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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)
The symptoms may be similar to those classically associated with renal cell carcinoma, and may include polycythemia, abdominal pain, hematuria 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 more commonly calcified than any other kidney neoplasm. Surgery is curative and no other treatment is recommended. There is so far no evidence of metastases or local recurrence.
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".
Wilms tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, rarely in adults. It is named after Dr. Max Wilms, the German surgeon (1867–1918) who first described it.
Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occur in otherwise normal children; a minority (25%) are associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years.
Patients typically present with a non-productive cough and weight loss.
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.
Kidney tumours (or kidney tumors), also known as renal tumours, are tumours, or growths, on or in the kidney. These growths can be benign or malignant (kidney cancer).
In addition to renal cell carcinoma and renal pelvis carcinoma, other, less common types of kidney cancer include:
- Squamous cell carcinoma
- Juxtaglomerular cell tumor (reninoma)
- Angiomyolipoma
- Bellini duct carcinoma
- Clear-cell sarcoma of the kidney
- Mesoblastic nephroma
- Wilms' tumor, usually is reported in children under the age of 5.
- Mixed epithelial stromal tumor
Rarely, some other types of cancer and potentially cancerous tumors that more usually originate elsewhere can originate in the kidneys. These include:
- Clear cell adenocarcinoma
- Transitional cell carcinoma
- Inverted papilloma
- Renal lymphoma
- Teratoma
- Carcinosarcoma
- Carcinoid tumor of the renal pelvis
Cancer in the kidney may also be secondary, the result of metastasis from a primary cancer elsewhere in the body.
The most common signs and symptoms of kidney cancer are a mass in the abdomen and/or blood in the urine (or hematuria). Other symptoms may include tiredness, loss of appetite, weight loss, a high temperature and heavy sweating, and persistent pain in the abdomen. However, many of these symptoms can be caused by other conditions, and there may also be no signs or symptoms in a person with kidney cancer, especially in the early stages of the disease.
One clinically significant subtype is "large-cell neuroendocrine carcinoma" (LCNEC), which is believed to derive from neuroendocrine cells.
In addition, a "subvariant", called "combined large-cell neuroendocrine carcinoma" (or c-LCNEC), is recognized under the new system. To be designated a c-LCNEC, the tumor must contain at least 10% LCNEC cells, in combination with at least 10% of other forms of NSCLC.
A ureteral neoplasm is a type of tumor that can be primary, or associated with a metastasis from another site.
Treatment may involve removal of the kidney and ureter, or just the ureter.
Classification of cancers often is oriented around the embryological origin of the tissue. In some contexts, the primary division is at the border of kidney and ureter, and in other contexts, the primary division is between derivatives of the metanephric blastema and those of the ureteric bud. Because of this, neoplasia of the ureters are sometimes grouped with tumors of the renal pelvis.
Adenocarcinomas are highly heterogeneous tumors. Several major histological subtypes are currently recognized by the WHO and IASLC/ATS/ERS
- Non-invasive or minimally invasive adenocarcinoma
- Adenocarcinoma in situ of the lung (Bronchioalveolar carcinoma)
- Minimally invasive adenocarcinoma of the lung
- Invasive adenocarcinoma
- Acinar predominant adenocarcinoma
- Papillary predominant adenocarcinoma
- Micropapillary predominant adenocarcinoma
- Solid predominant adenocarcinoma
- Invasive mucinous adenocarcinoma
In as many as 80% of tumors that are extensively sampled, components of more than one of these subtypes will be recognized. In such cases, resected tumors should be classified by comprehensive histological subtyping. Using increments of 5% to describe the amount of each subtype present, the predominant subtype is used to classify the whole tumor. The predominant subtype is prognostic for survival after complete resection.
Signet ring and clear cell adenocarcinoma are no longer histological subtypes, but rather cytological features that can occur in tumour cells of multiple histological subtypes, most often solid adenocarcinoma.
Some variants are not clearly recognized by the WHO and IASLC/ATS/ERS classification:
- Enteric adenocarcinoma of the lung
- Cribriform adenocarcinoma of the lung
Nearly 40% of lung cancers in the US are adenocarcinoma, which usually originates in peripheral lung tissue. Most cases of adenocarcinoma are associated with smoking; however, among people who have smoked fewer than 100 cigarettes in their lifetimes ("never-smokers"), adenocarcinoma is the most common form of lung cancer. Its incidence has been increasing in many developed Western nations in the past few decades, where it has become the most common major type of lung cancer in smokers (replacing squamous cell lung carcinoma) and in lifelong nonsmokers. According to the Nurses' Health Study, the risk of adenocarcinoma of the lung increases substantially after a long duration of previous tobacco smoking, with a previous smoking duration of 30 to 40 years giving a relative risk of approximately 2.4 compared to never-smokers, and a duration of more than 40 years giving a relative risk of approximately 5.
This cancer usually is seen peripherally in the lungs, as opposed to small cell lung cancer and squamous cell lung cancer, which both tend to be more centrally located, although it may also occur as central lesions. For unknown reasons, it often arises in relation to peripheral lung scars. The current theory is that the scar probably occurred secondary to the tumor, rather than causing the tumor. The adenocarcinoma has an increased incidence in smokers, and is the most common type of lung cancer seen in non-smokers and women. The peripheral location of adenocarcinoma in the lungs may be due to the use of filters in cigarettes which prevent the larger particles from entering the lung. Deeper inhalation of cigarette smoke results in peripheral lesions that are often the case in adenocarcinomas of the lung. Generally, adenocarcinomas grow more slowly and form smaller masses than the other subtypes. However, they tend to form metastases widely at an early stage. Adenocarcinoma is a non-small cell lung carcinoma, and as such, it is not as responsive to radiation therapy as is small cell lung carcinoma, but is rather treated surgically, for example by pneumonectomy or lobectomy.
