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Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and
Genetically Distinct From Adult Cystic Nephroma
#MMPMID28177962
Li Y
; Pawel BR
; Hill DA
; Epstein JI
; Argani P
Am J Surg Pathol
2017[Apr]; 41
(4
): 472-481
PMID28177962
show ga
The term cystic nephroma has traditionally been used to refer to 2 neoplasms, a
lesion in adults that is now thought to be part of the spectrum of mixed
epithelial stromal tumor (MEST) and a pediatric lesion that has been associated
with mutations in the DICER1 gene. A direct detailed morphologic,
immunohistochemical, and genetic comparison of these 2 lesions has not been
performed. In this study, we compare the morphologic features, immunoreactivity
for estrogen receptor and inhibin, and DICER1 genetic status of 12 adult cystic
nephroma/MEST (median age 50.5 y, all females) and 7 pediatric cystic nephroma
(median age 1.3 y, male:female=6:1). Both lesions (11 of 12 adult cases, 6 of 7
pediatric cases) frequently demonstrated subepithelial accentuation of stromal
cellularity, though the increased cellularity frequently included inflammatory
cells in the pediatric cases. All adult and pediatric cases labeled for estrogen
receptor; however, whereas most (83%) of adult cases labeled for inhibin at least
focally, no pediatric case labeled for inhibin. Most adult cases (58%)
demonstrated wavy, ropy collagen in association with cellular stroma, whereas
this was not found in pediatric cases. 86% of pediatric cases demonstrated DICER1
mutations, whereas only 1 of 10 adult cases demonstrated a DICER1 mutation. In
summary, although cellular stroma and estrogen receptor immunoreactivity are
commonly present in both adult and pediatric cystic nephroma, ropy collagen and
inhibin immunoreactivity are far more common in adult cystic nephroma/MEST,
whereas DICER1 mutations are far more prevalent in pediatric cystic nephroma.
These results support the current World Health Organization Classification's
separation of adult and pediatric cystic nephromas as distinct entities.
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