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Juxtaglomerular Cell Tumor
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells, with less than 100 cases reported in literature. This tumor typically secretes renin, hence the former name of reninoma. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain. Pathophysiology By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. Both of these conditions may be corrected by surgical removal of the tumor. Asymptomatic cases have been reported. Histopathology JCT is morphologically characterized by multiple foci malignant mesenchy ...
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Kidney Tumour
Kidney tumours are tumours, or growths, on or in the kidney. These growths can be benign or malignant (kidney cancer). Presentation Kidney tumours may be discovered on medical imaging incidentally (i.e. an incidentaloma), or may be present in patients as an abdominal mass or kidney cyst, hematuria, abdominal pain, or manifest first in a paraneoplastic syndrome that seems unrelated to the kidney. Other markers or complications that may arise from kidney tumours can appear to be more subtle, including; low hemoglobin, fatigue, nausea, constipation, and/or hyperglycemia. Diagnosis A CT scan is the first choice modality for workup of solid masses in the kidneys. Nevertheless, hemorrhagic cysts can resemble renal cell carcinomas on CT, but they are easily distinguished with Doppler ultrasonography (Doppler US). In renal cell carcinomas, Doppler US often shows vessels with high velocities caused by neovascularization and arteriovenous shunting. Some renal cell carcinomas are hypovasc ...
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S100 Protein
The S100 proteins are a family of low molecular-weight proteins found in vertebrates characterized by two calcium-binding sites that have helix-loop-helix (" EF-hand-type") conformation. At least 21 different S100 proteins are known. They are encoded by a family of genes whose symbols use the ''S100'' prefix, for example, ''S100A1'', ''S100A2'', ''S100A3''. They are also considered as damage-associated molecular pattern molecules (DAMPs), and knockdown of aryl hydrocarbon receptor downregulates the expression of S100 proteins in THP-1 cells. Structure Most S100 proteins consist of two identical polypeptides (homodimeric), which are held together by noncovalent bonds. They are structurally similar to calmodulin. They differ from calmodulin, though, on the other features. For instance, their expression pattern is cell-specific, i.e. they are expressed in particular cell types. Their expression depends on environmental factors. In contrast, calmodulin is a ubiquitous and univer ...
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Karyotyping
A karyotype is the general appearance of the complete set of metaphase chromosomes in the cells of a species or in an individual organism, mainly including their sizes, numbers, and shapes. Karyotyping is the process by which a karyotype is discerned by determining the chromosome complement of an individual, including the number of chromosomes and any abnormalities. A karyogram or idiogram is a graphical depiction of a karyotype, wherein chromosomes are organized in pairs, ordered by size and position of centromere for chromosomes of the same size. Karyotyping generally combines light microscopy and photography, and results in a photomicrographic (or simply micrographic) karyogram. In contrast, a schematic karyogram is a designed graphic representation of a karyotype. In schematic karyograms, just one of the sister chromatids of each chromosome is generally shown for brevity, and in reality they are generally so close together that they look as one on photomicrographs as well ...
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Solitary Fibrous Tumor
Solitary fibrous tumor (SFT), also known as fibrous tumor of the pleura, is a rare mesenchymal tumor originating in the pleuraTravis WD, Brambilla E, Muller-Hermelink HK, Harris CC (Eds.): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. IARC Press: Lyon 2004. or at virtually any site in the soft tissue including seminal vesicle. Approximately 78% to 88% of SFT's are benign and 12% to 22% are malignant.Robinson LA. Solitary fibrous tumor of the pleura. Cancer Control 2006;13:264-9. The World Health Organization (2020) classified SET as specific type of tumor in the category of malignant fibroblastic and myofibroblastic tumors. Signs and symptoms About 80% of pleural SFTs originate in the visceral pleura, while 20% arise from parietal pleura.Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature" ''Cancer'' 1981;47:2678-89. Althou ...
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Wilms' Tumor
Wilms' tumor or Wilms tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, rarely in adults.; and occurs most commonly as a renal tumor in child patients. It is named after Max Wilms, the German surgeon (1867–1918) who first described it. Approximately 650 cases are diagnosed in the U.S. annually. The majority of cases occur in children with no associated genetic syndromes; however, a minority of children with Wilms' tumor have a congenital abnormality.  It is highly responsive to treatment, with about 90 percent of children being cured. Signs and symptoms Typical signs and symptoms of Wilms' tumor include the following: * a painless, palpable abdominal mass * loss of appetite * abdominal pain * fever * nausea and vomiting * blood in the urine (in about 20% of cases) * high blood pressure in some cases (especially if synchronous or metachronous bilateral kidney involvement) * Rarely as varicoceleErginel B, Vural S, Akın M, ...
