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Kidney tumor - cysts, children, adult benign

Cystic non-neoplastic lesions

Medullary sponge kidney


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 29 December 2011, last major update December 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
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● Sporadic cystic disease characterized by bilateral cystic dilations of medullary collecting ducts; normal cortex

Epidemiology
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● 1 per 5000 births; no gender preference; not familial
● Associated with hemihypertrophy of body (25% of cases), Marfanís, Caroliís and Ehlers-Danlos

Clinical features
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● Usually presents in adulthood
● Patients are usually asymptomatic with normal renal function
● Calcifications on Xray, stones, hematuria and infection at age 30+ years
● Does not progress to end stage renal disease
● Diagnosed with intravenous pyelography

Treatment
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● Cranberry juice to maintain urinary acidity
● Nephrectomy is NOT recommended

Gross description
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● Normal sized kidneys with multiple, small cysts in medullary pyramids and papillae, giving medulla a sponge-like appearance
● Most often bilateral

Gross images
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Cysts of inner medullary and papillary region

Extensive cysts communicating with each other

Papillae are interspersed due to variably sized cysts

Small cysts with stones, papillae on right is not affected

Numerous medullary cysts and isolated cortical cyst

Cysts at corticomedullary junction

Micro description
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● Medullary cysts lined by cuboidal epithelium or urothelium
● May have concretions adherent to cyst wall
● Often severe inflammation and scarring in interstitium, often with tubular atrophy near papillary tips

Micro images
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Cysts in papilla

With microlithiasis and chronic pyelonephritis

Cortical cyst, multiple cysts at the corticomedullary junction and atypically located papillary cyst

Additional references
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eMedicine #1; #2

End of Kidney tumor - cysts, children, adult benign > Cystic non-neoplastic lesions > Medullary sponge kidney


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