1/231. Cystic partially differentiated nephroblastoma with skeletal muscle differentiation: a case report.Cystic partially differentiated nephroblastoma (CPDN) with skeletal muscle differentiation is reported for its rarity and its unique place in the spectrum from multilocular cyst to wilms tumor. Unlike wilms tumor CPDN does not metastasize and is cured by nephron sparing surgery.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/231. Bench surgery with autotransplantation for bilateral synchronous Wilms' tumor: a report of three cases.The surgical management of bilateral synchronous nephroblastoma remains controversial. The authors describe three cases treated using ex vivo tumor dissection followed by autotransplantation in an attempt to preserve functioning renal tissue. Two children are alive and tumor free with adequate renal function at 30 months and 3 years, respectively. One died from tumor recurrence with metastases 9 months after surgery. This technique is an acceptable alternative to bilateral nephrectomy followed by transplantation.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
3/231. diagnosis and referral of Wilms' tumor.Wilms' Tumor, also know as nephroblastoma, is a childhood renal tumor. The assessment and diagnosis of a Wilms' tumor is one of the many challenges faced in the primary care setting. This article presents a brief review of Wilms' tumor in the pediatric population and its occurrence in a case presentation.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
4/231. Cystic partially differentiated nephroblastoma in an adult: an immunohistochemical, lectin histochemical and ultrastructural study.AIMS: Cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal multicystic tumour, usually affecting early infants. To our knowledge, this report describes the first case of CPDN occurring in an adult. methods AND RESULTS: A 45-year-old man was found incidentally to have a left renal cystic tumour, measuring 20 mm in diameter, at the lower pole far from the pelvis. The tumour was composed of multilocular cystic spaces of variable size and intervening septa without solid nodular areas. The cysts were lined by a single layer of flattened, hobnail, or columnar epithelium. The septa were made of mesenchymal cells, which were admixed with small numbers of loosely aggregated blastemal cells, occasional tubular structures in various stages of development, and a few glomeruloid structures. The tumour cells had no anaplasia, and mitoses were rare. Immunohistochemical and lectin histochemical studies revealed that the cyst lining epithelium and the tubular structures in the septa expressed predominantly the markers for distal tubules and collecting ducts. Ultrastructurally, the cyst lining cells closely resembled collecting duct cells while some tubular structures showed an immature nephrogenic morphology. The patient was alive and well without evidence of recurrence 11 months after surgery. CONCLUSIONS: CPDN does occur in adults, as experienced in Wilms' tumour, though its incidence is extremely low. This study suggests that CPDN may show maturation intermediate between cystic nephroma and Wilms' tumour, even in adult cases.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
5/231. Extrarenal Wilms' tumor of the uterus.Wilms' tumor (nephroblastoma) is rare in adults. Its occurrence as a primary tumor outside the kidney is exceptional. Only 4 cases [1-4] have occurred in the uterus. This report adds the fifth instance of primary uterine Wilms' tumor. The patient, a 77-year-old woman (G2P2), is the oldest ever described with a Wilms' tumor.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
6/231. Spectrum of cystic variants of Wilm's tumour: cystic nephroma (multilocular cyst) and cystic partially differentiated nephroma--a report of four cases.Two cases of cystic nephroma (multilocular cyst of the kidney), and one case each of cystic partially differentiated nephroblastoma (CPDN) and rhabdomyomatous Wilms' tumour are described. All were male and in the pediatric age group. Grossly tumours were unilateral, unicentric and multiloculated. The need for proper designation of these lesions is highlighted because of difference in the treatment and prognosis of these tumours.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
7/231. Perlman syndrome: four additional cases and review.Perlman syndrome was first described in 1973 and comprises nephromegaly with renal dysplasia and wilms tumor, macrosomia, cryptorchidism, and multiple facial anomalies. polyhydramnios and hypoglycaemia are often found. Twelve children have been described from six different families. Five came from one family whose Yemenite Jewish parents were second cousins. Autosomal recessive inheritance has been suggested. prognosis is severe with neonatal death in most children. We report on 4 new cases of Perlman syndrome from 3 families; all parents were non-consanguineous. Some of the observed manifestations have been described only once in this syndrome (cardiac defect, hepatic fibrosis with portoportal bridging, haemangioma) or never before (volvulus, intestinal atresia, and agenesis of the corpus callosum in 1 patient, a cleft palate in another). All children died within the first year. The 2 sibs were born prematurely with nephromegaly but without hamartomas or nephroblastomatosis. This is consistent with the hypothesis that dysplastic medullary parenchyma in preterm infants develops into nephroblastomatosis and hamartoma and eventually wilms tumor.- - - - - - - - - - ranking = 0.4keywords = nephroblastoma (Clic here for more details about this article) |
8/231. Juxtaposed cystic nephroma and Wilms' tumor.We report a case of juxtaposed Wilms' tumor (WT) and cystic nephroma (CN) in a 21-month-old girl which gave rise to radiological diagnostic difficulty. Preoperative chemotherapy was given, resulting in marked tumor necrosis but the cystic nephroma remained untouched. Histological examination showed characteristic features of a triphasic WT and a CN; the two lesions were separated by a thick fibrous capsule. While everybody agrees that WT and cystic partially differentiated nephroblastoma (CPDN) are closely related, there are two opposite views about their relationship to CN. One is that CN may represent the final step in maturation of WT and CPDN. Other authors argue that there is no evidence to support this theory but believe CN might have something in common with nephrogenic rests. We suggest that the two lesions in the present case may have originated from two intralobar nephrogenic rests, which would strengthen the latter view.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
9/231. Wilms' tumor of the cervix: a case report and review of the literature.INTRODUCTION: Extrarenal Wilms' tumors are rare, with only 55 well-documented cases in the literature and only 4 confined to the uterus. A fifth case along with review of the current literature is presented. CASE: A 13-year-old girl presented with a 2-month history of irregular vaginal spotting. A 6.5 x 4.5 x 2.5-cm polypoid mass meeting criteria for an extrarenal wilms tumor was removed from the superior aspect of the cervix, but recurred 8 months later on the posterior lip. The patient underwent a Schauta procedure, lymph node dissection, and bilateral oophoropexy. The surgery was followed by typical renal Wilms' tumor chemotherapy. The patient is without evidence of disease 5 years after surgery. DISCUSSION: This case uniquely suggests that limited excision of the original tumor has inherent risks for relapse. Oophoropexy appears to be reasonable when fertility preservation is desired and reconfirms that treatment mimicking renal nephroblastomas offers good long-term survival.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
10/231. Partial nephrectomy in cystic partially differentiated nephroblastoma.Cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm in children. It is now recognized as a tumor with low but definite malignant potential. The authors report a patient that was treated with partial nephrectomy and chemotherapy with successful outcome. The literature on CPDN is briefly discussed.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
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