1/4. Parotid carcinoma after autologous bone marrow transplantation for relapsed nephroblastoma.Abdominal irradiation, especially if associated with doxorubicin administration, increases the risk of a secondary malignant neoplasm (SMN) after treatment of nephroblastoma. Secondary malignant salivary tumors are rare and usually occur in patients with previous cranial irradiation. The authors describe the case of a parotid mucoepidermoid carcinoma arising 13 years after diagnosis of nephroblastoma. This patient showed no characteristics reported in the literature as statistically significant for the development of an SMN. The authors believe that long-term, regular clinical examination is necessary even in patients at low risk of developing an SMN.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/4. Hepatic rhabdomyomatous tumor: late sequel of a fetal rhabdomyomatous nephroblastoma.A mesenchymal tumor with the macroscopic and microscopic features of a fetal rhabdomyoma arose in the liver of a 14-year-old boy. Thirteen years previously this boy had been treated for a fetal rhabdomyomatous nephroblastoma with nephrectomy and--for subsequent peritoneal disseminations--with surgical excision, radiotherapy, and chemotherapy. The unusual hepatic location of the rhabdomyomatous tumor in this patient supports the view that this mature tumor developed from a metastasis of the original nephroblastoma. As such, this case may represent an example of irreversible change of a malignant process into a benign tumor probably caused by the action of systemic chemotherapy.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
3/4. 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 = 1.1666666666667keywords = nephroblastoma (Clic here for more details about this article) |
4/4. Extrarenal Wilms tumour.INTRODUCTION: Nephroblastoma is one of the most common solid tumours in children. It also is the most frequent tumour found in the kidneys. In 5 % of cases it affects both kidneys at the same time. About 70 - 80 new cases of Wilms tumour are registered in poland annually, usually in patients aged from 1 to 7 years. Extrarenal Wilms tumours are extremely rare. Due to its rarity, series with more cases are based upon material collected from many clinical centers. AIM: We would like to present a case of a boy in whom we diagnosed nephroblastoma in the retroperitoneal space 14 years after he had completed a complex therapy for bilateral Wilms tumour. CONCLUSION: The development of an extrarenal tumour 14 years after complex treatment for bilateral nephroblastoma is related to the survival of metanephros located outside the kidney.- - - - - - - - - - ranking = 0.33333333333333keywords = nephroblastoma (Clic here for more details about this article) |