Rationale: Primary adenocarcinoma of the renal pelvis is a rare malignant tumor, with approximately 100 cases reported in the English literature. This malignancy is characterized by high-grade aggressiveness, diagnostic challenges at initial presentation, and advanced disease at diagnosis, leading to poor overall prognosis. Historically, treatment has focused on surgical resection combined with chemotherapy, but documented cases remain scarce. The emergence of immune checkpoint inhibitors (ICIs) necessitates the exploration of novel therapeutic combinations to improve survival outcomes. We present a case of primary renal pelvic adenocarcinoma managed with a multimodal approach integrating radical surgery, adjuvant chemotherapy, and concurrent ICI therapy, resulting in prolonged survival.

Patient concerns: A 61-year-old male presented with a 3-month history of recurrent left flank and abdominal pain. He reported no hematuria or weight loss but expressed concern about worsening pain unresponsive to prior interventions. A left nephrostomy performed at an outside hospital revealed a renal pelvic mass, prompting a referral for further evaluation.

Diagnoses: Previous left nephrostomy and drainage at a local hospital revealed a renal pelvic mass, with a biopsy confirming adenocarcinoma. Contrast-enhanced computed tomography demonstrated abnormal thickening of the left renal pelvis, calyces, and upper-middle ureter, retroperitoneal lymphadenopathy, postnephrostomy changes, and bilateral renal calculi. Serum carbohydrate antigen 19-9 (CA19-9) was elevated to 431.793 U/mL.

Interventions: Laparoscopic radical left nephroureterectomy with partial cystectomy was performed on January 12, 2023, revealing a thickened left ureter and retroperitoneal lymphadenopathy. Pathology confirmed an 8 cm moderately differentiated adenocarcinoma infiltrating renal/ureteral/bladder tissues. Immunohistochemistry: CK(+)/CK7(+)/CEA(+), Ki-67 20% to 30%. Intraoperative pirarubicin instillation was administered.

Outcomes: Postoperative stage IV disease received 2 cycles of adjuvant docetaxel. Disease progression (April 2023) prompted gemcitabine + tislelizumab (6 cycles), achieving a partial response (lymph node regression; CA19-9: 431.8→22.4 U/mL). Acute kidney injury (August 2023) necessitated tislelizumab monotherapy (3 cycles). Lymph node recurrence (January 2024) led to gemcitabine-tislelizumab rechallenge (2 cycles), followed by tislelizumab-pemetrexed (3 cycles) for stable disease. Bilateral lung metastases emerged post-treatment discontinuation (January 2025), treated with toripalimab-lenvatinib (1 cycle). The final follow-up (March 2025) documented 28-month survival (Eastern Cooperative Oncology Group 1).

Lessons: This case underscores the imperative for multimodal integration (surgery, chemotherapy, and ICIs) to optimize survival in advanced renal pelvic adenocarcinoma while highlighting the necessity of dynamic therapeutic adaptation-including regimen rechallenge and tyrosine kinase inhibitor combinations-to address recurrence and resistance. Proactive toxicity management (e.g., dose de-escalation for renal injury) and rigorous biomarker-driven surveillance (serial CA19-9 tracking with 3-month imaging) emerge as critical strategies to balance efficacy and safety in this aggressive malignancy.

Keywords: adenocarcinoma of the renal pelvis; case report; chemotherapy; immunotherapy; surgery.

Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc.

理由：

肾盂原发性腺癌是一种罕见的恶性肿瘤，在英文文献中大约有100例报道。这种癌症的特点是高度恶性的侵袭性、初次诊断时的挑战性和晚期诊断，导致整体预后不良。历史上治疗主要集中在手术切除结合化疗上，但记录在案的病例仍然很少。免疫检查点抑制剂（ICIs）的出现需要探索新的联合治疗方法以提高生存率。我们介绍了一例通过多模式方法管理原发性肾盂腺癌的案例，该方法整合了根治性手术、辅助化疗和同期ICI治疗，延长了患者的生存期。

患者主诉：

一位61岁的男性因左腰区及腹部疼痛3个月就诊。他没有血尿或体重减轻的症状，但表达了对之前干预措施无效的担忧，并表示疼痛逐渐加重。在外地医院进行左侧肾造瘘术后发现有肾盂肿块，因此转诊进一步评估。

诊断：

前次左侧肾造瘘术和引流在外院完成时发现了肾盂肿块，活检证实为腺癌。增强CT显示左肾盂、肾盏及上中段输尿管异常增厚，腹膜后淋巴结增大，肾造瘘术后改变以及双侧肾脏结石。血清糖类抗原19-9（CA19-9）升高至431.793 U/mL。

干预措施：

2023年1月12日进行了腹腔镜左肾输尿管根治性切除术和部分膀胱切除术，发现左侧输尿管增厚及腹膜后淋巴结增大。病理学检查确认了侵入肾脏/输尿管/膀胱组织的8厘米中度分化腺癌。免疫组化：CK(+)/CK7(+)/CEA(+)，Ki-67 20%至30%。术中给予吡柔比星灌注。

结果：

术后分期为IV期的患者接受了两周期的辅助多西他赛治疗。疾病进展（2023年4月）促使使用吉西他滨+tislelizumab（6个疗程），取得了部分反应（淋巴结退缩；CA19-9：从431.8降至22.4 U/mL）。急性肾损伤（2023年8月）导致tislelizumab单药治疗（三个周期）。淋巴结复发（2024年1月）促使重新使用吉西他滨+tislelizumab（两个周期），随后采用tislelizumab+培美曲塞（三个周期）维持病情稳定。在停用治疗后（2025年1月）出现双肺转移，给予托里帕利姆-乐伐替尼（一个疗程）。最后一次随访（2025年3月）记录了患者生存期为28个月（东部协作肿瘤组评分为1级）。

教训：

本案例强调了多模式整合（手术、化疗和ICIs）在优化晚期肾盂腺癌患者的生存率中的重要性，同时突出了动态治疗调整的必要性——包括方案重新挑战以及酪氨酸激酶抑制剂联合疗法的应用以应对复发及耐药问题。积极管理毒性反应（例如针对肾脏损伤降低剂量）、严格的生物标志物驱动监控（如每三个月追踪CA19-9和成像）在平衡此恶性肿瘤治疗中的疗效与安全性中显得尤为关键。

关键词：

肾盂腺癌；病例报告；化疗；免疫疗法；手术。