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中华腔镜泌尿外科杂志(电子版) ›› 2026, Vol. 20 ›› Issue (05) : 601 -608. doi: 10.3877/cma.j.issn.1674-3253.2026.05.017

MDT精选病例

广东省医学会泌尿外科疑难病例多学科会诊(第35期)——转移性复发性嗜铬细胞瘤
冯嘉耀1, 曾翔宇1, 赖文杰1, 赵忠祥1, 唐柯1, 赖洪燊1, 钟碧玲2, 邱晓拂1,()   
  1. 1510317 广州,暨南大学附属广东省第二人民医院泌尿外科
    2510317 广州,暨南大学附属广东省第二人民医院病理科
  • 收稿日期:2026-06-29 出版日期:2026-10-01
  • 通信作者: 邱晓拂
  • 基金资助:
    佛山市南海区重点领域科技攻关项目(2230032004653); 广东省第二人民医院临床研究项目(LCYJ2025003)

Multidisciplinary consultation on difficult cases in Guangdong Urological Association (Phase 35): metastatic recurrent pheochromocytoma

Jiayao Feng1, Xiangyu Zeng1, Wenjie Lai1, Zhongxiang Zhao1, Ke Tang1, Hongshen Lai1, Biling Zhong2, Xiaofu Qiu1,()   

  1. 1Department of Urology, the Affiliated Guangdong Second Provincial General Hospital of Jinan University, Guangzhou 510317, China
    2Department of Pathology, the Affiliated Guangdong Second Provincial General Hospital of Jinan University, Guangzhou 510317, China
  • Received:2026-06-29 Published:2026-10-01
  • Corresponding author: Xiaofu Qiu
引用本文:

冯嘉耀, 曾翔宇, 赖文杰, 赵忠祥, 唐柯, 赖洪燊, 钟碧玲, 邱晓拂. 广东省医学会泌尿外科疑难病例多学科会诊(第35期)——转移性复发性嗜铬细胞瘤[J/OL]. 中华腔镜泌尿外科杂志(电子版), 2026, 20(05): 601-608.

Jiayao Feng, Xiangyu Zeng, Wenjie Lai, Zhongxiang Zhao, Ke Tang, Hongshen Lai, Biling Zhong, Xiaofu Qiu. Multidisciplinary consultation on difficult cases in Guangdong Urological Association (Phase 35): metastatic recurrent pheochromocytoma[J/OL]. Chinese Journal of Endourology(Electronic Edition), 2026, 20(05): 601-608.

本文报道1例转移性复发性肾上腺嗜铬细胞瘤。患者为青年男性,因反复胸闷、胸痛就诊,2019年首次发现右侧肾上腺占位并行腹腔镜下右侧肾上腺肿物切除术,术后病理证实为嗜铬细胞瘤,Ki-67约1%~2%(+),SDH-B(+)。2022年患者出现术区复发并腹膜、大网膜、淋巴结转移,接受减瘤手术及环磷酰胺、长春新碱和达卡巴嗪联合方案化疗。2025年患者再次出现术区复发及腹膜多发转移,行二次减瘤手术并取组织行患者来源类器官培养,药敏结果显示舒尼替尼可显著抑制类器官三磷酸腺苷活性及肾上腺素分泌,经广东省医学会泌尿外科疑难病例多学科会诊(MDT)讨论后给予舒尼替尼37.5 mg/d口服靶向治疗。随访至今,患者一般状况良好,无胸闷、心悸等不适。本例提示,转移复发性嗜铬细胞瘤的临床管理不能局限于传统病理低危表象,需结合复发模式、转移证据及全病程演变综合评估其恶性潜能。此次MDT整合了泌尿外科、影像科、病理科及肿瘤内科等多学科分析,明确手术减瘤、系统治疗选择及长期随访策略的个体化治疗方案,体现出MDT在复杂病例的诊疗中具有重要临床指导价值。

