Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two new cases of malignant rhabdoid tumor of the kidney (RTK) in childhood are reported. Both presented with large abdominal masses and developed hypertension and one became hypercalcemic during the course of the disease. In each case disseminated disease and death occurred within three months of diagnosis. The findings on imaging consisted of large renal masses with a central site of origin, distant metastases at the time of presentation, and a subcapsular fluid collection in one of the two patients. The clinical and imaging features of RTK may suggest this diagnosis when faced with a primary renal neoplasm in childhood.
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PMID:Malignant rhabdoid tumor of the kidney: imaging features in two cases. 228 37

A 4-month-old male infant with Stage I rhabdoid tumor of the kidney at presentation subsequently developed pulmonary metastatic disease shortly after diagnosis and initiation of Vincristine and Actinomycin D chemotherapy. The patient was then treated with cis-platinum and Adriamycin. Within 28 days he achieved a complete remission which was maintained for 5 months. Subsequent recurrent pulmonary lesions failed to regress with radiotherapy and high-dose cyclophosphamide when used as single sequential agents. Further clinical trials with cis-platinum and Adriamycin seem warranted since prognosis with this tumor is poor and successful chemotherapy after metastatic dissemination has not been previously reported.
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PMID:Rhabdoid tumor of the kidney: complete remission induced by cis-platinum and adriamycin. 626 11

Rhabdoid tumor of the kidney (RTK), originally described as a monophasic sarcomatous variant of Wilms' tumor, is now recognized as a highly malignant, non-Wilms' tumor possibly of neuroectodermal origin. Twenty-one National Wilms' Tumor Study patients with this tumor were treated in the years 1969 through 1978. Mean patient age was 18 months with 16 of the 21 younger than 2 years at diagnosis. Two patients were Stage 1, 10 Stage II, 5 Stage III, and 4 Stage IV. One patient only is continuously disease free and another is surviving disease free following excision of bilateral pulmonary metastases. One patient died of sepsis early during therapy. Thus 18 of the 19 patients who relapsed died, 15 within 1 year of diagnosis, all with progressive tumor growth. The rapid appearance of metastases (mean 4 months), often to multiple sites, and short subsequent survival signal a very malignant tumor resistant to current treatment stratagems.
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PMID:Clinical aspects of the rhabdoid tumor of the kidney: a report of the National Wilms' Tumor Study Group. 631 Mar 57

Severe hypercalcemia occurred in a child with metastatic disease from a rhabdoid tumor of the kidney. Because there was no evidence of skeletal involvement by tumor, an investigation of the cause for hypercalcemia was undertaken. A greatly elevated serum concentration of immunoreactive parathyroid hormone (iPTH) was documented. This together with the observation of histologically normal parathyroid glands and the immunohistologic demonstration of parathyroid hormone within tumor cells supports the hypothesis of ectopic iPTH production by the tumor. The concurrence of an unusual metabolic complication with an infrequently encountered tumor variant is notable.
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PMID:Hypercalcemia associated with immunoreactive parathyroid hormone in a malignant rhabdoid tumor of the kidney (rhabdoid Wilms' tumor). 633 31

Malignant rhabdoid tumor is a rare, aggressive, invariably lethal tumor that is resistant to multimodal treatment. In this report, two patients with malignant rhabdoid tumor of the kidney (RTK) are described. The first patient is the first case of RKT with hyperreninemia, and the second case is also the first case with a specific chromosomal abnormality, del 11p13. The first patient presented with hematuria and a mass in the left kidney. Plasma renin, angiotensin, and aldosterone levels were elevated and paralleled the tumor progression. The karyotype of the tumor cells was normal (46,XX). In the second patient, who presented with a mass in the right kidney, the concentration of plasma tissue polypeptide antigen was elevated and paralleled the tumor progression. The karyotype of the tumor cells was 46,XX, del(11)(pter-p13::p12-qter). RTK with a cytogenetic abnormality of del(11p13), which is usually found in aniridia-Wilms' tumor syndrome, has not been known. Both patients died of metastatic disease within 7 months of diagnosis in spite of the multimodal therapy. The clinicopathology of RTK and the differences between Wilms' tumor and RTK raise compelling questions which should be the subject of future studies.
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PMID:Rhabdoid tumor of the kidney: a report of two cases with respective tumor markers and a specific chromosomal abnormality, del(11p13). 869 95

Rhabdoid tumor of the kidney (RTK) is a quite rare malignant neoplasm of early childhood. It has a very unfavourable prognosis, since it tends to give early metastases and shows a poor response to chemotherapy regimens. We report a case of an infant with RTK, who had a rapidly progressive course. Based upon our case and the review of the literature, we would like to stress the importance of a differential diagnosis with another kidney cancer, namely Wilms tumor, which is more frequent and has by far a better prognosis.
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PMID:[Rhabdoid tumor of the kidney: a rare malignant neoplasm of infancy. A case report and review of the literature]. 959 90

