Cases reported "Kidney Neoplasms"

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1/39. 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.
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2/39. Renal cell carcinoma with involvement of iris and conjunctiva.

    PURPOSE: To report a case of metastatic renal cell carcinoma with involvement of the iris and bulbar conjunctiva. methods: Case report. A 70-year-old man without known systemic disease developed an iris mass in his left eye. Iridocyclectomy was performed to remove the iris mass. RESULTS: Histopathologically, the iris mass was consistent with metastatic renal cell carcinoma. Further evaluation disclosed a mass of the left kidney. The patient underwent a left nephrectomy and was found to have renal cell carcinoma, with focal penetration into the renal capsule. One month after the nephrectomy, he developed a highly vascular nodule of the left bulbar conjunctiva. An excisional biopsy was performed, and histopathology disclosed an additional focus of renal cell carcinoma. CONCLUSIONS: iris and conjunctival involvement may be a clinical manifestation of renal cell carcinoma. Renal cell carcinoma should be considered in the differential diagnosis of a fleshy, vascular iris and a conjunctival nodule.
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3/39. Sustained ventricular tachycardia and its successful prophylaxis during high-dose bolus interleukin-2 therapy for metastatic renal cell carcinoma.

    In the setting of interleukin-2 (IL-2) administration, tachycardias of ventricular origin are classified as serious, grade IV toxicities, necessitating the discontinuation of therapy. In this report, we describe a patient with renal cell carcinoma who experienced ventricular tachycardia while undergoing treatment with high-dose bolus IL-2. Prophylaxis with sotalol permitted the successful completion of his first cycle of treatment, without any recurrent rhythm disturbances.
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4/39. Sarcomatoid renal cell carcinoma with scant carcinomatous components.

    A 30-year-old male underwent radical nephrectomy for a right renal tumor 15 cm in diameter. On microscopic examination of initial 17 sections, the tumor consisted of pleomorphic giant cells and spindle neoplastic cells. There was no carcinomatous component. Immunohistochemically, the neoplastic cells were negative for keratin and epithelial membrane antigen but positive for vimentin. The giant cells were also scatteringly, weakly positive for myoglobin. At that time a diagnosis of rhabdomyosarcoma of the kidney was made. However, further microscopic examination of another eight sections revealed small areas of clear cell-type renal cell carcinoma (RCC) which transited to sarcomatous components and led to a diagnosis of sarcomatoid RCC. The patient underwent three cycles of adjuvant chemotherapy. He has been free of the disease for 14 months after nephrectomy.
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5/39. Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney: a case report and review of the literature.

    Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.
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6/39. Expression of apoptosis, cell proliferation, and drug resistance genes in pediatric Wilms' tumors.

    The expression of genes associated with apoptosis, cell proliferation and drug resistance in tumor cells was investigated in two pediatric Wilms' tumor patients (MCH-WT-1 and MCH-WT-3) for their association with cell cycle, daunorubicin accumulation and clinical data. dna content, cell cycle and drug accumulation were analyzed immediately after surgery by flow cytometry and mRNA expression by reverse transcriptase-polymerase chain reaction (RT-PCR) assay. Primary cell cultures were established from tumor specimens and tumor cells in both cases showed epithelial morphology. Although cell proliferation markers (Ki67 and PCNA) were expressed in both cases, MCH-WT-3 showed higher levels of mRNA expression, which corresponded, with metastatic behavior of the tumor in the patient. While p53 and p21 mRNAs were expressed at low levels in MCH-WT1, MCH-WT-3 showed high levels of p21 mRNA only. The increased expression of cyclin kinase inhibitor (p21) in MCH-WT-3 compared to MCH-WT-1 correlated with a higher percentage of G0/G1 cell population in the tumor specimen. Despite the expression of multidrug resistance markers (MDR1 and LRP) in MCH-WT-1, flow cytometric analysis showed tumor cell populations with very low and high daunorubicin accumulation and with the absence of any effect for verapamil and dipyridamole on daunorubicin accumulation of tumor cells.
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7/39. Metastatic primitive neuroectodermal tumor of the kidney in adults.

    OBJECTIVE: Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. The purpose of our study was to present information about the management of patients with metastatic disease. methods: The records of 2 patients (30-year-old female and 32-year-old male) with metastatic PNET of the kidney were reviewed and our data compared with the literature. RESULTS: Neither clinical evaluation nor radiological methods allowed to distinguish PNET from renal cell carcinoma. immunohistochemistry revealed strong positivity for CD99 in tumor 1 and weak positivity for NSE and vimentin in both tumors. In tumor 2, EWS/FLI1 translocation was detected by RT-PCR. Patient 1 underwent nephrectomy, seven cycles of polychemotherapy, two cycles of high-dose chemotherapy, autologous bone marrow rescue, radiotherapy of suspicious skeletal foci and is without evidence of recurrent disease 28 months after therapy. Patient 2 underwent six cycles of polychemotherapy, nephrectomy, high-dose chemotherapy with cyclophosphamide and abdominal radiotherapy. Because of relapse high-dose chemotherapy with stem cell rescue was not performed. He underwent three further cycles of polychemotherapy and died one year after diagnosis due to cerebral metastasis. CONCLUSIONS: The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences because polychemotherapy and high-dose chemotherapy may lead to dramatic tumor reduction or even complete remission.
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8/39. Retroperitoneoscopic radiofrequency ablation of a solid renal mass.

