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Related Concept Videos

Nephrons01:10

Nephrons

The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma happens...
Renal Corpuscle01:20

Renal Corpuscle

The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
Diabetic Nephropathy01:28

Diabetic Nephropathy

Definition Diabetic nephropathy is a chronic kidney complication that results from prolonged hyperglycemia.Prevalence It is the most common cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide, affecting up to half of individuals with diabetes.Pathophysiology • Sustained hyperglycemia triggers multiple hemodynamic and metabolic changes in the kidney. • Early in the disease, increased renal blood flow and glomerular hyperfiltration occur due to afferent arteriolar...
Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the daughter...

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Related Experiment Video

Updated: Jul 4, 2026

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
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Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

Diffuse hyperplastic perilobar nephroblastomatosis.

Jimena Vicens1, Alejandro Iotti, Mercedes Garcia Lombardi

  • 1Pathology Department, Center of Medical Education and Clinical Investigation, Av. Galván 4102, Buenos Aires, Argentina. jimena_v@yahoo.com

Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
|July 4, 2008
PubMed
Summary
This summary is machine-generated.

Nephroblastomatosis, a rare preneoplastic lesion, presents as nephrogenic rests. This case highlights diffuse hyperplastic perilobar nephroblastomatosis in a child, emphasizing diagnostic and therapeutic challenges.

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Area of Science:

  • Pediatric Nephrology
  • Oncology
  • Pathology

Background:

  • Nephroblastomatosis is a rare preneoplastic lesion characterized by nephrogenic rests, classified into perilobar, intralobar, combined, and universal types.
  • It represents a spectrum of renal abnormalities with potential to develop into Wilms tumor.
  • Understanding its subtypes is crucial for accurate diagnosis and management.

Observation:

  • A case of diffuse hyperplastic perilobar nephroblastomatosis in a 1-year-old boy presenting with an abdominal mass.
  • Imaging revealed a homogeneous, enlarged left kidney.
  • Fine needle aspiration cytology suggested Wilms tumor, necessitating further investigation.

Findings:

  • Nephrectomy specimen showed a thick cortical rim of hyperplastic nephrogenic tissue, distinct from preserved renal parenchyma.
  • The lesion was well delineated without a pseudocapsule, characteristic of perilobar nephroblastomatosis.
  • Histopathological confirmation of diffuse hyperplastic perilobar nephroblastomatosis.

Implications:

  • Diagnosis relies on advanced imaging (CT, MRI), with fine needle aspiration cytology having limited value.
  • Therapeutic management remains controversial, often involving neoadjuvant chemotherapy followed by surgical excision for refractory cases.
  • This case underscores the importance of accurate pathological diagnosis in managing nephroblastomatosis and its potential malignant transformation.