Epithelioid angiomyolipomas (perivascular epithelioid cell tumors) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to classic angiomyolipoma. 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 epithelioid angiomyolipomas of the kidney, which we designate as pure (monotypic) epithelioid PEComas to contrast them from classic angiomyolipomas that are regarded by some as PEComas. We use the terminology "pure" to separate these cases from those that may have variable epithelioid components. The mean age of the patients was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of patients 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 patients had a history of associated tuberous sclerosis. Recurrence and metastasis were seen in 17% and 49% of patients; 33% of patients died of disease. Lymph node involvement was seen in 24% of patients; the liver (63%), lung (25%), and mesentery (18.8%) were the most common metastatic sites. Clinicopathologic parameters associated with disease progression (recurrence, metastasis, or death due to disease) in univariate analysis included associated tuberous sclerosis complex or concurrent angiomyolipoma (any metastasis, P=0.046), necrosis (metastasis at diagnosis, P=0.012), tumor size >7 cm (progression, P=0.021), extrarenal extension and/or renal vein involvement (progression, P=0.023), and carcinoma-like growth pattern (progression, P=0.040) (the 5 adverse prognostic parameters for pure epithelioid PEComas). Tumors with <2 adverse prognostic parameters (13 cases) were considered to be low risk for progression tumor, with 15% having disease progression. Tumors with 2 to 3 adverse prognostic parameters (14 cases) were considered to be "intermediate risk," with 64% having disease progression. Tumors with more than 4 or more adverse prognostic parameters (6 cases) were considered to be high risk, with all patients having disease progression. Of tumors with 3 or more adverse prognostic parameters, 80% had disease progression. An exact logistic regression analytic model showed that only carcinoma-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 pure epithelioid PEComas and provide adverse prognostic parameters for risk stratification in these patients.
Sir, We report a case of a mixed epithelial and stromal tumor of the kidney with malignant transformation of its mesenchymal component. A 46-year-old woman was operated on for a computed tomography-proven solidified cyst in the upper pole of the left kidney. No tumorous metastatic dissemination was found during further clinical examination.The kidney contained a cystic tumor 7 cm in diameter bulging into the perirenal fat. The gray-brown wall of the cyst was 0.2-to 0.3-cm thick, the inner surface was smooth, covered by light and dark brown, hemorrhagic material. A flat, red-brown prominence measuring 4×3×1.5 cm, multilocular on a cut surface, protruded into the intracystic space. The margins of the tumor were sharply demarcated from the renal parenchyma but were indistinct on the side of perirenal fat. The hemorrhagic contents of the cyst, submitted separately in a total volume of about 200 ml, were characteristic of blood clots, seemingly free of tumorous material. The renal parenchyma outside the tumor area, renal pelvis and ureter were unremarkable.The tumor showed a histological structure of a benign multilocular cyst containing cysts and septa, which comprised occasional small tubular structures. Septa were formed by fibrous stroma with uneven cellularity focally displaying ovarian stroma-like features (Fig. 1) and immunohistochemically showing positivity of vimentin, smooth muscle actin, muscle actin and desmin. Ultrastructurally, the bland-looking cells surrounded by external lamina and connected by primitive attachment sites were set in the background of collagenous matrix. They contained numerous organelles, including copious cisternae of the rough and smooth endoplasmic reticulum, intermediate filaments and Golgi complex. Numerous patches of microfilaments were focally present in the cytoplasm of the benign stromal cells. The tubules and cysts were lined with a single layer of epithelial cells, mostly showing a hobnail appearance, oxyphilic, periodic acid-Schiff base (PAS)-negative cytoplasm and large, irregular nuclei. PAS-positive material was present in some lumina. Immunohistochemically, the cells were cytokeratin and epithelial membrane antigen (EMA) positive. No heterologous tissue, renal blastema cells or poorly differentiated epithelial structures were found.
HPV has carcinogenic effects at several anatomical sites in women and men. Whether the presence of HPV in the genitourinary tract of men is associated with a higher prostate cancer risk has been a matter of research for a long-time and the results are still not fully conclusive. Similarly, the question of the reservoir of HPV infection in men is not clearly resolved. HPV DNA presence and types were evaluated by means of polymerase chain reaction in the tissue of 146 patients with benign prostate hyperplasia and prostate cancer. HPV-specific antibodies were analyzed by enzyme-linked immunosorbent assay in the sera of all patients and 172 controls. In addition, 256 biopsies taken from non-tumorous tissues were analyzed. No statistically significant differences were observed in HPV DNA prevalence between patients with benign prostate hyperplasia (2%) and patients with prostatic cancer (2%; P = 1.000). The seropositivity rates did not differ significantly between groups of subjects except for antibodies against HPV 6 VLPs which were found more often in prostate cancer patients (adjusted P = 0.018). Similarly, no difference in the seroprevalence rates for HPV 16 E6 and/or E7 oncoproteins between groups of patients and healthy controls was detected. The overall HPV prevalence in 256 healthy tissue samples was 4%. The results indicate that HPV infection is not associated with prostate oncogenesis in men. However, they imply that multiple tissues of the male genitourinary tract may be important reservoirs for the transmission of some HPV types.
Colonic inflammation induces local glucocorticoid activation via 11betaHSD1 and impairs glucocorticoid inactivation via 11betaHSD2. The observed changes indicate a role for local metabolism of glucocorticoids in the control of colonic inflammation.
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