    PURPOSE: To report a new technique for radiofrequency (RF) ablation of a solid renal mass. PATIENT AND methods: An 83-year-old man with a history of chronic renal insufficiency was found to have solid mass in the right kidney. Retroperitoneoscopic localization of the renal mass was accomplished using intraoperative ultrasonography. The lesion was treated with a 14-gauge RITA Starburst XL probe (Rita Medical Systems, Inc., Mountain View, CA). RESULTS: The total treatment time included two cycles of 5.5 minutes. There were no intraoperative complications. Tissue desiccation was noted during treatment. A CT scan 48 hours after ablation showed a decrease in the density of the lesion suggestive of coagulation necrosis. The postoperative hospital course was uneventful. CONCLUSION: The retroperitoneal laparoscopic technique is a feasible approach to performing RF ablation of a solid renal mass. It facilitates direct insertion of the RF probe, allows viewing and avoidance of adjacent structures such as bowel, and permits better staging by enabling biopsy of perirenal fatty tissue.
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9/39. Donor-derived small cell lung carcinoma in a kidney transplant recipient.

    BACKGROUND: Transplantation of donor-derived malignancies during organ transplantation fortunately is very rare. Discontinuation of immunosuppressive medications under these circumstances has previously resulted in complete tumor rejection. Ectopic adrenocorticotropic hormone (ACTH) production may result in cushing syndrome and is not an uncommon paraneoplastic feature of small cell carcinoma of the lung. Theoretically, in the organ transplantation setting, the resulting high cortisol levels could suppress a tumor-rejection immune response. However, to the authors' knowledge, no such clinical scenario has been described in the literature published to date. methods: A 25-year-old living related kidney transplant recipient presented with cushing syndrome 32 months after transplantation. The donor had been diagnosed with small cell carcinoma of the lung 22 months earlier. On further evaluation, the kidney recipient was diagnosed with donor-derived small cell lung carcinoma of the transplanted kidney. She was found to have extensive disease involving the liver and retroperitoneum. Despite discontinuation of immunosuppressive medications, the disease progressed and cortisol levels remained elevated during 6 weeks of observation. RESULTS: The patient received six cycles of cisplatin and etoposide, which resulted in resolution of her hypercortisolemia and a complete remission of her donor-derived small cell carcinoma. At last follow-up, she was 12 months from completing her therapy and continued in complete remission. CONCLUSIONS: Donor-derived small cell carcinoma and ectopic ACTH production can occur in a patient after kidney transplantation.
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10/39. Active chemotherapy for collecting duct carcinoma of the kidney: a case report and review of the literature.

    BACKGROUND: Collecting (Bellini) duct carcinoma (CDC) of the kidney is associated with an aggressive course and an extremely poor prognosis. To the authors' knowledge, there are no standard treatment regimens and neither immunotherapy nor chemotherapy have been found to be effective. methods: In the current study, the authors report a 49-year-old man who presented with a 7.0 cm x 6.0 cm renal mass with extensive regional, paraaortic, and left supraclavicular lymphadenopathy. Radical nephrectomy revealed a CDC. The patient was treated with doxorubicin, 50 mg/m(2) (Day 1), and gemcitabine, 2000 mg/m(2) (Day 1), (AG) every 2 weeks with granulocyte-colony-stimulating factor (GCSF) support. RESULTS: The left supraclavicular lymphadenopathy significantly decreased in size after the first cycle. Computed tomography (CT) scan after the third cycle revealed a significant (68%) reduction in the tumor volume. Toxicity was comprised of only CTC version 2.0, 1998; Grade 1 nausea and fatigue. After Cycle 6, a repeat CT scan demonstrated minimal disease progression. Based on recent Phase II data of an active regimen comprised of AG alternating with ifosfamide, paclitaxel, and cisplatin (ITP) in patients with transitional cell carcinoma, the patient was treated with ifosfamide, 1500 mg/m(2) (Days 1-3); paclitaxel, 175 mg/m(2) (Day 1); and cisplatin, 35 mg/m(2) (Days 1 and 2), every 4 weeks with GCSF support. After two cycles of ITP, the patient developed disease progression in bone and received palliative radiation therapy. Follow-up CT scan demonstrated new liver metastases. The patient received palliative care without further chemotherapy and died approximately 10 months after the initial diagnosis of CDC. CONCLUSIONS: Immunohistologic and molecular analyses indicate that CDC more closely resembles transitional cell carcinoma than renal cell carcinoma. Chemotherapy regimens used to treat advanced transitional cell carcinoma such as AG should be evaluated as first-line therapy for CDC.
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