1/159. 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 = tumour (Clic here for more details about this article) |
2/159. 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 = 1.1666666666667keywords = tumour (Clic here for more details about this article) |
3/159. Recurrent Wilms' tumour or retroperitoneal teratoma? A challenging case.The clinical and pathological features of a left renal tumour and a subsequent retroperitoneal tumour in a 2-year-old boy are presented. The nephrectomy specimen showed typical features of a triphasic Wilms' tumour with focal heterologous elements and intralobar nephrogenic rests. The tumour was assessed as clinical stage III and post-operative chemotherapy and radiotherapy were administered. A retroperitoneal mass, detected following completion of postoperative therapy, was mainly made up of skeletal muscle and mature adipose tissue. Nests of epithelium resembling ameloblastic nests and a unique structure reminiscent of a developing tooth were present.- - - - - - - - - - ranking = 1.3333333333333keywords = tumour (Clic here for more details about this article) |
4/159. Bilateral renal cancer in children: a difficult, challenging and changing management problem.Bilateral disease occurs in 6% of patients with Wilms' tumour. Bilateral renal involvement is present in 25%-50% of children with B-cell non-Hodgkin's lymphoma. Paediatric oncologists therefore encounter bilateral renal disease relatively frequently. A patient with bilateral Wilms' tumour is presented, in whom at least 16 synchronous tumours in the kidneys were treated successfully by primary chemotherapy and 'nephron-sparing' surgery, without renal radiotherapy. We believe the successful treatment without radiotherapy will allow greater potential for normal growth in the future. The case history of a patient with renal failure due to the infiltration of both kidneys by lymphoma and who was treated successfully by chemotherapy, is also presented. One kidney has completely returned to normal function and growth; the other has completely failed and almost disappeared, demonstrating complete reversibility of the damage up to some critical point, beyond which, failure and atrophy result. These two case histories demonstrate extreme examples of clinical dilemmas involving bilateral renal tumours and allow discussion of modern management aimed at preserving renal function.- - - - - - - - - - ranking = 0.66666666666667keywords = tumour (Clic here for more details about this article) |
5/159. A malignant rhabdoid tumor of the kidney occurring concurrently with a brain tumor: report of a case.Malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms to occur in young infants. Cases of MRTK accompanying an embryonal tumor in the central nervous system have occasionally been described. We present herein an interesting case of MRTK that was clinically diagnosed preoperatively. A male infant aged 6 months with both a midline brain tumor and a renal neoplasm was transferred to our institution. Although roentgenographic evaluation suggested that the renal lesion was a Wilms' tumor, midkine (MK), a growth and differentiation factor characteristically present in the urine of patients with Wilms' tumor, was not detected. A preoperative diagnosis of MRTK was established based on the lack of urinary MK in addition to the typical clinical features of the young age and the concurrent brain tumor.- - - - - - - - - - ranking = 0.086615037594323keywords = rhabdoid (Clic here for more details about this article) |
6/159. Primary lumbosacral Wilms tumour associated with occult spinal dysraphism.A 4-year-old child presenting with sudden-onset paraplegia and a sacral tumour in association with spina bifida occulta is reported. There were no stigmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tumour with extradural extension up to T10 and spinal dysraphism. The histological features of the extradural and sacral components of the tumour were consistent with a Wilms tumour. The differential diagnosis included a primary sacral teratoma containing Wilms tumour elements or a primary extrarenal Wilms tumour arising in association with a spinal dysraphism. There was no clinical response to chemotherapy or radiotherapy.- - - - - - - - - - ranking = 1.6666666666667keywords = tumour (Clic here for more details about this article) |
7/159. Lumbosacral extrarenal Wilms' tumour: a case report and literature review.Occurrence of extrarenal Wilms' tumour (WT) is very exceptional and the diagnosis is almost always made after surgical intervention. The tumour can be located in the retroperitoneum, uterus, cervix, testes, skin and even in the thorax. The exact mechanism whereby a WT occurs in extrarenal tissues is not known. The presence of ectopic metanephric blastema cells or the WT gene that cause transformation of extrarenal primitive mesonephric or pronephric remnants into WT are both considered in the embryogenesis. Although ultrasonography and CT scan are both helpful in the definition of retroperitoneal tumours, there is no characteristic finding to diagnose an extrarenal WT before surgery. However the histological characteristics are the same as in intrarenal WT, a retroperitoneal teratoma should be clearly investigated for a possible admixture of WT cells. patients with extrarenal WT are given the same treatment protocol as patients with stage III WT. In this paper, a 5-year-old female patient with an extrarenal WT located in the lumbosacral region is presented. Conclusion: as a rule, diagnosis of extrarenal Wilms' tumour is made after surgery. Surgical excision is the treatment of choice and the same general therapeutic rules should be followed as when the kidney were affected. Stage III guidelines for chemotherapy and radiotherapy are appropriate in these patients.- - - - - - - - - - ranking = 1.3333333333333keywords = tumour (Clic here for more details about this article) |
8/159. Wilms' tumour metastasis to the testis: long-term survival.We report the case of a 3-year-old boy with a Wilms' tumour of unfavourable histology with metastatic spread to the right testis. orchiectomy and chemotherapy led to disease-free survival for more than 8 years.- - - - - - - - - - ranking = 0.83333333333333keywords = tumour (Clic here for more details about this article) |
9/159. Extrarenal Wilm's tumour - a rare entity.A rare case of extrarenal Wilms' tumour (ERWT) that presented as an asymptomatic subcutaneous lumbar mass is reported. The diagnosis could only be established postoperatively.- - - - - - - - - - ranking = 0.83333333333333keywords = tumour (Clic here for more details about this article) |
10/159. Fatty degeneration in a Wilms' tumour after chemotherapy.We report a case of extensive fatty change in a Wilms' tumour after chemotherapy demonstrated on CT associated with an increase in tumour volume, in a 10-month-old girl with beckwith-wiedemann syndrome. Changes in tumour characteristics after chemotherapy on imaging usually reflect necrosis, haemorrhage and calcification. Assessment of response to therapy is dependent on a documented reduction in tumour volume. In this case, CT showed an increase in tumour size with development of an extensive fatty component following treatment. Subsequent histological examination on the nephrectomy specimen confirmed an extensive fatty component with no evidence of residual blastema. The development of such an extensive fatty component is very unusual. In this case such fatty change was an indicator of tumour sensitivity and response to treatment.- - - - - - - - - - ranking = 1.6666666666667keywords = tumour (Clic here for more details about this article) |
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