@entity1824 (so-called epithelioid XXXX ) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification.

@entity1 @entity1824 @entity39 @entity5 @entity189 @entity2916 @entity3 @entity359 @entity204
@entity359 ( @entity1824 ) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to @entity359 . Although approximately 120 cases are published, mostly as case reports with variably used diagnostic criteria, the pathologic prognostic predictors of outcome are unknown. We analyzed the clinicopathologic parameters in a large series of 41 cases of pure @entity359 of the kidney, which we designate as pure (monotypic) epithelioid @entity1824 to contrast them from classic @entity359 that are regarded by some as @entity1824 . We use the terminology "pure" to separate these cases from those that may have variable epithelioid components. The mean age of the @entity1 was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of @entity1 were symptomatic at presentation with metastatic disease at onset in 12 cases. Follow-up and/or disease progression information were available for 33 of 41 cases (mean, 44.5 mo and median, 24.5 mo; range, 4 to 240); 9 @entity1 had a history of associated @entity2916 . Recurrence and @entity3 were seen in 17% and 49% of @entity1 ; 33% of @entity1 died of disease. Lymph node involvement was seen in 24% of @entity1 ; the liver (63%), lung (25%), and mesentery (18.8%) were the most common metastatic sites. Clinicopathologic parameters associated with disease progression (recurrence, @entity3 , or @entity204 due to disease) in univariate analysis included associated @entity2916 complex or concurrent @entity359 (any @entity3 , P=0.046), @entity39 ( @entity3 at diagnosis, P=0.012), @entity5 size >7 cm (progression, P=0.021), extrarenal extension and/or renal vein involvement (progression, P=0.023), and @entity189 -like growth pattern (progression, P=0.040) (the 5 adverse prognostic parameters for @entity1824 ). @entity5 with <2 adverse prognostic parameters (13 cases) were considered to be low risk for progression @entity5 , with 15% having disease progression. @entity5 with 2 to 3 adverse prognostic parameters (14 cases) were considered to be "intermediate risk," with 64% having disease progression. @entity5 with more than 4 or more adverse prognostic parameters (6 cases) were considered to be high risk, with all @entity1 having disease progression. Of @entity5 with 3 or more adverse prognostic parameters, 80% had disease progression. An exact logistic regression analytic model showed that only @entity189 -like growth pattern and extrarenal extension and/or renal vein involvement were significant predictors of outcome (P=0.009 and 0.033, respectively). Our data of a large series with uniform definitional criteria confirm the malignant potential for @entity1824 and provide adverse prognostic parameters for risk stratification in these @entity1 .