We present our data on the treatment of Wilms' Tumor (WT) with an emphasis on both the positive effect and the adverse effect of preoperative chemotherapy with regard to surgical intervention. From 1980 to 2000 70 children were treated. 57 % received preoperative chemotherapy (ChTx) and 43 % were operated on primarily. 75 % of the tumors responded to ChTx with significant shrinkage of the size. After preoperative ChTx 54 % of the cases were regrouped as stage I, whereas after primary operation 46 % of the patients were grouped as stage I, thus indicating a downstaging with preoperative ChTx. In 8 % of the patients with preoperative chemotherapy intraoperative complications occurred with a rupture of the tumor in 1 case. In contrast, there were intraoperative complications in 25 % of the patients with a primary operation with rupture of the tumor in 3 cases. 1 child (1.5 %) was treated with chemotherapy who did not have a Wilms' tumor but a benign nephroma (CMN). 3 cases had a clear cell sarcoma (CCSK) and in one case histology revealed a rhabdoid tumor (MRTK). In one case of CCSK only histology of the metastases disclosed the correct diagnosis. The rate of postoperative complications such as ileus was the same for both groups. Irrespective of the known adverse effects such as changing tumor histology, which may affect the correct staging, and the remaining risk of an initial inadequate treatment, our data show that the regimen of preoperative chemotherapy as proposed by the SIOP study should not be abandoned. However, the relatively small number of our patients does not allow a significant statement to be made but confirms the results of past SIOP studies.
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PMID:Surgical complications in the treatment of Wilms' tumor. 1641 59

Rhabdoid tumor of the kidney (RTK) is a rare and highly malignant neoplasm of infancy, with a strong tendency for early metastasis to distant regions. RTK is unique in its significant association with primary or metastatic brain tumors. The authors report the first case of RTK presenting initially with hemiplegia. The patient was found thereafter to have RTK concurrent with pulmonary metastases, a brain tumor, and a cerebral ischemic lesion. Intensive chemotherapy consisting of carboplatin and etoposide alternating with cyclophosphamide was unsuccessful and the patient died 5 months later because of severe respiratory distress resulting from widespread pulmonary metastases.
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PMID:Rhabdoid tumor of the kidney presenting with hemiplegia: report of a case. 1745 78

The patient was a 30-year-old man who had undergone living-donor kidney transplantation for renal failure caused by IgA nephropathy at age 29. On post-transplantation day 83, he visited our department with a chief complaint of asymptomatic hematuria. CT performed on post-transplantation day 95 revealed a tumor (size, 4 cm) in the right native kidney that had not been observed at the time of transplantation. CT performed on post-transplantation day 153 showed that the tumor had enlarged to 6 cm, while retrograde pyelogram performed on post-transplantation day 171 was negative for renal pelvic tumor. On post-transplantation day 193, radical right nephrectomy was performed. The tumor had directly invaded the diaphragm and the lower surface of the liver, and was histopathologically diagnosed as rhabdoid tumor of the kidney. As the pathological tissue was extremely malignant, hepatic posterior segmentectomy, right adrenalectomy, and lymph node dissection were further performed for metastases on post-transplantation day 200. On the 23rd day after radical right nephrectomy (post-transplantation day 216), the patient developed dyspnea. Chest CT showed pleural effusion, hemothorax in right lung and metastases in both lungs. The patient's general status gradually worsened thereafter, and he died on the 53rd day after radical right nephrectomy (post-transplantation day 246). Rhabdoid tumor of the kidney is a rare renal tumor that affects children, and only four adult cases have been reported to date. We report our experience with this rare case.
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PMID:[Case report of rhabdoid tumor of the kidney occurring in own kidney following kidney transplantation from the living relative]. 2071

The pathogenesis of pediatric malignant tumors is associated with congenital abnormalities. Oncogenes and antioncogenes are identified in some of these cases. Neuroblastoma arises from the adrenal medulla and sympathetic ganglia. Most neuroblastomas produce catecholamine. Urinary vanillylmandelic acid(VMA)and homovanillic acid(HVA), metabolites of catecholamine, are sensitive tumor markers. Risk stratification according to tumor stage and a combination of prognostic factors helps determine the appropriate therapeutic strategy in clinical settings. Nephroblastoma(Wilms tumor)is the most common pediatric renal tumor and is often accompanied by congenital anomalies. Surgical resection of the tumor and the involved kidney is the initial treatment recommendation in the US and Japan. Consecutive chemotherapy and radiotherapy are administered after surgical staging and a definite histopathological diagnosis. Prognosis is relatively good for most nephroblastoma cases with a favorable histology. In addition to nephroblastoma, clear cell sarcoma of the kidney, characterized by a tendency to metastasize to the bone, is a renal tumor with poor prognosis. Rhabdoid tumor of the kidney is another tumor type; however, its pathogenesis is still unknown and it is associated with extremely poor prognosis because of the lack of effective therapeutic measures. Hepatoblastoma is the most common malignant liver tumor. The serum alpha-fetoprotein level is the most effective tumor marker. Complete surgical resection of the involved liver lobe is the definitive approach for cure. Preoperative chemotherapy increases the possibility of complete surgical resection. High-risk patients have a poor prognosis.
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PMID:[Clinical features of solid malignant tumors in childhood]. 2386 21


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