1/9. Management of Wilms' tumor with intracardiac extension.We review our experience and the literature in treating 4 patients with Wilms' tumor (WT) with intracardiac extension among 92 patients with this neoplasm. cardiopulmonary bypass with circulatory arrest and profound hypothermia was used. There were 3 boys (3 years, 4 years 5 months, and 15 years) and 1 girl (6 years). The follow-up periods were 8 months, 3 years, 2 years 6 months, and 15 years, respectively. We had no surgical complications and conclude that the preoperative diagnosis is extremely important. These patients must be transferred to institutions where concomitant cardiac procedures can be performed. In treating patients with WT, Doppler ultrasound must be used preoperatively in all cases, not only those in which clinical and radiologic signs of intravascular involvement are found. We propose that preoperative chemotherapy should be used, as it shrinks the thrombus and causes desirable adherence of the thrombus to the venous wall, reducing the probability of thromboembolism during the surgical procedure. We also find this method safer than in our 1st case, where neither cardiac arrest nor hypothermia was used. Our results agree with the literature that intracardiac extension of WT does not worsen its prognosis when a rational surgical approach is used.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
2/9. Increased prevalence of renal cell carcinoma in patients with hiv infection.Following the observation of the occurrence of renal cell carcinoma (RCC) in a local hiv patient, a retrospective case review was conducted on our local hospital population to determine if the prevalence of RCC in patients with hiv infection was greater than in the non-hiv population. All 66,715 unique adult patient admissions (over 18) to the Medical Center of Central georgia from 1990 through 1994 were reviewed to determine the total number of hiv admissions, the total number of patients with RCC, and the total number of patients with concomitant RCC and hiv infection. The expected prevalence of hiv-positive adults with RCC in this hospital population was then calculated based on local RCC prevalence data using the Poisson equation. Three hundred eight admissions were hiv infected, two of which had concomitant RCC. Forty-eight additional cases of RCC were documented during this time in non-hiv patients. The probability of an adult coming to this institution with RCC is 0.0007. Using this density in the Poisson equation, the probability of observing two cases of RCC in 308 hiv admissions was 0.01873, p < 0.05. The difference between the two proportions equation yielded a z value of 1.121. Data reveal that the prevalence of RCC in our hospital hiv patients is 8.5 times greater than in our non-hiv population, with an average age of occurrence approximately 15 years younger than national statistics.- - - - - - - - - - ranking = 2keywords = probability (Clic here for more details about this article) |
3/9. Metanephric adenoma: case report and review of the literature.Metanephric adenomas are benign tumors frequently found post-mortem (from 7% to 22% of autopsies) which originate from distal tubules; they are generally small in dimensions (smaller than 1 cm) and located in the renal cortex. Etiology is unknown, even though they could be associated with smoke, tubular nephrosclerosis, dialysis. An endocrinal relationship was proposed, because of its more frequent incidence in female (2:1). Metanephric adenoma is an uncommon kind of renal adenoma with no malignant potentiality: from the clinical and diagnostic viewpoint its own greater importance depends on the probability of diagnostic misunderstanding with Wilms' tumor; furthermore its echographic, tomographic and arteriographic characteristics are often similar to small renal adenocarcinoma ones (100). Polycytemia is frequently associated to metanephric adenoma as paraneoplastic syndrome and, more rarely, abdominal mass, abdominal pain, hematuria and hypertension (140). The most important study on metanephric adenoma is the one realized by E.K. Mostofi, including 50 cases from the Department of Genitourinary pathology, Armed Forces Institute of pathology, washington, D.C., published in 1995; in this study half of findings was incidental; mean dimension of tumor about 5,5 cm and in 50% it could be seen a distinct capsule sourrounding the tumor (in the remaining cases the capsule was discontinuos or absent) (210). An important radiological characteristic is that metanephric adenomas are more frequent calcificated than other histotypes and, from the pathological viewpoint, the most important differential element seems to be the smaller dimensions of its cells with hyperchromatic nuclei in the absence or lack in mitotic activity and in the absence of chromosome aberrations. In the case of difficult histological diagnosis, cytogenetic and immunohistochemical analysis can be useful. In conclusion, because it is impossible to distinguish in the preoperatory between the metaneprhic adenomas and the other tumoral types on the bases of the symptoms, dimensions or radiographic appearance, it is mandatory to treat it as malignant eteroformations in a therapeutical strategy, when it is possible.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
