Cases reported "Kidney Neoplasms"

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1/886. Renal cell carcinoma in children with diffuse cystic hyperplasia of the kidneys.

    We report the clinical, pathologic, and genetic features of renal malignancy in two children with diffuse cystic hyperplasia. Both presented with massive bilateral nephromegaly. Neither had a family history or clinical findings suggestive of tuberous sclerosis or von hippel-lindau disease. The kidneys of both children were extensively replaced by tubulocystic hyperplasia with large eosinophilic epithelial cells. The masses of hyperplastic tissue were nodular, compressing remnants of uninvolved renal parenchyma. Tubulopapillary carcinoma was present in both children, one of whom had bilateral multicentric carcinoma. No loss of heterozygosity was detected in the tumors at the TSC1, TSC2, or VHL gene regions, and no alterations in the VHL gene were detected using single-strand conformation polymorphism analysis. These cases of bilateral renal enlargement with diffuse cystic hyperplasia appear to represent a new clinical syndrome that may warrant bilateral nephrectomy because of the risk of malignancy.
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ranking = 1
keywords = malignancy
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2/886. Primary primitive neuroectodermal tumor (PNET) of the kidney: a case report.

    OBJECTIVES: Primary primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare entity and the case is presented to highlight the histological diagnostic problems. methods AND RESULTS: A 23-year-old female presented with a lump in the left hypochondrium that had appeared 3 months, earlier, which suggested a malignancy on radiological investigation. The nephrectomy specimen revealed a large tumor mass with only a small portion of normal renal parenchyma. histology showed a small cell malignant tumor and the diagnosis of PNET (primary of kidney) and small cell carcinoma were suspected. The tumor showed NSE and vimentin positivity and cytokeratin negativity, which conformed with the diagnosis of PNET. CONCLUSION: The biological behaviour of PNET is more aggressive, with rapid progression and infiltration into the surrounding tissues and distant metastases. Thus it differs from the small renal cell malignancies and hence recognition of this tumor as a distinct entity is essential.
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ranking = 0.5
keywords = malignancy
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3/886. Metanephric adenoma of the kidney.

    BACKGROUND: Metanephric adenoma is a rare renal neoplasm that is histologically and clinically unique. We found this neoplasm in a 62-year-old female, whose renal tumor was incidentally detected on abdominal ultrasound examination. methods/RESULTS: Ultrasound, computed tomography and magnetic resonance imaging were compatible with those of renal cell carcinoma. With a diagnosis of renal cell carcinoma, right partial nephrectomy was performed. This tumor showed histologic similarity to developing metanephric tubular epithelium. It was composed of uniformly small epithelial cells, which formed tubules. CONCLUSIONS: The patient has been well and healthy for 20 months after surgery. The unique features of metanephric adenoma should be clinically and pathologically recognized because of its invariably benign course.
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ranking = 52.543948139811
keywords = neoplasm
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4/886. Metanephric adenoma-like tumors of the kidney: report of 3 malignancies with emphasis on discriminating features.

    BACKGROUND: Metanephric adenoma is a very rare benign renal tumor; only 80 well-documented cases have been reported to date. We have seen several renal tumors that were originally incorrectly diagnosed as metanephric adenoma. DESIGN: We present 3 unusual renal tumors (2 primary and 1 metastatic), each of which illustrates important pathologic features useful in discriminating metanephric adenoma from malignant mimics. RESULTS: Case 1 involved a 46-year-old man with multiple small, cortical, solid, papillary (chromophil) renal cell carcinomas in his right kidney; the patient developed multiple, histologically identical, solid, papillary (chromophil) carcinomas in the opposite kidney 17 months later. Case 2 involved a 32-year-old woman with a 14-cm right renal tumor who developed soft tissue and bone metastases over a 17-year period. Case 3 involved a 52-year-old woman who presented with a 1.8-cm corticomedullary renal nodule, which eventually proved to represent a metastasis from a poorly differentiated (insular) carcinoma of the thyroid. All 3 tumors superficially resembled metanephric adenoma and consisted of primitive, dark-staining cells arranged in tubules or sheets. Each tumor, however, also had features inconsistent with the diagnosis of metanephric adenoma, including multifocal lesions with a variable nuclear-cytoplasmic ratio and diffuse cytokeratin 7 and epithelial membrane antigen immunopositivity in case 1, a 14-cm-diameter tumor with occasional mitoses in case 2, and a distinct fibrous capsule with capsular and vascular invasion in case 3. In addition, all 3 tumors lacked the cytologic features of bland overlapping nuclei with imperceptible cytoplasm consistently seen in metanephric adenoma. CONCLUSION: Adherence to strict histopathologic criteria will discourage misdiagnosis of a malignant or potentially malignant renal neoplasm as the rare and always benign metanephric adenoma.
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ranking = 26.271974069906
keywords = neoplasm
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5/886. Subcapsular hemorrhage of adult Wilms' tumor.

