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Biopsy proven medullary sponge kidney: clinical findings, histopathology, and
role of osteogenesis in stone and plaque formation
#MMPMID25615853
Evan AP
; Worcester EM
; Williams JC Jr
; Sommer AJ
; Lingeman JE
; Phillips CL
; Coe FL
Anat Rec (Hoboken)
2015[May]; 298
(5
): 865-77
PMID25615853
show ga
Medullary sponge kidney (MSK) is associated with recurrent stone formation, but
the clinical phenotype is unclear because patients with other disorders may be
incorrectly labeled MSK. We studied 12 patients with histologic findings
pathognomonic of MSK. All patients had an endoscopically recognizable pattern of
papillary malformation, which may be segmental or diffuse. Affected papillae are
enlarged and billowy, due to markedly enlarged inner medullary collecting ducts
(IMCD), which contain small, mobile ductal stones. Patients had frequent dilation
of Bellini ducts, with occasional mineral plugs. Stones may form over white
(Randall's) plaque, but most renal pelvic stones are not attached, and have a
similar morphology as ductal stones, which are a mixture of calcium oxalate and
apatite. Patients had no abnormalities of urinary acidification or acid
excretion; the most frequent metabolic abnormality was idiopathic hypercalciuria.
Although both Runx2 and Osterix are expressed in papillae of MSK patients, no
mineral deposition was seen at the sites of gene expression, arguing against a
role of these genes in this process. Similar studies in idiopathic calcium stone
formers showed no expression of these genes at sites of Randall's plaque. The
most likely mechanism for stone formation in MSK appears to be crystallization
due to urinary stasis in dilated IMCD with subsequent passage of ductal stones
into the renal pelvis where they may serve as nuclei for stone formation.
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