4/9. Concurrent hepatic and ruptured renal angiomyolipoma in tuberous sclerosis complex.angiomyolipoma of the liver or kidney is one of the clinical manifestations of tuberous sclerosis complex. However, concurrence of angiomyolipoma in both liver and kidney associated with tuberous sclerosis complex is a rare entity. Renal angiomyolipomas with large aneurysms confer a higher probability of rupture as compared to small aneurysms. Herein, we document a case of tuberous sclerosis coexisting with hepatic and renal angiomyolipoma in a 37 year-old woman who presented with an acute abdomen due to ruptured tumor. Computed tomography of the abdomen revealed multiple tumors over the bilateral kidneys and liver. A right nephrectomy was performed. During surgery, a liver biopsy was performed from which a preliminary diagnosis of necrosis was established. However, immunoreactivity staining using monoclonal antibody HMB-45 (Human melanoma, Black) led to the final diagnosis of angiomyolipoma. We emphasized that pathologists and clinicians should be aware that cases of tuberous sclerosis complex may be associated with renal and hepatic angiomyolipoma. To avoid an inappropriate diagnosis, before diagnosing liver necrosis, immunohistochemical staining for HMB-45 is recommended.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
5/9. Metastatic anaplastic carcinoma of the thyroid mimicking squamous cell carcinoma: report of a case of a challenging cytologic diagnosis.BACKGROUND: The probability of anaplastic transformation in a differentiated thyroid carcinoma (DTC) is <2%. Admixture of DTC and undifferentiated carcinoma can occasionally be found in thyroidectomy specimens, or the undifferentiated tumor may develop in metastatic foci months or years after removal of the primary tumor. CASE: Anaplastic transformation of a metastatic DTC was diagnosed at autopsy in a 71-year-old female. At the time of her total thyroidectomy for follicular carcinoma, she already had several lung nodules, which diminished in size upon 131I treatment. Five years later the patient developed a new, pleura-based lung mass. The mass extended along the paraspinal soft tissue and involved the kidney. Fine needle aspiration biopsy of the paraspinal mass resulted in a diagnosis of metastatic squamous cell carcinoma. Two months later the patient died. Histologic evaluation of the lung nodules and pleura-based mass revealed areas of follicular carcinoma intermixed with a various patterns of undifferentiated carcinoma. The paraspinal and kidney mass consisted entirely of anaplastic tumor, including areas of squamoid differentiation. CONCLUSION: In patients with a history of DTC who develop a rapidly growing mass elsewhere, a possibility of metastatic DTC with anaplastic transformation must be considered.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
6/9. Combined intra-arterial actinomycin D and radiation therapy for surgically unresectable hypernephroma.One primary goal of preoperative radiotherapy for hypernephroma is to reduce the volume of tumor and, therefore, improve the possibility of resection. It is important that this goal be accomplished promptly so that 4 to 6 weeks after radiation therapy nephrectomy can be attempted. A longer waiting period may allow fibrosis of the normal surrounding tissues and make surgery more difficult. In addition, longer waiting periods could theoretically increase the probability of metastasis. Therefore, we plan to continue clinical investigation on the use of combined intra-arterial actinomycin D and radiotherapy as a possible useful means of improving the possibility of prompt surgical resection, since theoretically this regimen may be a method of increasing the effective radiation dose to the hypernephroma without increasing the effective radiation dose to surrounding normal tissue, such as bowel. The method may also have merit as an improved means of palliating selected patients with metastases who are symptomatic from a bulky primary hypernephroma.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
7/9. Inactivation of WT1 in nephrogenic rests, genetic precursors to Wilms' tumour.Nephrogenic rests consist of foci of primitive renal cells, typically microscopic, that are found within the normal kidney tissue of children with Wilms' tumour. To study the relationship between nephrogenic rests and the associated tumours, we screened these lesions for mutations in the 11p13 Wilms' tumour suppressor gene, WT1. In two cases in which the Wilms' tumour contained a somatic WT1 mutation, the nephrogenic rest had the identical mutation. Nephrogenic rests and Wilms' tumours are therefore topographically distinct lesions that are clonally derived from an early renal stem cell. Inactivation of WT1 appears to be an early genetic event which can lead to the formation of nephrogenic rests, enhancing the probability that additional genetic hits will lead to Wilms' tumour.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
8/9. Malignant melanoma of the kidney presenting as a primary tumor.We report a case of malignant melanoma of the kidney presenting as a primary tumor. This tumor was found incidentally in a 74-year-old woman. The patient underwent a right radical nephrectomy, and has been living tumor free for 2 years and 3 months. This is the first reported case of primary renal malignant melanoma. We discuss the probability that this tumor is renal in origin and directly linked to the origin of malignant melanoma.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
9/9. Successful double autografts for patients with relapsed clear cell sarcoma of the kidney.Although the prognosis of clear cell sarcoma of the kidney (CCSK) has improved, when metastases occur the probability of cure is very low. We have treated two pediatric patients with relapsed CCSK, one with multiple bone metastases and another with brain metastases. After one or two courses of re-induction chemotherapy and radiation therapy to the sites of metastasis, they received double high-dose chemotherapy with autologous bone marrow rescue. Conditioning regimens were ifosphamide plus melphalan for the first autograft and busulfan plus thiotepa for the second. Hematological recovery was prompt, and no severe complications were observed. They are doing well without evidence of recurrence at 19 and 49 months after the second autograft, respectively.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |