Cases reported "Sickle Cell Trait"

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1/8. Collecting duct carcinoma of the kidney associated with the sickle cell trait.

    Collecting duct carcinoma of the kidney is a rare neoplasm that arises from the medullary collecting ducts. It has an aggressive clinical course and patients usually present with metastatic disease. Treatment approaches apart from surgery have been unrewarding. Renal medullary carcinoma is also an aggressive tumour which occurs predominantly in young, black, male patients with sickle cell trait. We describe a case of collecting duct carcinoma in a 61-year-old black female who exhibited the sickle cell trait. Histopathology revealed a high-grade tubular carcinoma with marked desmoplasia, adjacent dysplastic collecting ducts and positive immunohistochemical staining for high and low molecular weight cytokeratins and epithelial membrane antigen. The association of collecting duct carcinoma with sickle cell trait has not been previously reported. This case supports the postulate that collecting duct carcinoma and renal medullary carcinoma may be part of the same spectrum of renal malignancies.
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2/8. Sickle cell episode manifesting as superficial thrombophlebitis of the penis.

    In 1955, the first reported case of Mondor's disease (superficial thrombophlebitis) of the penis was published. Since then, there have been scattered reports of penile Mondor's disease in the literature. Most studies suggest sexual trauma or neoplasm as the most frequent etiologic factor. The authors report a case of a sickle cell episode presenting with superficial thrombophlebitis of the penis. The patient was treated with an antiinflammatory agent and reassured that this is a self-limiting process. Resolution of symptoms occurred within 6 weeks.
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3/8. Renal medullary carcinoma: a report of 2 cases and review of the literature.

    Renal medullary carcinoma is a recently described aggressive neoplasm of the kidney. With the exception of 2 patients, all other reported cases have been associated with sickle cell hemoglobinopathies, mainly sickle cell trait and hemoglobin sc disease. Renal medullary carcinoma is a highly malignant tumor with evidence of angiolymphatic and distant metastasis at the time of diagnosis. No specific genetic abnormality has been identified in this neoplasm despite its close association with a genetic disease. We describe 2 cases of renal medullary carcinoma, one associated with hemoglobin sc disease, and the other with what we believe to be the first reported case associated with sickle cell disease.
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4/8. Renal medullary carcinoma in a six-year-old boy with sickle cell trait.

    Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglobinopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.
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5/8. Renal medullary carcinoma.

    PURPOSE: Renal medullary carcinoma is a rare and extremely aggressive neoplasm that almost always develops in young patients with sickle cell trait. To our knowledge all cases to date have been metastatic at surgical resection. Pathological examination reveals an aggressive tumor mainly involving the renal medulla with a varied morphology. The prognosis is dismal. Mean survival from the time of resection is 15 weeks (range 2 to 52). The disease course has not been altered by surgery, radiotherapy or various regimens of chemotherapeutic agents. MATERIALS AND methods: We add to the literature our experience treating renal medullary carcinoma in 2 cases and review the existing literature on this disease. RESULTS: Both patients whom we treated died of the disease, as have the other 35 patients described in the literature. CONCLUSIONS: A high index of suspicion may lead to earlier diagnosis and treatment, and survival of patients with renal medullary carcinoma.
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6/8. Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents.

    An extremely aggressive malignant epithelial neoplasm of the kidney has recently been described and named renal medullary carcinoma. The finding of this tumor is highly predictive of drepanocytes (sickle cells) in tissue sections and thus the presence of sickle hemoglobin, specifically sickle cell trait, in the patient. We present a case report of this rare tumor in a 10-year-old male. The tumor displayed a variable histologic architecture including gland-like areas with intra- and extracytoplasmic material resembling mucin with hematoxylin and eosin stain. This material was negative with periodic acid-Schiff and mucicarmine stains, stained only weakly with alcian blue, and was positive using antibodies against peanut agglutinin. Tumor cells stained positively with antibodies to epithelial membrane antigen, cytokeratin, vimentin, and ulex europaeus lectin. The luminal face of tumor cells stained with peanut agglutinin. Stains using antibodies against carcinoembryonic antigen and alpha-fetoprotein were negative. Ultrastructurally, the tumor cells were characterized by short microvilli lining the luminal surface and lateral complex infoldings of adjacent plasma membranes. We discuss the relationship of this neoplasm to another renal pelvic neoplasm, collecting duct carcinoma, which may rarely occur in children. Renal medullary carcinoma should be included in the differential diagnosis of gross hematuria, which is most commonly benign self-limited hematuria, in young patients with sickle cell trait.
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7/8. Renal medullary carcinoma in patients with sickle cell trait.

    Renal medullary carcinoma has recently been described as an aggressive neoplasm affecting young african americans with sickle cell disease or sickle cell trait. We report the presentation, treatment, and outcome in 3 patients with renal medullary carcinoma along with a description of the unsuccessful treatment attempts. A brief discussion and review of the literature is included.
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8/8. Renal medullary carcinoma: an aggressive neoplasm in patients with sickle cell trait.

    Renal medullary carcinoma is a recently described tumor that occurs exclusively in patients with sickle cell trait. Although extremely rare, the distinctive demographic, clinical, and radiologic findings should suggest the diagnosis of this aggressive neoplasm.
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ranking = 5
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