Tannous Elie, Patel Shreya, Muratoglu Burak, Lightle Andrea R, Pacheco Richard R, Hosseini Reza, Pacheco Robert R, Kim Peter, Cetinkaya Gamze T, Baydar Dilek Ertoy, Kosemehmetoglu Kemal, Karabulut Yasemin Yuyucu, Appu Sree, Galea Laurence A, Bernstein Adrien N, Akgul Mahmut
Department of Pathology and Laboratory Medicine, Albany Medical Center, 43 New Scotland Avenue, Room F110G3, Albany, NY, USA.
Department of Pathology, Yale University, New Haven, CT, USA.
Virchows Arch. 2025 Jul;487(1):227-236. doi: 10.1007/s00428-024-03930-5. Epub 2024 Sep 28.
Localized cystic disease of the kidney (LCDK) is rare without hereditary background and does not progress. It can mimic neoplastic process, leading to unnecessary surgical intervention. We present 14 patients [male-to-female 9:5; mean age 50.3 years (range: 3-79)] with LCDK in a multinational cohort. Flank pain (n=5) and incidental lesions (n=4) were common. All cases were unilateral (9 right, 5 left), and contralateral kidneys were mostly normal (n=11). No family history was present, and none had extrarenal solid organ cysts. Radical and partial nephrectomies were performed in 9 and 5 cases, respectively. All lesions were multilocular, ranging from 1.8 - 20cm. 2 cases had diffuse renal involvement. Cystic septa contained nonneoplastic elements including renal tubules and glomeruli without primitive epithelial cellular elements, blastema, or immature stromal cells. In addition, we also comprehensively reviewed 75 previously reported cases. Conclusions. LCDK should be considered in the differential of cystic kidney lesions.
局限性肾囊性疾病(LCDK)在无遗传背景的情况下较为罕见,且不会进展。它可能会模拟肿瘤性病变,导致不必要的手术干预。我们在一个跨国队列中报告了14例LCDK患者[男:女为9:5;平均年龄50.3岁(范围:3 - 79岁)]。胁腹疼痛(n = 5)和偶然发现的病变(n = 4)较为常见。所有病例均为单侧(9例右侧,5例左侧),对侧肾脏大多正常(n = 11)。均无家族史,且均无肾外实性器官囊肿。分别有9例和5例进行了根治性肾切除术和部分肾切除术。所有病变均为多房性,大小范围为1.8 - 20厘米。2例有弥漫性肾脏受累。囊性间隔包含非肿瘤性成分,包括肾小管和肾小球,但无原始上皮细胞成分、胚基或未成熟的基质细胞。此外,我们还全面回顾了75例先前报道的病例。结论。在鉴别肾囊性病变时应考虑LCDK。