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Angiomyolipoma
Angiomyolipomas are the most common benign tumour of the kidney. Although regarded as benign, angiomyolipomas may grow such that kidney function is impaired or the blood vessels may dilate and burst, leading to bleeding. Angiomyolipomas are strongly associated with the genetic disease Tuberous Sclerosis, in which most individuals have several Angiomyolipomas affecting both kidneys. They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis. Angiomyolipomas are less commonly found in the liver and rarely in other organs. Whether associated with these diseases or sporadic, Angiomyolipomas are caused by mutations in either the ''TSC1'' or ''TSC2 ''genes, which govern cell growth and proliferation. They are composed of blood vessels, smooth muscle cells, and fat cells. Large Angiomyolipomas can be treated with embolisation. Drug therapy for Angiomyolipomas is at the research stage. The Tuberous Sclerosis Alliance has published guidelines on diagno ...
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Metanephric Adenoma
Metanephric adenoma (MA) is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma (Wilms tumours), or a papillary renal cell carcinoma. It should not be confused with the pathologically unrelated, yet similar sounding, '' mesonephric adenoma''. Symptoms The symptoms may be similar to those classically associated with renal cell carcinoma, and may include polycythemia, abdominal pain, hematuria Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. “Gross hematuria” occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable w ... and a palpable mass. Mean age at onset is around 40 years with a range of 5 to 83 years and the mean size of the tumour is 5.5 cm with a range 0.3 to 15 cm (1). Polycythemia is more frequent in MA than in any other type of renal tumour. Of further relevance is that this tumour is mo ...
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Glomus Tumor
:''Glomus tumor was also the name formerly (and incorrectly) used for a tumor now called a paraganglioma.'' A glomus tumor (also known as a "solitary glomus tumor," "solid glomus tumor,") is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . They account for less than 2% of all soft tissue tumors. The majority of glomus tumors are benign, but they can also show malignant features. Glomus tumors were first described by Hoyer in 1877 while the first complete clinical description was given by Masson in 1924. Histologically, glomus tumors are made up of an afferent arteriole, anastomotic vessel, and collecting venule. Glomus tumors are modified smooth muscle cells that control the thermoregulatory function of dermal glomus bodies. As stated above, these lesions should not be confused with paragangliomas, which were former ...
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Hemangiopericytoma
A hemangiopericytoma is a type of soft-tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with a special aggressive behavior. It was first characterized in 1942. Signs and symptoms Symptoms of hemangiopericytoma vary greatly depending on both tumor stage and affected organs. Most patients report pain and mass-related symptoms, while others also report vascular disease-related symptoms, and some have no symptoms until late in the disease process. Hemangiopericytomas are most commonly found in the meninges, lower extremities, retroperitoneum, pelvis, lungs, and pleura. Histopathology Hemangiopericytomas are tumors that are derived from specialized spindle shaped cells called pericytes, which line capillaries. Hemangiopericytoma located in the cerebral cavity is an aggressive tumor of the mesenchyme with oval nuclei with scant cytoplasm. "There is dense inter ...
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Differential Diagnosis
In healthcare, a differential diagnosis (abbreviated DDx) is a method of analysis of a patient's history and physical examination to arrive at the correct diagnosis. It involves distinguishing a particular disease or condition from others that present with similar clinical features. Differential diagnostic procedures are used by clinicians to diagnose the specific disease in a patient, or, at least, to consider any imminently life-threatening conditions. Often, each individual option of a possible disease is called a differential diagnosis (e.g., acute bronchitis could be a differential diagnosis in the evaluation of a cough, even if the final diagnosis is common cold). More generally, a differential diagnostic procedure is a systematic diagnostic method used to identify the presence of a disease entity where multiple alternatives are possible. This method may employ algorithms, akin to the process of elimination, or at least a process of obtaining information that shrinks th ...
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Renal Cell Carcinoma
Renal cell carcinoma (RCC) is a kidney cancer that originates in the lining of the Proximal tubule, proximal convoluted tubule, a part of the very small tubes in the kidney that transport primary urine. RCC is the most common type of kidney cancer in adults, responsible for approximately 90–95% of cases. RCC occurrence shows a male predominance over women with a ratio of 1.5:1. RCC most commonly occurs between 6th and 7th decade of life. Initial treatment is most commonly either partial or complete removal of the affected kidney(s). Where the cancer has not metastasised (spread to other organs) or burrowed deeper into the tissues of the kidney, the five-year survival rate is 65–90%, but this is lowered considerably when the cancer has spread. The body is remarkably good at hiding the symptoms and as a result people with RCC often have advanced disease by the time it is discovered. The initial symptoms of RCC often include haematuria, blood in the urine (occurring in 40% of af ...
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