1/17. Teratoid Wilms' tumor: a case report.Teratoid Wilms' tumor is rarely seen and is a description used only recently. The term describes classical nephroma with a diversity of cell types and tissues. In this reported case, the epithelial component consisting of squamous areas made up 70 percent of the tumor; no criteria of dysplasia nor any nephroblastomatosis areas or endodermal elements were presented. Although it is reported that teratoid Wilms' tumor is not usually aggressive or metastatic, a case of unilateral teratoid Wilms' tumor in a 2.5-year-old-boy who died because of metastatic disease is presented and the literature reviewed.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/17. Teratoid wilms tumor in childhood: CT and ultrasonographic appearances.Teratoid wilms tumor is defined as an unusual variant of nephroblastoma, in which there is a significant diversity of cell types and tissues in a neoplasm, where areas of classic nephroblastoma tissue are also present. We report a case of teratoid wilms tumor demonstrated as a unilateral cystic and solid renal mass containing fatty tissue on ultrasonography and computed tomography.- - - - - - - - - - ranking = 2keywords = nephroblastoma (Clic here for more details about this article) |
3/17. A cystic partially differentiated nephroblastoma producing alpha-fetoprotein.A case of cystic partially differentiated nephroblastoma with immunohistochemical and serological demonstration of alpha-fetoprotein (AFP) production is described. To our knowledge, this is the first such case reported. The morphological heterogeneity of this rare tumor led us to describe the criteria of its nosological classification and of its differential diagnosis with intrarenal teratoma. AFP, besides being a marker of germline-derived tumor, was associated to this rare variant of Wilm's tumor. Although the tumor did not show regression either on clinical or pathologic assessments, serum AFP levels decreased after preoperative chemotherapy and returned to normal limits after nephrectomy.- - - - - - - - - - ranking = 5keywords = nephroblastoma (Clic here for more details about this article) |
4/17. Nephroblastoma arising in a germ cell tumor of testicular origin.We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man. orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma. Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy. Excision demonstrated metastatic teratoma at both sites. No recurrence was noted with 21 months of additional follow-up. Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites. Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors. loss of heterozygosity was found in 4 dna loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component. loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor. loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors. These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.- - - - - - - - - - ranking = 7keywords = nephroblastoma (Clic here for more details about this article) |
5/17. Currarino syndrome: proposal of a diagnostic and therapeutic protocol.BACKGROUND/PURPOSE: The Currarino syndrome (CS) is a peculiar form of caudal regression syndrome (CRS) characterized by the association of hemisacrum, anorectal malformation (arm), and presacral mass. The authors analyzed retrospectively their series, and they propose a multidisciplinary diagnostic and therapuetic protocol that until now has not been introduced. methods: A series of 6 patients with CS is presented. Five of them were treated initially in other centers. None of them had an early diagnosis. All presented associated anomalies; in 50%, Hirschsprung's disease (HD) and other dysganglionoses were present. One patient died of a presacral ectopic nephroblastoma. RESULTS: Depending on the expressivity, 3 types of CS can be identified, complete, mild, and minimal. Dysganglionoses and HD can be considered part of CS. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of CS families, magnetic resonance (MR) evaluation of patient spine and pelvis, suction rectal biopsies, and search for associated anomalies. CONCLUSIONS: This protocol could give a valid contribution to the treatment of CS, allowing an early diagnosis and proposing a rational timing of multidisciplinary surgical procedures. early diagnosis and treatment are essential to avoid morbidity and mortality from an undiagnosed presacral mass.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
6/17. Teratoid Wilms' tumor: report of a unilateral case.A unilateral teratoid Wilms' tumor was removed 2.5 weeks after the institution of chemotherapy. Teratoid Wilms' tumor is an extremely rare renal tumor, and only four cases, all bilateral, have been reported. Because of the finding of deep cortical intralobar nephroblastomatosis, strongly associated with bilateral Wilms' tumors, the patient has been closely followed since surgery without evidence of tumor in the remaining kidney at 2 years.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
7/17. A case of retroperitoneal teratoma with nephroblastoma.We present a case of retroperitoneal teratoma in a 4-year-old girl in which a Wilms' tumor-like element was predominant, unlike the usual pattern of the immature or malignant teratoma. Mature elements were composed of adipose tissue, neural plexus and ganglia, cartilage, smooth and skeletal muscles, and glandular epithelium of the respiratory and gastrointestinal types. Three months after complete excision of the mass, a recurrent tumor developed. It consisted of only nephroblastomatous elements without teratomatous components. Theories for the histogenesis of this rare tumor are discussed.- - - - - - - - - - ranking = 5keywords = nephroblastoma (Clic here for more details about this article) |
8/17. Teratoid Wilms' tumor: a case report with literature review.Teratoid Wilms' tumor is an unusual histologic variant of nephroblastoma in which the heterologous tissue predominates. The treatment for this tumor has not been established because of its rarity and varying tumor components. The authors report a case of localized teratoid Wilms' tumor in a 4-month-old male infant successfully treated with nephrectomy only. The tumor originated from the right kidney with multiple cysts and calcifications; renal teratoma was suspected preoperatively, and the operation was performed. The tumor was excised completely, and the specimen weighed 340 g. Histopathologic examination showed that various teratoid elements occupied most of the tumor with sparse islands of triphasic nephroblastoma, which was consistent with teratoid Wilms' tumor. Anaplastic elements, tumor capsule invasion, renal sinus soft tissue invasion, or tumors in intrarenal vessels were not observed. The patient was disease free for 3 years after surgery without receiving chemotherapy. We selected this therapeutic approach because of the chemoresistance of this tumor and the excellent prognosis in small (<550 g) stage I/favorable-histology classical Wilms' tumor diagnosed at less than 24 months of age. Therefore, nephrectomy alone may be an acceptable treatment of this stage of tumor in patients with teratoid Wilms' tumor.- - - - - - - - - - ranking = 2keywords = nephroblastoma (Clic here for more details about this article) |
9/17. Retroperitoneal teratoma with predominance of nephroblastic elements--a case report.The morphological features of a retroperitoneal teratoma in a 10-month-old girl are reported. Unlike the usual pattern of the teratoma, this tumor was composed predominantly of nephroblastomatous tissue. Histologically, glomeruloid and tubular structures were identified in nests of undifferentiated blastemal elements. hyaline cartilage, adipose tissue, glial tissue and glands lined by mucin-secreting columnar epithelium were minor elements. A focal cystic structure lined by thin flattened epithelium was also noted. Retroperitoneal teratoma with predominance of nephroblastic elements is of interest not only because of its rarity but also because it needs to be differentiated from extrarenal Wilms' tumor, since both of these tumors have different origins.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
10/17. Fatty Wilms' tumor simulating teratoma; occurrence in a child with horseshoe kidney.An unusually fatty wilms' tumor occurred in a young girl. Because of the abundant fat it radiologically simulated a teratoma.- - - - - - - - - - ranking = 0.019625028025278keywords = wilms (Clic here for more details about this article) |
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