1/387. Successful treatment of a patient with stage IV rhabdoid tumor of the kidney: case report and review.The clinical course of a 31-month-old patient with advanced (stage IV) rhabdoid tumor of the kidney (RTK) and an analysis of treatment variables that may impact survival are presented. Treatment included complete resection of abdominal disease, radiation therapy to the abdomen and chest, and chemotherapy on a schedule of dose intensification by reduction of the interval between cycles. Inclusion of doxorubicin in treatment was associated with survival among patients in published series (P = 0.002). The patient was in continuous complete remission 60 months from diagnosis. Stage IV rhabdoid tumor of the kidney can be effectively treated with intensive multimodal therapy. doxorubicin may be an important component of a successful therapeutic regimen.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
2/387. Metanephric adenofibroma: report of a case and review of the literature.The recent recognition of a variety of pediatric renal tumors of different biologic behavior places an ever-increasing demand on the surgical pathologist for an accurate diagnosis. Although metanephric adenofibroma is one of the rarest benign renal tumors, the clinical importance of correctly diagnosing it cannot be overemphasized because it can potentially be mistaken as Wilms' tumor. We describe the clinical, radiologic, and pathologic features of a case of metanephric adenofibroma and discuss its differential diagnosis. The neoplasm was composed of two discrete components: a major fibroblastic element and a minor immature epithelial element. The latter formed a small nodule beneath the renal capsule, which could barely be detected by magnetic resonance imaging. This subcapsular nodule, however, was slightly soft and tan and was distinctly different from the white, whorled cut surface of the main tumor. It was formed by closely packed small immature epithelial cells in a slightly edematous background, which was histologically identical to metanephric adenoma and closely resembled epithelial Wilms' tumor. Unlike Wilms' tumor, however, the epithelial cells were very bland with no mitoses. The main bulk of the tumor was formed by spindle fibroblastic cells that were cytologically similar to the spindle cells in congenital mesoblastic nephroma. The tumor, however, was well demarcated without the irregular infiltrating edges of congenital mesoblastic nephroma. In contrast to the randomly distributed epithelial element throughout the stromal component in previous reported cases of metanephric adenofibroma, our finding of the exceedingly small and discrete epithelial component expands the known histologic spectrum of the disease. In addition, the presence of such minute epithelial nodule underscores the importance of diligent pathologic examination and careful sampling of tissue for histologic examination.- - - - - - - - - - ranking = 0.93014565118786keywords = back (Clic here for more details about this article) |
3/387. Renal cell carcinoma in an intrathoracic kidney: radiographic findings and surgical considerations.Ectopic intrathoracic kidney is a rare phenomenon and is usually an incidental finding on a chest radiograph. Of all intrathoracic kidneys, congenital ectopia is most often shown, with a traumatic etiology occurring much less frequently. We report a case of an ectopic intrathoracic kidney with associated renal cell carcinoma. Management, which was based on current treatment recommendations for isolated renal masses, consisted of radical nephrectomy. The patient has been without evidence of disease recurrence for 36 months after surgery.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
4/387. myelolipoma of the renal sinus. An unusual site for a rare extra- adrenal lesion.Extra-adrenal myelolipomas are rare; approximately 36 cases have been reported to date. We document a case of myelolipoma presenting as a localized mass in the renal sinus of a 66-year-old man. The chief clinical and radiologic differential diagnostic considerations in this case included a malignant renal tumor arising in the hilum. The patient was being investigated for recurrent urinary tract infections and vague abdominal pains. Histologically, the lesion showed features characteristic of a myelolipoma. There was also marked chronic inflammation in and around the mass. The uneventful follow-up of 62 months is in keeping with the benign nature of this lesion. This report expands the possibilities of the differential diagnoses of renal hilar neoplasms, particularly in view of the increased use of imaging techniques that are bound to detect many incidental lesions in this region.- - - - - - - - - - ranking = 13.850275573582keywords = abdominal pain (Clic here for more details about this article) |
5/387. Renal cell carcinoma: a rare source of cauda equina metastasis. Case report.The authors present the case of a patient in whom intradural metastasis from renal cell carcinoma spread to the cauda equina. To the authors' knowledge, this is only the second report of its kind. This male patient had undergone nephrectomy for the treatment of renal cell carcinoma for 5 years and was diagnosed as having metastatic lung disease 1 year prior to admission. The patient presented with lower back pain that radiated to both legs, but he exhibited no sensorimotor deficits. The majority of cauda equina tumors are primary tumors, and metastases are very rare. The literature is reviewed with reference to current molecular genetic paradigms of metastatic renal cell carcinoma.- - - - - - - - - - ranking = 12.917475359351keywords = back pain, back (Clic here for more details about this article) |
