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Query: UMLS:C0020538 (hypertension)
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Nephrogenic adenofibroma is a novel kidney tumor of young people (mean age of presentation, 13 years), who present with polycythemia, hypertension, or hematuria, which resolve following nephrectomy. The typical nephrectomy specimen contains a solitary, nonencapsulated, vaguely circumscribed, irregularly shaped or spherical, firm mass with either tan, gray-white, or pale yellow coloration. Cysts are sometimes present within the tumor. The histologic appearance is distinctive and characterized by a marked proliferation of spindled mesenchymal cells resembling the classical type of congenital mesoblastic nephroma, encasing discrete nodules of embryonal epithelium similar to the hyperplastic nephrogenic rests (nephroblastomatosis) usually associated with Wilms' tumor. The mesenchymal component consists of a fascicular proliferation of tightly interlaced, uniform, benign-appearing spindled cells that immunostatin for vimentin and fibronectin, but not desmin or actin. The epithelial component consists of discrete islands of blastemal cells that are partially or fully differentiated toward tubular, tubulopapillary, or papillary structures. Psammoma bodies are plentiful. Embryonal epithelium immunostains for cytokeratin but not epithelial membrane antigen. The overall histologic appearance of the mesenchymal and epithelial components is benign, and preliminary clinical data suggest that the tumor has a benevolent course. Two cases, however, contained small, well-circumscribed papillary lesions near the renal pelvis that resembled low-grade collecting duct carcinoma. The clinical implications of the latter finding are unclear.
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PMID:Nephrogenic adenofibroma. A novel kidney tumor of young people. 137 78

Using immunohistochemical techniques, we have determined the localization and distribution of CRH immunoreactivity (CRH-IR) in the human placenta, fetal membranes, decidua, and umbilical cord. Tissues were obtained at 6-8 weeks of pregnancy, at term, in association with premature birth, and from patients with pregnancy-induced hypertension or diabetes mellitus. A polyclonal antibody to the epithelial cell marker cytokeratin was used to identify trophoblast cells. CRH-IR was not detected in placenta or decidua at 6-8 weeks gestation. In tissues obtained after idiopathic premature delivery after 21 weeks gestation, positive CRH staining was found in placenta in syncytiotrophoblast and intermediate trophoblast, but not cytotrophoblast. CRH-IR was present in intermediate trophoblast cells that had invaded maternal blood vessels in decidua basalis. In the fetal membranes, CRH-IR was localized in the epithelium and subepithelial cells of amnion, in the trophoblast layer, in some cells of the reticular and cellular layers of chorion, and in some stromal cells and invasive trophoblast cells of decidua. CRH-IR was found in the amniotic epithelium of the umbilical cord and in the musculature of the umbilical vessels. This pattern of distribution of CRH-IR was found in tissues from 21 weeks gestation to term and postterm, and was similar in tissues examined from patients with pregnancy-induced hypertension and diabetes mellitus. These results show clearly that in placenta and membranes, CRH is localized primarily to syncytiotrophoblast and intermediate trophoblast, but not to cytotrophoblast cells. We suggest that the localization of CRH-IR is consistent with CRH affecting paracrine/autocrine interactions within the placenta, fetal membranes, and decidua that may be involved in the maturation of the fetal hypothalamic-pituitary-adrenal axis and in the stimulus and maintainance of labor.
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PMID:The localization and distribution of corticotropin-releasing hormone in the human placenta and fetal membranes throughout gestation. 202 3

Pre-eclampsia/toxemia (PET) is an idiopathic hypertensive disorder of pregnancy elicited in susceptible mothers by exposure to placental trophoblast. Three facts regarding the placenta in PET are known: an association with large placentas (excessive trophoblast), a tendency for superficial implantation, and inappropriate trophoblastic immaturity, as assessed by ultrastructural and biochemical criteria. A unitary hypothesis is that PET is related to a maturation defect leading to excessive accumulation of inappropriately immature intermediate trophoblast in the placental implantation site. We studied the implantation site of PET and control placentas from three gestational age groups (25 to 30, 30 to 35, and 36 to 40 weeks old [five per group]) by morphometry and immunohistochemistry using antibodies to three phenotypic markers (cytokeratin, human placental lactogen (HPL), and beta 2-microglobulin) and two markers of cell dynamics (proliferating cell nuclear antigen [PCNA] and bcl-2]). Implantation sites in the PET group had increased amounts of intermediate trophoblast (cell number and longitudinal extent) with an increased proliferative index (percentage of PCNA positive) and evidence of phenotypic immaturity (HPL negative). Intermediate trophoblast from both groups was uniformly bcl-2 negative and beta 2-microglobulin positive. Based on these data and the findings of other investigators, we propose that the diagnostic term "atypical implantation site" be added to acute atherosis, villous infarction, and increased syncytial knotting as a characteristic of placentas from pre-eclamptic pregnancies.
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PMID:Pre-eclampsia is associated with an excess of proliferative immature intermediate trophoblast. 777 87

