What are nephrogenic rests?
Nephrogenic rests (NR) are abnormally persistent clusters of embryonal cells, representing microscopic malformations (dysplasias) of the developing kidney. Though NR are best known as precursors of Wilms tumor (WT), many alternative fates are observed, and most rests are destined for eventual atresia.
What is the most common presenting feature of Wilms Tumour?
The most common manifestation of Wilms tumor is an asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at presentation. Abdominal pain or hematuria occurs in 25%.
Which diagnostic test is used to confirm a Wilms tumor?
Ultrasound (sonogram) Ultrasound is often the first imaging test done if the doctor suspects your child has a tumor in the abdomen.
What is Wilms tumor associated with?
Wilms’ tumor is associated with several congenital syndromes such as WAGR (Wilms’ tumor, aniridia, genitourinary malformation, mental retardation) syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndrome, etc.
What is Denys Drash Syndrome?
Denys-Drash syndrome is characterized by kidney disease that begins within the first few months of life. Affected individuals have a condition called diffuse glomerulosclerosis, in which scar tissue forms throughout glomeruli, which are the tiny blood vessels in the kidneys that filter waste from blood.
What is Mesoblastic Nephroma?
Mesoblastic nephroma (also called fetal renal hamartoma) is the most common renal tumor identified in the neonatal period and the most frequent benign renal tumor in childhood. It represents 3-10% of all pediatric renal tumors. This tumor was first described as a separate entity by Bolande et al in 1967.
Why is nephroblastoma called Wilms?
Wilms’ tumor, also known as nephroblastoma, is a cancer of the kidneys that typically occurs in children, rarely in adults. 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.
What causes nephroblastoma?
It’s not clear what causes Wilms’ tumor, but in rare cases, heredity may play a role. Cancer begins when cells develop errors in their DNA. The errors allow the cells to grow and divide uncontrollably and to go on living when other cells would die. The accumulating cells form a tumor.
How is nephroblastoma treated?
Treatment for Wilms’ tumor usually involves surgery and chemotherapy, and sometimes radiation therapy.
What is Ureterovesical reflux?
Vesicoureteral reflux (VUR) is when pee moves backward from the bladder to the kidneys. Normally, pee flows from the kidneys down to the bladder. Kids with mild cases of VUR often don’t need treatment. Those with more serious symptoms might need to take antibiotics to prevent infection.
What is a nephroblastoma?
Wilms tumor (also called Wilms’ tumor or nephroblastoma) is a type of childhood cancer that starts in the kidneys. It is the most common type of kidney cancer in children. About 9 of 10 kidney cancers in children are Wilms tumors. Cancer starts when cells in the body begin to grow out of control.
What is Galloway Mowat syndrome?
Galloway-Mowat syndrome is an extremely rare genetic disorder that is characterized by a variety of physical and developmental abnormalities, particularly neurological abnormalities and early onset progressive kidney disease.
What is nephrogenic rest Wilms tumor?
Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms’ tumor A new classification and terminology is proposed for precursor lesions of Wilms’ tumor (WT), based upon morphology and natural history. The generic term nephrogenic rest (NR) is used for all WT precursors.
What is Wilms tumor?
Definition / general Nephroblastoma (or Wilms tumor) is a malignant embryonal tumor originating from nephrogenic blastema, which imitates the histology of developing kidney Primarily occurs in children Named after the German surgeon Max Wilms (who is often wrongly attributed to be the first one describing this entity)
What is a nephrogenic rest?
Nephrogenic rests are the consequence of residual metanephric tissue in a fully developed kidney. They usually occur along the perimeter of a mature renal lobe (i.e., perilobar), within the lobe itself (i.e., intralobar), or both (i.e., combined). Nephrogenic rests can be grossly obvious or microscopically discrete.
What is the difference between Universal nephroblastomatosis and nephrogenic rest?
Nephroblastomatosis designates nephrogenic rests that are multifocal or diffuse, and implies more extensive disease. Universal (panlobar) nephroblastomatosis denotes complete replacement of the renal lobe by nephrogenic tissue.