Giant-cell fibroma is a type of fibroma not associated with trauma or irritation. It can occur at any age and on a mucous membrane surface. The most common oral locations are on the gingiva of the mandible, tongue, and palate. It is a localized reactive proliferation of fibrous connective tissue.
Giant-cell fibroma (GCF) is a benign non-neoplastic lesion first described by Weathers and Callihan (1974). It occurs in the first three decades of life and predominates in females (Houston, 1982; Bakos, 1992). Clinically, the GCF presents as an asymptomatic, papillary and pedunculated lesion. The most predominant location is the mandibular gingiva (Houston, 1982; Bakos, 1992). Histologically, the GCF is distinctive, consisting of fibrous connective tissue without inflammation and covered with stratified squamous hyperplastic epithelium. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells. These cells occur in a variety of lesions, such as the fibrous papule of the nose, ungual fibroma, acral fibrokeratoma, acral angiofibroma and desmoplastic fibroblastoma (Swan, 1988; Pitt et al., 1993; Karabela-Bouropoulou et al., 1999; Jang et al., 1999).
Despite many studies, the nature of the stellated multinucleate and mononuclear cell is not clear (Weathers and Campbell, 1974; Regezi et al., 1987; Odell et al., 1994; Magnusson and Rasmusson, 1995).
When a diagnosis of multicystic kidney is made in utero by ultrasound, the disease is found to be bilateral in many cases. Those with bilateral disease often have other severe deformities or polysystemic malformation syndromes. In bilateral cases, the newborn has the classic characteristic of Potter's syndrome.
The bilateral condition is incompatible with survival, as the contralateral system frequently is abnormal as well. Contralateral ureteropelvic junction obstruction is found in 3% to 12% of infants with multicystic kidney and contralateral vesicoureteral reflux is seen even more often, in 18% to 43% of infants. Because the high incidence of reflux, voiding cystourethrography usually has been considered advisable in all newborns with a multicystic kidney.
PCNA and Ki67 immunoreactivity happens in case of fibroma and peripheral granuloma.
Multicystic dysplastic kidney (MCDK) is a condition that results from the malformation of the kidney during fetal development. The kidney consists of irregular cysts of varying sizes. Multicystic dysplastic kidney is a common type of renal cystic disease, and it is a cause of an abdominal mass in infants.
Renal ectopia or ectopic kidney describes a kidney that is not located in its usual position. It results from the kidney failing to ascend from its origin in the true pelvis or from a superiorly ascended kidney located in the thorax.
It has an incidence of approximately 1/900.
Horseshoe kidney, also known as "ren arcuatus" (in Latin), renal fusion or super kidney, is a congenital disorder affecting about 1 in 600 people, more common in men.
In this disorder, the patient's kidneys fuse together to form a horseshoe-shape during development in the womb. The fused part is the isthmus of the horseshoe kidney.
Fusion abnormalities of the kidney can be categorized into two groups: horseshoe kidney and crossed fused ectopia. The 'horseshoe kidney' is the most common renal fusion anomaly.
While most cases of horseshoe kidneys are asymptomatic and discovered upon autopsy, the condition may increase the risk for:
- Kidney obstruction – abnormal placement of ureter may lead to obstruction and dilation of the kidney.
- Kidney infections – associated with vesicoureteral reflux.
- Kidney stones – deviant orientation of kidneys combined with slow urine flow and kidney obstruction may lead to kidney stones.
- Kidney cancer – increased risk of renal cancer, especially Wilms' tumor, transitional cell carcinoma, and an occasional case report of carcinoid tumor. Despite increased risk, the overall risk is still relatively low.
The prevalence of horseshoe kidneys in females with Turner Syndrome is about 15%.
It can be associated with trisomy 18.
It can be associated with venous anomalies like left sided IVC 9.
Uterine adenosarcoma, also adenosarcoma of the uterus, and Müllerian adenosarcoma of the uterus, is an uncommon form of cancer that arises from mesenchymal tissue of the uterus and has a benign glandular component.
Plasma cell granuloma is a lesional pattern of inflammatory pseudotumour, different from the "inflammatory myofibroblastic tumor" pattern.
It is linked to IgG4-related disease.
CFHR5 nephropathy usually presents with microscopic amounts of blood in the urine, detectable on a routine urine dipstick test. Sometimes the disease is associated with visible blood in the urine, usually at the time of respiratory or other infections and this is thought to result from stimulation of the immune system leading to damage in the kidneys.
Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent, potentially lethal, monogenic human disorder. It is associated with large interfamilial and intrafamilial variability, which can be explained to a large extent by its genetic heterogeneity and modifier genes. It is also the most common of the inherited cystic kidney diseases — a group of disorders with related but distinct pathogenesis, characterized by the development of renal cysts and various extrarenal manifestations, which in case of ADPKD include cysts in other organs, such as the liver, seminal vesicles, pancreas, and arachnoid membrane, as well as other abnormalities, such as intracranial aneurysms and dolichoectasias, aortic root dilatation and aneurysms, mitral valve prolapse, and abdominal wall hernias. Over 50% of patients with ADPKD eventually develop end stage kidney disease and require dialysis or kidney transplantation. ADPKD is estimated to affect at least 1 in every 1000 individuals worldwide, making this disease the most common inherited kidney disorder with a diagnosed prevalence of 1:2000 and incidence of 1:3000-1:8000 in a global scale.
Patients typically have no symptoms until the third or fourth decade of life. In most cases, the disease is discovered incidentally on routine chest Xray. The most common symptoms include the following:
- dyspnea
- dry cough
- chest pain
- sporadic hemoptysis
- asthenia
- pneumothoraces