We report a case of bilaterally multifocal renal oncocytomas in a patient with chronic renal disease and a long history of hypertension. Histologic study of the multiple oncocytic neoplasms with hematoxylin-eosin, periodic acid-Schiff, trichrome, and colloidal iron stains revealed novel, intracytoplasmic lumina, which were accentuated by immunoperoxidase staining with high-molecular-weight cytokeratin, epithelial membrane antigen, and Arachis hypogaea lectin. Ultrastructural studies demonstrated multiple microvilli lining the intracytoplasmic lumina. We also review the literature on bilaterally multifocal oncocytomas.
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PMID:Intracytoplasmic lumina in a case of bilaterally multifocal renal oncocytomas. 813 31

A paraganglioma of the urinary bladder in a 60-year-old woman presented with irritative voiding symptoms, without hematuria or hypertension. Sonography revealed a well-limited ovoid mass of the posterior wall, and cystoscopy showed that it was covered by normally appearing mucosa. Treatment consisted of transurethral resection, and the patient has been followed for 2 years without recurrence. Histologically the lesion consisted of small nests of spindle cells with clear to acidophilic cytoplasm; mitotic activity was inconspicuous. Immunohistochemical analyses revealed that the tumor cells were strongly positive for neuron-specific enolase and chromogranin A, and negative for cytokeratin, vimentin, neurofilaments, glial fibrillary acid protein and HMB 45. Sustentacular cells at the periphery of neoplastic cell clusters were positive for S-100 protein.
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PMID:Paraganglioma of the urinary bladder. 877 25

We describe two cases of corticomedullary tumors of the adrenal gland. The patients suffered from Cushings syndrome and paroxysmal hypertension. The corticomedullary tumors consisted of benign looking cortical adenoma cells growing on the background of the pheochromocytoma cells. We further present the ultrastructural and immunohistochemical features of these tumors. Focally a spindle cell sarcoma arising from the corticomedullary tumor was found in one case. The spindle cell sarcoma was immunohistochemically negative with antibodies to chromogranin, synaptophysin, cytokeratin and S-100 protein. Ultrastructurally the sarcoma was composed of undifferentiated primitive cells poorly endowed with cytoplasmic organelles. Focal transitions of the pheochromocytoma into the spindle cell sarcoma were seen. It is hypothesized that the spindle cell sarcoma was arising from the pheochromocytoma component of the corticomedullary tumor.
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PMID:Corticomedullary tumors of the adrenal glands. Report of two cases. Association of corticomedullary tumor with spindle cell sarcoma. 912 25

Clear cell sarcoma of the kidney is a distinct, highly malignant pediatric neoplasm. Its occurrence in adults is extremely rare and the subject of isolated case reports. We present a series of four cases (three males and one female) identified in an adolescent and in young adults (16, 18, 20, and 25 years) with flank mass (three cases), hematuria (two cases), flank pain (two cases), and hypertension (one case). Three patients had stage III disease and one had stage I disease (National Wilms' Tumor Study staging system). All tumors had predominantly or exclusively the classic histology of a monotonous proliferation of uniform small round cells with evenly distributed fine chromatin, although focal microcyst formation (two cases) and spindled architecture (one case) (variant patterns) were also noted. Therapy in all cases consisted of surgery and chemotherapy with or without radiation. Follow-up data (29-202 months) showed distant metastases in all four cases, including the lung (four cases), bone (two cases), and the liver (two cases). Three patients died of disease at 29, 59, and 63 months (mean, 50.3 months), and one patient is alive with no evidence of disease at 202 months. Ultrastructural features included scattered primitive junctions, short and irregular cytoplasmic extensions, and scant to a moderate amount of mitochondria. Immunohistochemical study (three cases) showed immunoreactivity with vimentin (two cases) and no reaction with cytokeratin, epithelial membrane antigen, S-100 protein, or desmin. Flow cytometric analysis showed diploid DNA content in three primary tumors and tetraploidy in one metastatic tumor. The proliferative activity (S-phase fraction) was low to intermediate (mean, 9.8%). Our data suggest that clear cell sarcoma of the kidney in the young adult age group resembles its pediatric counterpart in ultrastructural and immunohistochemical characteristics, proclivity for skeletal and visceral metastasis, DNA diploid status with relatively low S-phase, and aggressive clinical course. Clear cell sarcoma of the kidney in adult patients, although rare, must be differentiated from sarcomatoid carcinoma, sarcomas, and round cell tumors because of its unique characteristics in comparison to other renal neoplasms.
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PMID:Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis. 1058 98

