Cystic partially differentiated nephroblastoma in an adult: a case imitating the process of normal nephrogenesis along with corresponding WT1 expression.
نویسندگان
چکیده
Cystic partially differentiated nephroblastoma (CPDN) is extremely rare in adults. Only 2 cases have been documented in the English literature. Herein, we present a third case of CPDN with unique morphological and immunohistochemical features. A 45-year-old man had a multicystic right renal mass, with a maximum diameter of 3 cm on magnetic resonance imaging. Being unable to rule out malignancy, partial nephrectomy was performed. The surgically resected specimen contained a multicystic mass, 3 × 3 × 2.5 cm in size, without an expansile solid nodule. Histopathological examination revealed nephroblastomatous elements without identifiable blastema; transition from cap-mesenchyme-like cells to an immature glomerulus was observed and maturing tubules and a glomerulus were present. Despite the lack of a blastema, the diagnosis of CPDN was the most appropriate. Immunohistochemical WT1 expression imitated the pattern of ongoing normal nephrogenesis. Therefore, we believe that the blastema disappeared because of maturation.
منابع مشابه
Expression of the Wilms' tumour gene WT1 in the developing human and in paediatric renal tumours: an immunohistochemical study.
AIMS The Wilms' tumour gene (WT1) product is expressed during the development of the urogenital system. This study was undertaken to evaluate four anti-WT1 antibodies and use the most specific one to examine the expression of WT1 in formalin fixed, paraffin wax embedded tissues from human embryos, fetuses, and paediatric renal neoplasms. METHODS The antibodies were assessed on paraffin sectio...
متن کاملCystic partially differentiated nephroblastoma: A report of two cases with review of the literature
Cystic partially differentiated nephroblastoma is a rare tumor of the kidney usually affecting infants. It should be distinguished from Wilms’ tumor with multicystic areas and multilocular cystic nephroma. Multilocular cystic nephroma is a benign tumor whereas Wilms’ tumor with multicystic areas is at the malignant end of the range of classification of such tumours. Cystic partially differentia...
متن کاملYAC complementation shows a requirement for Wt1 in the development of epicardium, adrenal gland and throughout nephrogenesis.
The Wilms' Tumour gene WT1 has important functions during development. Knock-out mice were shown to have defects in the urogenital system and to die at embryonic day E13.5, probably due to heart failure. Using a lacZ reporter gene inserted into a YAC construct, we demonstrate that WT1 is expressed in the early proepicardium, the epicardium and the subepicardial mesenchymal cells (SEMC). Lack of...
متن کاملOver-expression of Wilm’s Tumor Gene 1 (WT1) in Iranian Patients with Acute Myeloblastic Leukemia
Background: The Wilm’s tumor gene 1 (WT1) encodes a zinc finger transcription factor that is inactivated in a subset of Wilm’s tumors. It plays a crucial role in growth, proliferation and development of some embryonic and adult organs. WT1 is expressed as a tumor associated antigen (TAA) in various types of solid and hematopoietic malignancies and can be employed as a useful marker for targeted...
متن کاملOsr1 Interacts Synergistically with Wt1 to Regulate Kidney Organogenesis
Renal hypoplasia is a common cause of pediatric renal failure and several adult-onset diseases. Recent studies have associated a variant of the OSR1 gene with reduction of newborn kidney size and function in heterozygotes and neonatal lethality with kidney defects in homozygotes. How OSR1 regulates kidney development and nephron endowment is not well understood, however. In this study, by using...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- International journal of clinical and experimental pathology
دوره 8 1 شماره
صفحات -
تاریخ انتشار 2015