This article reports a case of metastatic recurrent adrenal pheochromocytoma in a young man presenting with recurrent chest tightness and chest pain. In 2019, a right adrenal mass was detected, and laparoscopic resection of the right adrenal tumor was performed. Pathology confirmed pheochromocytoma, with a Ki-67 index of 1%-2% and positive SDH-B expression. In 2022, recurrence at the operative site with peritoneal, greater omental, and lymph node metastases was identified, and the patient underwent debulking surgery followed by cyclophosphamide, vincristine, and dacarbazine (CVD) chemotherapy. In 2025, recurrent disease at the operative site and multiple peritoneal metastases were detected again. A second debulking surgery was performed, and tumor tissue was obtained for patient-derived organoid culture. Drug sensitivity testing showed that sunitinib markedly inhibited adenosine triphosphate (ATP) activity and epinephrine secretion in the organoids. After multidisciplinary consultation on difficult cases in Guangdong Urological Association, oral targeted therapy with sunitinib at 37.5 mg/day was initiated. At the latest follow-up, the patient remained in good general condition, without chest tightness, palpitations, or other discomfort. This case suggests that the management of metastatic recurrent pheochromocytoma should not rely solely on conventionally low-risk pathological features, but should incorporate recurrence pattern, metastatic evidence, and the longitudinal disease course to assess malignant potential. In this case, MDT discussion integrated input from Urology, Radiology, Pathology, Medical Oncology, and other specialties. This collaborative approach helped establish an individualized strategy involving surgical debulking, systemic treatment selection, and long-term follow-up, thereby highlighting the important clinical value of MDT-based decision-making in complex cases.

图1 转移性复发性嗜铬细胞瘤患者初次发病时的影像学、病理学及术后复查结果注:a~b为2019年5月增强CT示右侧肾上腺占位(箭头示);c为术后肿瘤大体标本;d~f为HE染色示(右肾上腺肿物)嗜铬细胞瘤,伴囊性变,局部Zellballen巢扩大,核分裂象0~1个/10 HPF,未见明确凝固性坏死,未见明确包膜、脉管侵犯,周围肾上腺及脂肪组织阴性;g~h为2019年7月术后复查CT示原右侧肾上腺肿物已切除,术区未见明确残留病灶(箭头示)
图2 转移性复发性嗜铬细胞瘤患者第一次复发时的影像学、病理学及术后复查结果注:a~b为2022年6月增强CT示右侧肾上腺肿瘤术后改变,现术区占位(箭头示),c~d示右网膜下多发肿大淋巴块(箭头示);e~h为2022年6月PET-CT示右侧肾上腺术区见一形态欠规则软组织肿块,右肾下极外侧局部腹膜结节状增厚,右中下腹大网膜及脐水平右腹膜后区见多发软组织密度结节(箭头示);i为肿瘤大体标本;j为(右肾上腺肿物)HE染色示嗜铬细胞瘤;k为(腹膜上肿物)HE染色示脂肪结缔组织内大小不等嗜铬细胞瘤结节浸润生长,个别结节合并大片凝固性坏死;l为(大网膜及其肿物)HE染色示网膜脂肪结缔组织内大小不等嗜铬细胞瘤结节浸润生长;m~p为2022年8月复查CT示原术区占位及右下网膜多发肿大淋巴结未再显示,术区未见明显异常密度影(箭头示)
图3 转移性复发性嗜铬细胞瘤患者第二次复发时的影像学、病理学、类器官培养及药敏试验结果注:a~b为2025年6月增强MRI示右侧肾上腺术区结节状异常信号影,增强扫描动脉期较均匀强化(箭头示);c~h为右肾上腺术区下腔静脉旁、右侧肾上腺区(肝右叶后内侧)肿块及腹膜多发结节,较前增多、增大,代谢活跃(箭头示);i为术后病理标本;j为HE染色示肿瘤细胞呈片状及小巢状排列,胞浆较丰富,核圆形或卵圆形,异型性不显著,间质血管较丰富,符合嗜铬细胞瘤的形态学特征;k~l为显微镜下患者来源嗜铬细胞瘤类器官培养结果,可见类器官形成稳定三维球状结构;m为不同药物处理后类器官肾上腺素分泌水平变化;n示不同药物处理后类器官三磷酸腺苷(ATP)活性变化;*P<0.05,**P<0.01,***P<0.001,NS表示P>0.05
图4 转移性复发性嗜铬细胞瘤患者诊疗时间轴注:CVD方案为环磷酰胺+长春新碱+达卡巴嗪
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