The association of membranous glomerulonephritis with benign tumors is rarely described. This report represents, to the best of our knowledge, the first documented account of the simultaneous occurrence of juxtaglomerular cell tumor of the kidney and membranous glomerulonephritis. A young Chinese woman presented with hypertension and proteinuria, whereupon investigations disclosed a renal tumor. She underwent surgical resection, and histologic examination of the tumor revealed CD34-positive uniform polygonal cells with accompanying mast cells, disposed in sheets with focal papillary pattern punctuated by cytokeratin-positive tubular structures. Characteristic rhomboid crystalline granules were identified ultrastructurally. The kidney adjacent to the tumor showed features of membranous glomerulonephritis and hypertensive arteriopathy. Proteinuria improved following tumor resection, but the patient had persistent hypertension attributable to renal hypertensive arteriopathy. This report also highlights the recently described observation of CD34 positivity in juxtaglomerular cell tumors.
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PMID:A case of juxtaglomerular cell tumor associated with membranous glomerulonephritis. 1457 36

Paraganglioma of the urinary bladder is a rare tumor with characteristic histologic and immunohistochemical features. However, in our experience, it may be misdiagnosed as urothelial cancer because of 1) its frequent involvement of the muscularis propria; 2) morphology that may suggest urothelial cancer in transurethral resection specimens, particularly if there are artifactual changes induced by that procedure; 3) failure of pathologists to include it in their differential diagnosis when evaluating a bladder tumor; and 4) only a minority of the cases are associated with symptoms that might prompt consideration of the diagnosis. Distinction between paraganglioma and urothelial cancer is important because of likely different therapeutic options. In this report, we describe our experience with the histopathology of paragangliomas of the urinary bladder with emphasis on the histologic features that have led to their being misdiagnosed as conventional urothelial cancer and, most importantly, those that will help pathologists recognize this rare tumor of the bladder. Fifteen cases of paraganglioma of the urinary bladder were studied, 11 of them consult cases. They affected patients (8 male, 7 female) with a mean age of 49.5 years; only two had symptoms suggestive of the diagnosis, including hypertension during cystoscopy and episodic headache. Three consult cases were submitted with a diagnosis of "transitional cell carcinoma" and 4 with a diagnosis only of "bladder tumor." Histologically, "zellballen" and diffuse patterns were present in 12 (80%) and 3 (20%) of the cases. A delicate fibrovascular stroma was obvious in 14 (93%) cases. Other patterns included irregular nests and pseudorosette formation. Tumor necrosis, significant cautery artifact, and muscularis propria invasion were present in 1 (7%), 3 (20%) cases, and 10 (67%) cases, respectively. All 15 tumors were composed of large polygonal cells with abundant granular cytoplasm. Focal clear cells were present in 3 (20%). The nuclei were mostly uniform, although occasional pleomorphic nuclei were seen in 6 (40%) cases, and 2 (13%) had frequent pleomorphic nuclei. Mitoses were rare overall, and no abnormal mitotic figures were found. The major histologic features that led to misdiagnosis included a diffuse growth pattern, focal clear cells, necrosis, and muscularis propria invasion, with significant cautery artifact compounding the diagnostic problems. Immunohistochemically, 2 of 2 tumors were positive for neuron-specific enolase, 9 of 10 tumors for chromogranin, and 2 of 3 tumors for synaptophysin; 3 of 3 tumors were negative for cytokeratin and 1 of 1 tumor negative for HMB-45. Paraganglioma of the urinary bladder may be misdiagnosed as urothelial cancer, but a careful search for the characteristic histologic features and, if necessary, supportive immunohistochemical studies, should lead to a correct diagnosis.
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PMID:Paraganglioma of the urinary bladder: a lesion that may be misdiagnosed as urothelial carcinoma in transurethral resection specimens. 1470 70

Pre-eclampsia is one of the most common causes of perinatal and maternal morbidity and mortality. High blood pressure and proteinuria are important clinical signs of pre-eclampsia. Sympathetic overactivity and elevated level of circulating vaso active substances, such as monoamines has been shown. Extracellular concentrations of monoamines are normally kept low by specific transporter proteins of which many are expressed in the placenta. In this study we used in situ hybridization and real-time PCR to study the gene expression of monoamine transporters, such as NET, SERT, VMAT2, EMT and OCT1/2, in normal as well as in pre-eclamptic placentae. We demonstrated high expression of NET mRNA in the trophoblast cells of the anchoring villi and a lower expression intensity in the chorionic villi. SERT mRNA was mainly detected in chorionic villi. VMAT2 mRNA was not detected in the central part of the placenta but was present in the spiral arteries of placenta bed biopsies, in cytokeratin positive cells. EMT mRNA was mainly detected in the intra lobular septa and together with OCT1 and OCT2 mRNAs also expressed in scattered cells of placental vessel adventitias. Moreover, quantitative analysis showed a significant lower expression of NET and EMT mRNAs in pre-eclamptic placentae as compared to the control group. A defective gene expression or function of these monoamines transporters might explain the elevated concentrations of monoamines in pre-eclamptic patients. Monoamine transporters may serve as a protective mechanism preventing vasoconstriction in the placental vascular bed and thereby securing a stable blood flow to the fetus.
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PMID:Norepinephrine transporter (NET), serotonin transporter (SERT), vesicular monoamine transporter (VMAT2) and organic cation transporters (OCT1, 2 and EMT) in human placenta from pre-eclamptic and normotensive pregnancies. 1513 35


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