Classical renal angiomyolipoma (AML) is a common tumor that, in most cases, follows a benign course and has clearly defined radiologic and histological characteristics. However, rare cases of clinically aggressive or malignant AML, the epithelioid variant of AML (EAML), have been reported. Here, we review the five cases of EAML diagnosed since 2000 at our institution to highlight the spectrum of clinical, radiological and histological characteristics. In all cases, renal lesions seen on computed tomography (CT) were considered as suspicious for renal cell carcinoma (RCC) without tumor extension or metastasis. One of the two patients with definitive tuberous sclerosis complex had a small conventional AML with fat content in the other kidney. Histologically, these tumors can resemble sarcomatoid RCC or high grade RCC. The final diagnosis is established by the presence of perivascular epithelioid cells and immunohistochemistry markers. There was no evidence of local recurrence or metastatic disease found by sonography, CT and magnetic resonance imaging during the follow-up period. EAMLs are capable of aggressive or malignant clinical behavior. Approximately one third of the reported EAMLs show advanced disease; therefore, we recommend that it is important to treat and closely follow-up such cases, similar to that for RCC, because of its malignant potential.
BackgroundFew studies have reported the association between lifestyle factors and prognosis of esophageal squamous cell carcinoma (ESCC) and among these, the effects of habitual areca nut chewing have never been examined.Methodology/Principal FindingsData from 718 pathology-proven ESCC patients recruited in a multicenter hospital-based case-control study between 2000 and 2008 in Taiwan were analyzed. Clinical and lifestyle information were obtained by chart review and questionnaire survey. Death was confirmed using the National Death Index. The mean age at diagnosis was 59.8 years and 506 (70.5%) patients presented with stage III or IV diseases. The overall 1- and 5-year survival rates were 41.8% and 9.75% respectively. In addition to clinical stage, habitual alcohol drinking was found to be the strongest predictor for ESCC survival, followed by areca chewing and smoking. Compared with non-users, patients who regularly used all three substances (alcohol, areca nut, and cigarette) had 1.52 times the risk of early death (adjusted hazard ratio = 1.52, 95% CI = 1.02–2.27, p = 0.04). In addition, the more the number of substances used, the worse the prognosis of ESCC (adjusted p for trend = 0.01).Conclusions/SignificanceOur study found that indulgence in more substances is a significant predictor of ESCC survival. Further mechanistic studies are necessary to elucidate how these substances lead to an adverse outcome.
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