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[Atrophic kidney-like lesion: a clinicopathological study of three cases]
Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology
|January 6, 2025
Summary
Atrophic kidney-like lesion (AKLL) is a rare, benign kidney condition that can be mistaken for renal neoplasm. Characteristic immunophenotypes and morphology aid in its accurate diagnosis and differential diagnosis.
Area of Science:
- Nephrology
- Pathology
- Oncology
Background:
- Atrophic kidney-like lesion (AKLL) is a rare renal entity.
- It can be challenging to distinguish from renal neoplasms based on gross and histological examination.
Purpose of the Study:
- To investigate the clinicopathological characteristics, immunophenotypes, diagnostic criteria, and differential diagnosis of AKLL.
- To provide insights for accurate diagnosis and management of this rare renal disease.
Main Methods:
- Analysis of clinical, morphological, and immunohistochemical features of three AKLL cases.
- Review of relevant literature.
- Targeted next-generation sequencing on one case.
Main Results:
- AKLL presents as a unilateral renal mass with variable gross appearance.
- Histologically, it shows atrophic tubules and cystic structures with specific cellular linings.
- Immunohistochemistry revealed distinct markers: WT1 positive, PAX8 negative, CK7 negative in cyst lining cells, and WT1 negative, PAX8 positive, CK7 positive in atrophic tubules.
Conclusions:
- AKLL is a rare, benign renal disease that requires careful evaluation.
- Distinct morphological and immunophenotypical features are crucial for differentiating AKLL from renal neoplasms.
- Partial nephrectomy is an effective treatment, with no observed recurrence in the study cohort.
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