TY - JOUR
T1 - Nested (ossifying) stromal epithelial tumor of the liver
T2 - Case report
AU - Meir, Karen
AU - Maly, Alexander
AU - Doviner, Victoria
AU - Gross, Eitan
AU - Weintraub, Michael
AU - Rabin, Lionel
AU - Pappo, Orit
PY - 2009/5
Y1 - 2009/5
N2 - Nested stromal-epithelial tumor (NSET) of the liver is an extremely rare primary hepatic tumor with uncertain malignant potential. To date, only 11 cases have been described. We describe the case of a 21/2-year-old girl with an incidental liver mass. The mass was discovered on follow-up abdominal imaging for asymptomatic hydronephrosis diagnosed on antenatal ultrasound. Needle biopsy showed a mixed stromal and epithelial process in a nested pattern, with foci of ossification and no significant pleomorphism or necrosis. The nest cells stained with WT-1, cytokeratin 18, and CD56. Ossifying stromal epithelial tumor of the liver was strongly suspected. The findings were confirmed in the subsequent partial hepatectomy specimen. To our knowledge, this is the 12th case of NSET in the English-language literature and the 3rd case of NSET associated with genitourinary system abnormalities. Possible associations with dysregulated WT-1 expression are discussed.
AB - Nested stromal-epithelial tumor (NSET) of the liver is an extremely rare primary hepatic tumor with uncertain malignant potential. To date, only 11 cases have been described. We describe the case of a 21/2-year-old girl with an incidental liver mass. The mass was discovered on follow-up abdominal imaging for asymptomatic hydronephrosis diagnosed on antenatal ultrasound. Needle biopsy showed a mixed stromal and epithelial process in a nested pattern, with foci of ossification and no significant pleomorphism or necrosis. The nest cells stained with WT-1, cytokeratin 18, and CD56. Ossifying stromal epithelial tumor of the liver was strongly suspected. The findings were confirmed in the subsequent partial hepatectomy specimen. To our knowledge, this is the 12th case of NSET in the English-language literature and the 3rd case of NSET associated with genitourinary system abnormalities. Possible associations with dysregulated WT-1 expression are discussed.
KW - Genitourinary development
KW - Liver tumor
KW - WT-1
UR - http://www.scopus.com/inward/record.url?scp=66749114892&partnerID=8YFLogxK
U2 - 10.2350/08-03-0437.1
DO - 10.2350/08-03-0437.1
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C2 - 18699697
AN - SCOPUS:66749114892
SN - 1093-5266
VL - 12
SP - 233
EP - 236
JO - Pediatric and Developmental Pathology
JF - Pediatric and Developmental Pathology
IS - 3
ER -