6/387. angiotensin ii type 1 receptor expression in two cases of juxtaglomerular cell tumor: correlation to negative feedback of renin secretion by angiotensin ii.The angiotensin ii (Ang II) type 1 (AT1) receptor is highly expressed on juxtaglomerular (G) cells and is assumed to be involved in the negative short loop feedback regulation of renin secretion and in the suppression of Ang II-mediated JG cell proliferation and/or growth. However, as JG cell tumor is rare, expression and pathophysiological significance of AT1 receptor expression in JG cell tumor remain unknown. In the present study, we investigated renin responses to various treatments, including the angiotensin converting enzyme inhibitor captopril, and correlated the results with AT1 and Ang II type 2 (AT2) receptor mRNA expression levels in two cases of JG cell tumor. Whereas plasma renin activity (PRA) did not show any significant change in Case 1, it was increased by 72% in Case 2 in response to captopril challenge. In concordance with these results, AT1 receptor mRNA was not detected in tumor tissue of Case 1 but was clearly demonstrated in the tumor of Case 2. AT2 receptor mRNA expression was not detected in either of the cases. In contrast to captopril challenge, PRA was suppressed by 30% in Case 1 and 42% in Case 2 in response to saline infusion, and was increased by 230% in Case 1 and 59% in Case 2 in response to furosemide-upright posture for 2 h. These results suggest that the short loop feedback inhibition of renin secretion by Ang II in JG cell tumor is closely related to AT1 receptor expression levels in the tumor tissue. In addition, the result suggested that despite its autonomy, renin secretion from JG cell tumor is still under physiological regulatory control.- - - - - - - - - - ranking = 5.5808739071272keywords = back (Clic here for more details about this article) |
7/387. Clinics in diagnostic imaging (36). Benign renal oncocytoma.A 50-year-old Indian man presented with non-specific right-sided abdominal pain. Ultrasound of the abdomen demonstrated a small right renal tumour. Computed tomography showed features suggestive of a benign renal oncocytoma. This was confirmed by frozen section during surgical exploration. A partial nephrectomy was carried out. The role and limitations of imaging in the management of solid renal masses is discussed.- - - - - - - - - - ranking = 13.850275573582keywords = abdominal pain (Clic here for more details about this article) |
8/387. A case of renal juxtaglomerular cell tumor: usefulness of segmental sampling to prove autonomic secretion of the tumor.A 27-year-old female patient had been treated for hypertension with conventional therapy for years, because renal vein renin levels failed to show lateralization in renal venous samplings and a renal juxtaglomerular cell tumor (RJGCT) had gone undiagnosed. Abdominal computed tomography revealed a mass at the middle of the right kidney. The right renal venogram demonstrated distinct segmental veins from the upper pole and from the middle and lower poles in the right kidney. On segmental renin sampling from each renal vein, the plasma renin concentration (PRC) of the segmental veins from the middle and lower poles was higher than that from other sites. We diagnosed RJGCT of the right kidney and performed right-sided nephrectomy. After the resection, the PRC rapidly decreased. Immunohistochemical studies using antihuman renin antibodies revealed positive staining of the tumor cells. It is an important strategy to make a segmental sampling at the site as close as possible to the RJGCT.- - - - - - - - - - ranking = 7.2942157003601keywords = upper (Clic here for more details about this article) |
9/387. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990. The aim of the study, based on a review of the literature, also was to provide a comprehensive view of this uncommon disease. The clinical features were in keeping with previous studies, but we found that exertional dyspnea and pneumothorax were the most common features, and chylous involvement was less frequent. LAM was diagnosed after menopause in about 10% of cases. The onset of LAM occurred during pregnancy in 20% of cases, and a clear exacerbation of LAM was observed in 14% of cases during pregnancy. Pulmonary LAM was diagnosed on lung histopathology in 83% of cases, but renal angiomyolipoma, observed in 32% of our patients, may be a useful diagnostic criterion when associated with typical multiple cysts on chest CT scan or with chylous effusion. Chest CT scan was more informative than chest X-ray (normal in 9% of cases), and may be indicated in spontaneous pneumothorax or renal angiomyolipoma in women of childbearing age. About 40% of the patients had a normal initial spirometry, while an obstructive ventilatory defect (44%), a restrictive ventilatory defect (23%), was observed in other patients. Initial diffusing capacity for carbon monoxide was frequently decreased (82%). Hormonal therapy was administered in 57 patients, but a clear > or = 15% improvement of FEV1 was observed in only 4 evaluable patients, treated with tamoxifen and progestogens (n = 2), progestogen (n = 1), and oophorectomy (n = 1). Probably 1 of the most urgent needs for clinical research in LAM is to test the currently available hormonal treatments in the context of international multicenter prospective controlled studies. pleurodesis was performed in 40 patients. lung transplantation was performed in 13 patients, 7.8 /- 5.2 years after onset of LAM, in whom the mean FEV1 was 0.57 /- 0.15 L. After a follow-up of 2.3 /- 2.2 years, 9 patients were alive. Mean follow-up from onset of disease to either death or closing date was 8.2 /- 6.3 years. overall survival was better than usually reported in LAM, and Kaplan-Meier plot showed survival probabilities of 91% after 5 years, 79% after 10 years, and 71% after 15 years of disease duration.- - - - - - - - - - ranking = 2keywords = chest (Clic here for more details about this article) |
10/387. Fine-needle aspiration cytology of renal-cell adenocarcinoma metastatic to the breast: A report of three cases.Metastases to the breast from extramammary primary malignancies, including renal adenocarcinoma, are rare. Fine-needle aspiration biopsy (FNA) is a useful, noninvasive, and rapid procedure to evaluate these mammary lesions. This study describes the cytomorphology of 3 cases of renal-cell adenocarcinoma metastatic to the breast. All patients had a prior history of renal-cell adenocarcinoma treated with radical nephrectomy, and they presented with a solitary mammary mass. The cytologic findings showed irregular clusters and dispersed single cells with eccentric nuclei and abundant, vacuolated cytoplasm in a hemorrhagic background. The nuclei were round to oval, with fine granular chromatin and a single, prominent nucleolus. All aspirates were interpreted initially and correctly as consistent with metastatic renal-cell adenocarcinoma. In summary, a cytologic diagnosis of renal-cell adenocarcinoma metastatic to the breast can be made by correlating clinical and cytologic findings. The distinction between metastatic extramammary malignancies to the breast and primary mammary carcinoma is critical to avoid unnecessary surgery and to ensure appropriate chemotherapy or radiation therapy.- - - - - - - - - - ranking = 0.93014565118786keywords = back (Clic here for more details about this article) |
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