数为 3 至 12。血清 PSA 范围为 4.30 至 662ng/mL(中位数 13ng/mL)。在组织学检查中,具有多形性核的奇异巨细胞特征构成前列腺多形性巨细胞癌。PGCC 成分存在于 5%至 100%之间,其中一半患者的含量≥20%。一半的患者最初表现为 T4,26%的患者表现为 T3 疾病。所有患者的 Gleason 评分均为 9 至 10(ISUP 分级 5)。68%的患者接受了激素治疗联合化疗,或联合或不联合放射治疗。随访时,14 名患者(52%)患有疾病(1-69 个月)或死于疾病(1-38 个月)。在本研究中,较早诊断为较低 TNM 分期的患者比晚期 T 分期或转移性疾病患者的生存时间更长(p=0.02)。PGCC 的百分比与当前研究中的生存无关。3 个样本的分子改变显示微卫星稳定疾病,肿瘤突变负担低,PTEN、PTCH1、KDM6A、ARv7 和 PIK3CA 缺失/改变、TP53 突变、TMPRSS2-ERG 融合以及 MYC、PIK3CB、RICTOR 或 IRS2 扩增。我们的研究结果表明,PGCC 是一种罕见且侵袭性的前列腺癌亚型,其识别可能促使临床医生采用更具侵袭性的治疗方法,并研究新的治疗策略。