    Wilms' tumor is a rare malignancy of kidney in adults. It usually cannot be differentiated from other renal masses preoperatively. This is a presentation of a case of adult Wilms' tumor who developed spontaneous hemorrhage during radiological evaluation. As tumor rupture can change the stage of the tumor and alter the prognosis of the patient, urgent work-up and treatment is advised.
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ranking = 0.5
keywords = malignancy
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6/886. pemphigus foliaceus and renal cell carcinoma.

    It is possible to distinguish the various forms of pemphigus from one another using clinical, histologic, and immunologic criteria. Paraneoplastic pemphigus, a recently defined type that is severe and often fatal, is associated with an underlying malignancy. We present the second reported case of pemphigus associated with renal cell carcinoma. We do not believe that either case represents paraneoplastic pemphigus, which suggests the possibility of some other link between these two diseases.
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keywords = malignancy
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7/886. 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.
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ranking = 26.271974069906
keywords = neoplasm
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8/886. Congenital mesoblastic nephroma (CMN) with an unusual immunohistochemical feature.

    OBJECTIVES: To describe a case of congenital mesoblastic nephroma (CMN) treated by radical nephrectomy with no evidence of relapses after five years in spite of an unusual positivity for proliferating cellular nuclear antigen (PCNA). methods: A three-month-old child presented a right renal mass with compression of the inferior vena cava. Excretory urography showed an intrarenal mass with distortion of the calyceal system. There was no evidence of metastasis. Radical nephrectomy was carried out; no adjuvant therapy was given. Histological and immunohistochemical studies were performed. RESULTS: The tumor was a 6 x 6 x 5 cm solitary mass extending into perirenal tissue, involving the hilar vessels but not the ureter. Histologically, it has been classified as a congenital mesoblastic nephroma of the classic variant. Positive reaction for vimentin and actin was observed. Strong positivity for PCNA and negativity for P53 were revealed. CONCLUSIONS: PCNA is considered to be a reliable marker of potential malignancy. This, however, contrasts with the biological behavior of our case. Further evaluation is required for correct interpretation of this additional information and to avoid inappropriate aggressive therapy.
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ranking = 0.5
keywords = malignancy
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9/886. 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.
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ranking = 26.271974069906
keywords = neoplasm
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10/886. Collecting duct meningeal carcinomatosis.

    Collecting duct carcinoma (CDC) is an aggressive primary renal neoplasm that represents a distinct subtype of renal cell carcinoma. Histochemical (eg, mucicarmine) and immunohistochemical (eg, ulex europaeus) studies, taken in concert with the gross and histologic findings, allow differentiation of CDC from the conventional varieties of renal cell carcinoma in most cases. Collecting duct carcinoma generally pursues a more aggressive course than conventional renal cell carcinoma. Metastases to regional lymph nodes, bone, adrenal glands, lung, and skin have been reported in CDC. We describe the case of a 26-year-old man who presented with a clinical and radiologic impression of multifocal meningioma. Biopsies of the meninges and extracranial soft tissues revealed metastatic adenocarcinoma; subsequent studies suggested metastatic CDC. Ultrasound-guided biopsy was performed on a subsequently identified renal mass, which showed features consistent with CDC. To our knowledge, this is the first reported case of meningeal carcinomatosis due to CDC. The diagnostic features of this tumor are discussed.
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ranking = 26.271974069906
keywords = neoplasm
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