ABSTRACT
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney.
Subject(s)
Biomarkers, Tumor/biosynthesis , Carcinoma, Small Cell/diagnosis , Kidney Neoplasms/diagnosis , Oncogene Proteins, Fusion/biosynthesis , Wilms Tumor/diagnosis , Biomarkers, Tumor/genetics , Carcinoma, Small Cell/genetics , Carcinoma, Small Cell/pathology , Carcinoma, Small Cell/physiopathology , Child , Chromosomes, Human, Pair 11 , Chromosomes, Human, Pair 22 , Diagnosis, Differential , Humans , Immunohistochemistry , Kidney/metabolism , Kidney/pathology , Kidney Neoplasms/genetics , Kidney Neoplasms/pathology , Kidney Neoplasms/physiopathology , Male , Oncogene Proteins, Fusion/genetics , Wilms Tumor/genetics , Wilms Tumor/pathology , Wilms Tumor/physiopathologyABSTRACT
OBJECTIVE: To evaluate the prevalence of cardiovascular events (CVE) secondary to atherosclerosis in lupus patients and correlate them to the traditional risk factors, disease duration and drug therapy used. METHODS: A retrospective study was carried out based on data obtained from patients charts. Patients included were those who had a lupus diagnosis confirmed at least two years before inclusion in the study and had been followed since 1992. CVE were characterized as MI, angina pectoris and stroke non-related to lupus activity. Risk factors and drugs used for treatment were recorded. RESULTS: Seventy-one charts were analyzed. Patients mean age was 34.2+/-12.7 years; 68 were women and three were men; 58 were Caucasian (81.6%). Ten (14.08%) presented CVE. Patients in whom CVE were observed were older (42.7 vs. 32.8 years p=0.0021) and presented longer disease duration (10.8 vs. 7.2 years p=0.011). The traditional risk factors, daily and cumulative doses of steroids, immunosuppressive drugs and antimalarial drugs were not significant when patients with and without CVE were compared. CONCLUSION: The prevalence of CVE secondary to atherosclerosis in systemic lupus erythematosus (SLE) was 14.08%. The traditional risk factors were not associated with the development of CVE in lupus patients. Patients that presented cardiovascular events were older and presented longer disease duration. It is a premature conclusion to establish SLE as an independent risk factor for atherosclerosis development.
