Mixed epithelial and stromal tumor of the kidney in a 12-year-old girl.

Abstract:

:Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare kidney neoplasm that almost exclusively occurs in perimenopausal women, and long-term estrogen replacement is relevant to its pathogenesis. Herein is described an atypical case of MESTK uncovered in a 12-year-old premenarcheal girl without a history of prior estrogen use. On surgical specimen it was found that the well-circumscribed tumor measuring 14 cm arose from the lower pole of the right kidney, showing solid and fibrous-cystic areas. Microscopically, it was composed both of epithelial structures similar to renal tubules and stroma comprising non-specific spindle cells. Some intratumoral tubules showed affinities to distal-nephron-specific lectins, and those immunoreactive for proximal-tubule-specific CD15 were also present. In addition, primitive ductal structures were reactive both for CD15 and lectins, but immature epithelial elements typical of nephroblastoma were absent. Spindle cells were positive for actin, desmin and vimentin, and expressed progesterone and estrogen receptors. The tumor was comparable with MESTK, although some epithelia were associated with the immunophenotype of proximal tubules. The patient was free of disease postoperatively for 40 months. In the present case, remnants of the primitive periductal mesenchyme might be promoted to neoplastic cells by a sex-steroid surge during puberty.

journal_name

Pathol Int

journal_title

Pathology international

authors

Hara N,Kawaguchi M,Murayama S,Maruyama R,Tanikawa T,Takahashi K

doi

10.1111/j.1440-1827.2005.01888.x

subject

Has Abstract

pub_date

2005-10-01 00:00:00

pages

670-6

issue

10

eissn

1320-5463

issn

1440-1827

pii

PIN1888

journal_volume

55

pub_type

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