Renal Replacement Lipomatosis Presenting in the Setting of Ureteral Stricture with Absence of Renal Calculus Disease.

IF 0.7 Q4 PATHOLOGY Case Reports in Pathology Pub Date : 2021-10-23 eCollection Date: 2021-01-01 DOI:10.1155/2021/3640167
Katrina Collins, Eric Brocken, Laura M Warmke, Temel Tirkes, Michael Hwang
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Abstract

Renal replacement lipomatosis of the kidney is a rare, benign entity in which extensive fibrofatty proliferation of the renal sinus is associated with marked atrophy of the renal parenchyma. It is often associated with calculi or long-standing inflammation. This entity may be confused with a fatty neoplasm of the kidney. A 51-year-old woman with a past medical history of pancreas transplant for type 1 diabetes subsequently developed ureteral stricture. This was initially managed by a nephrostomy tube and nephroureterostomy stenting with periodic exchanges to help restore urine flow; however, the renal function of the kidney progressively declined with recurrent and complicated urinary tract infections. She presented for kidney transplant with right native nephrectomy. Gross examination of the right kidney revealed a 12.8 cm renal sinus lipomatous mass replacing much of the kidney. Microscopically, the mass consisted of mature adipose tissue with fibrous septae and occasional thick-walled vessels with prominent smooth muscle bundles. A rare atypical stromal cell was present, otherwise no significant cytologic atypia or lipoblasts were identified. After excluding fat-predominant angiomyolipoma and well-differentiated liposarcoma, a diagnosis of renal replacement lipomatosis was made. Renal replacement lipomatosis is a benign condition typically associated with a nonfunctioning or poorly functioning kidney often linked to renal calculus disease or chronic renal infection. The presentation in our case was atypical given an absence of associated renal calculus disease. This case is intended to increase awareness of this less commonly encountered entity as it may be confused with a fatty neoplasm of the kidney, some with malignant potential.

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无肾结石的输尿管狭窄患者的肾脏替代脂肪瘤病。
肾脏替代性脂肪瘤病是一种罕见的良性疾病,其表现为肾窦纤维脂肪增生伴肾实质明显萎缩。它通常与结石或长期炎症有关。该实体可能与肾脏脂肪性肿瘤混淆。51岁女性,既往因1型糖尿病进行胰腺移植,随后发生输尿管狭窄。最初通过肾造口管和肾输尿管造口支架置入进行治疗,并定期交换以帮助恢复尿流;然而,肾脏的肾功能逐渐下降与复发和复杂的尿路感染。她提出了肾移植和右原生肾切除术。右肾大体检查发现一个12.8厘米的肾窦脂肪瘤肿块,取代了大部分肾脏。显微镜下,肿块由成熟的脂肪组织和纤维间隔组成,偶尔有厚壁血管和突出的平滑肌束。存在罕见的非典型间质细胞,除此之外未发现明显的细胞学异型性或脂肪母细胞。排除脂肪为主的血管平滑肌脂肪瘤和高分化脂肪肉瘤后,诊断为肾脏替代性脂肪瘤病。肾脏替代脂肪瘤病是一种良性疾病,通常与肾功能不全或肾功能不佳有关,通常与肾结石疾病或慢性肾脏感染有关。我们病例的表现不典型,因为没有相关的肾结石疾病。本病例旨在提高对这种不常见的实体的认识,因为它可能与肾脏的脂肪性肿瘤混淆,有些具有恶性潜能。
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审稿时长
12 weeks
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