Chanroo Toni, Silverstein Allison, McAtee Casey L, Kamiyango William, Villiera Jimmy, Mehta Parth S, Peckham-Gregory Erin, Zobeck Mark, Scheurer Michael E, Allen Carl E, Mzikamanda Rizine, Ozuah Nmazuo W, El-Mallawany Nader Kim
Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
Department of Pediatrics, University of Colorado, Aurora, Colorado, USA.
Pediatr Hematol Oncol. 2025 Jun 22:1-11. doi: 10.1080/08880018.2025.2518365.
Kaposi sarcoma (KS) is a common childhood cancer in Malawi, but few studies have explored clinical characteristics of relapsed disease. We aimed to characterize clinical patterns of relapse to improve treatment and, ultimately, long-term survival in patients with pediatric KS. A retrospective cohort study was conducted among patients ages <19 years of age at time of KS diagnosis in Lilongwe, Malawi between August 1, 2010 and March 15, 2020. Specifically, emphasis was placed on patients who had relapsed disease and excluded patients with refractory disease or those who died whilst receiving front-line treatment. Salvage therapy typically involved an intensified chemotherapy regimen compared to front-line therapy - namely nonliposomal doxorubicin plus bleomycin/vincristine or paclitaxel monotherapy. One-hundred and ninety patients with pediatric KS were included in this analysis, 50 of whom experienced relapse (26%). Older median age was associated with occurrence of relapse (10 vs. 6.7 years, -value = 0.004). Median time from diagnosis to first relapse was 10.6 months (range 2.3-49 months). Three-year post-relapse overall survival (OS) for the entire cohort was 60% with a median follow-up time of 4.7 years after relapse. Survival was significantly higher for patients who relapsed with the woody edema clinical phenotype of pediatric KS versus those with visceral/disseminated disease - 3-year OS 79% (95% CI 62-100) vs. 29% (14-61). These data demonstrate potential for continued survival after KS relapse in the pediatric population and identify subsets of high-risk patients. The higher mortality observed in patients with visceral/disseminated KS highlights the need for improved therapeutic strategies.
卡波西肉瘤(KS)是马拉维常见的儿童癌症,但很少有研究探讨复发性疾病的临床特征。我们旨在描述复发的临床模式,以改善治疗,并最终提高小儿KS患者的长期生存率。对2010年8月1日至2020年3月15日期间在马拉维利隆圭诊断为KS时年龄小于19岁的患者进行了一项回顾性队列研究。具体而言,重点关注患有复发性疾病的患者,排除患有难治性疾病的患者或在接受一线治疗时死亡的患者。与一线治疗相比,挽救治疗通常采用强化化疗方案,即非脂质体阿霉素加博来霉素/长春新碱或紫杉醇单药治疗。本分析纳入了190例小儿KS患者,其中50例经历了复发(26%)。年龄中位数较大与复发的发生相关(10岁对6.7岁,P值=0.004)。从诊断到首次复发的中位时间为10.6个月(范围2.3 - 49个月)。整个队列复发后三年的总生存率(OS)为60%,复发后中位随访时间为4.7年。小儿KS的木样水肿临床表型复发的患者生存率明显高于内脏/播散性疾病患者 - 三年总生存率分别为79%(95%CI 62 - 100)和29%(14 - 61)。这些数据表明小儿人群KS复发后有继续生存的潜力,并确定了高危患者亚组。内脏/播散性KS患者中观察到的较高死亡率凸显了改进治疗策略的必要性。