Localized cystic disease of the kidney in pediatric patients: Clinical and imaging findings with long term follow up

Yoon, Haesung; Kim, Myung Joon; Shin, Hyun Joo; Lee, Mi Jung

doi:10.1016/j.jpurol.2021.11.002

Cited by 1 publication

(5 citation statements)

References 24 publications

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“…They are surrounded by unremarkable renal parenchyma, and the cysts are filled with clear yellow fluid with no evidence of tumor formation. 2 , 11 In the original study by Cho et al, 1 microscopic image demonstrated dilatation of ducts and tubules of the kidney forming cysts lined by flattened to cuboidal bland epithelium. No mitotic activity nor necrosis was seen.…”

Section: Discussionmentioning

confidence: 98%

“…However, there have been documented case reports of LCKD occurring in younger patient populations as well. 2 , 5 In a study by Yoon et al, 2 11 cases of pediatric LCKD were reported. These cases included infants as young as 6 months and 20 days of age with no family history of ADPKD.…”

Section: Discussionmentioning

confidence: 99%

“…Regardless of age, the clinical symptoms range from asymptomatic to the presence of hematuria, flank pain, and palpable abdominal masses. 2 , 6 - 12 …”

Section: Discussionmentioning

confidence: 99%

“…US findings typically show multiple, variably sized, round cysts with adjacent normal renal parenchyma. 2 CT findings may also reveal focal calcifications within the cyst walls in both the pediatric and adult populations, 2 which aids in differentiating it from other entities such as multilocular cystic nephroma (MLCN), which preferentially affects boys in the pediatric population and women in the adult population. 13 Angiograms can also be utilized to assess LCKD, revealing avascular masses devoid of neovascularity.…”

Section: Discussionmentioning

confidence: 99%

“…The condition was first described in 1979 by Cho et al, 1 and has been referred to by many names, most commonly unilateral polycystic kidney disease and segmental polycystic kidney disease. 2 , 3 However, the inclusion of the term “polycystic” in its name can be misleading and has the potential for a misdirected correlation with autosomal dominant polycystic kidney disease (ADPKD). Unlike more aggressive kidney disorders such as polycystic kidney disease, LCKD is an entity that refrains from inflicting renal degeneration or progressing to a decline in renal function.…”

Section: Introductionmentioning

confidence: 99%

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Localized cystic kidney disease: a case report unveiling clinical and histopathological challenges

Mendez,

Bahmad,

Polit

et al. 2024

Autops Case Rep

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Localized cystic kidney disease (LCKD) is a distinct renal disorder characterized by the presence of cysts within specific regions of the kidneys. We present a rare case of a 41-year-old African American man, who presented to our medical center with lower urinary tract symptoms and gross hematuria. The initial assessment culminated in the identification of an uncomplicated urinary tract infection, prompting the prescription of appropriate oral antibiotic therapy. On follow-up after 5 months, the patient presented with gross hematuria. Imaging studies revealed a mixed-density cystic lesion of 2.6 cm situated within the interpolar region of the right kidney. This cystic lesion exhibited intricate septations at the superior pole of the kidney. Robotic-assisted right partial nephrectomy was performed, and pathologic examination was diagnostic for LCKD. This report not only underscores the uniqueness of LCKD but also presents a comprehensive review of the existing literature that pertains to this condition. Particular emphasis is placed upon its inherent benign behavior and its marked divergence from the progressive trajectory commonly associated with other renal diseases. We also explored the incidental findings of the disease, its diverse clinical symptomatology, conceivable etiological underpinnings, and the array of diagnostic modalities used. Finally, similarities in histopathologic findings with polycystic kidney disease and other entities are discussed, underscoring the importance of accurate diagnosis and management.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

“…Regardless of age, the clinical symptoms range from asymptomatic to the presence of hematuria, flank pain, and palpable abdominal masses. 2 , 6 - 12 …”

Section: Discussionmentioning

confidence: 99%