Medullary Sponge Kidney Associated With Multivessel Fibromuscular Dysplasia: Report of a Case With Renovascular Hypertension

Bisceglia, Michele; Galliani, Carlos

doi:10.1177/1066896907307237

Cited by 7 publications

(1 citation statement)

References 14 publications

(24 reference statements)

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“…Medullary Sponge Kidney (MSK) 1,8,9 MSK is a nonhereditary disease-usually asymptomatic-characterized by the presence of ectasia of papillary collecting ducts in the renal medulla, congregating at the papillary tips, and involving one to all of pyramids (cystic disease of renal pyramids). Papillary duct ectasia is likely a congenital anomaly usually undetected, until complications supervene.…”

Section: Multicystic Dysplastic Kidney (Mcdk)mentioning

confidence: 99%

Selected Case From the Arkadi M. Rywlin International Pathology Slide Series

Galliani

Gómez²,

Panniello³

et al. 2015

Advances in Anatomic Pathology

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A Hispanic newborn male, the product of nonconsanguineous parents, exhibited major and minor signs of tuberous sclerosis complex (TSC). MRI of the abdomen disclosed a discrete unilateral, cystic, right upper pole renal mass that prompted a nephrectomy. Histologic examination showed a polycystic renal mass that involved all segments of the nephron, with a preponderantly glomerulocystic pattern. The cysts were rounded, uniform, and small, most measuring 2 to 3 mm in diameter. The lining of the cysts was hyperplastic, made up of tall epithelial cells with eosinophilic granular cytoplasm and large nuclei, and focally formed mounds and papillary tufts. DNA analysis detected a constitutional deletion of exon 1 in the TSC2 gene on chromosome 16p13.3. Cystogenesis in TSC2 is manifested because of alteration or dysfunction of the primary cilium, where polycystin, the gene product of PKD1 gene, is localized. Renal cysts are often seen in TSC, varying in number from a few to innumerable, involving all segments of the nephron, including Bowman spaces, and are currently considered as one of the minor diagnostic features. A glomerulocystic pattern is a rare form of kidney involvement in TSC that aptly describes the innumerable cystically dilated Bowman spaces. Glomerulocystic kidney associated with the aforementioned hyperplastic epithelial lining (TSC epithelium) is sufficiently characteristic that could conceivably serve as a major TSC feature in the future.

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Section: Multicystic Dysplastic Kidney (Mcdk)mentioning

confidence: 99%