20-8 (Neoplasms) Flashcards
What are 3 benign neoplasms of the kidney?
- Papillary Adenoma
- Angiomyolipoma
- Oncocytoma
What are 3 benign neoplasms of the kidney?
- Papillary Adenoma
- Angiomyolipoma
- Oncocytoma
What malignant neoplasm is the Papillary Adenoma histologically the same as?
Papillary Renal Cell Carcinoma
The Papillary Adenoma is less than 1.5 cm in diameter and is present in the ____
Cortex
Angiomyolipoma’s are common in patients with what disease and what are the symptoms?
Tuberous Sclerosis
= Epilepsy, low IQ, skin abnormalities and other benign tumors
What are Angiomyolipomas made of and what are they at a risk for?
Vessels, smooth muscle and fat
–> risk for spontaneous hemorrhage
Patients with epilepsy, low IQ, skin abnormalities and other benign tumors have what condition and are at risk for what benign renal neoplasm?
Tuberous Sclerosis
– Angiomyolipoma
Oncocytoma’s are composed of what cells and what is unique about what is inside those cells?
Eosinophilic cells with large nucleoli and lots of mitochondria
This benign neoplasm contains lots of eosinophilic cells with large nucleoli and lots of mitochondria
Oncocytoma
How do Oncocytoma’s look to the naked eye?
Tan-brown, homogenous with a central scar
What are 3 common malignant neoplasms of the kidney?
- Renal Cell Carcinoma
- Urothelial Carcinoma of the renal pelvis
- Wilms tumor
What are 3 common malignant neoplasms of the kidney?
- Renal Cell Carcinoma
- Urothelial Carcinoma of the renal pelvis
- Wilms tumor
Where in the kidney do Renal Cell Carcinomas usually affect?
Poles
In what people/conditions are Renal Cell Carcinomas seen more often?
Male smokers
Obese women
Increased Estrogen
HTN
Renal Cell Carcinomas have a tendency to invade what structures and what does that look like?
Renal Vein
–> extend up to the IVC as a solid column of cells
There are 4 syndromes that are inherited Autosomal Dominantly which produce Renal Cell Carcinomas. What are those syndromes?
- Von Hippel - Lindau
- Hereditary Leiomyomatosis
- Hereditary Papillary Carcinoma
- Birt-Hogg-Dube Syndrome
Hereditary Autosomal Dominant syndromes that produce Renal Cell Carcinomas usually involve one or multiple carcinomas?
Multiple
– Sporadic=single
Von Hippel Lindau Syndrome has what gene mutation and what malignancy in the kidney?
VHL gene
Renal Cell Carcinoma
Hereditary Leiomyomatosis involve what gene mutation and what renal malignancy?
FH (fumarate hydratase) mutations
Renal Cell Carcinoma
Hereditary Papillary Carcinoma involves what gene mutation and what renal malignancy?
MET mutations
Renal Cell Carcinoma
Birt Hogg Dube Syndrome involves what gene mutations and what renal/other malignancy?
BHD (folliculin) mutations
Renal Cell Carcinoma, Lungs and skin
What are the 4 major types of Renal Cell Carcinomas?
- Clear cell
- Papillary
- Chromophobe
- Xp11 translocation
What are the 4 major types of Renal Cell Carcinomas?
- Clear cell
- Papillary
- Chromophobe
- Xp11 translocation
Clear Cell Carcinomas involve what gene changes?
Deletion of the short arm of chromosome 3 that involves the VHL gene
Clear Cell Carcinomas have a deletion of the VHL gene, which normally inhibits what things?
HIF1, VEGF, IGF1 that all promote growth
Clear Cell Carcinomas arise from what epithelium and appear what color and why?
- Proximal tubule epithelium
- Appear yellow due to lipid accumulation
This type of Renal cell carcinoma has a deletion of chromosome 3, and is yellow due to lipid accumulation?
Clear Cell Carcinoma
Papillary Carcinomas have what gene changes?
Trisomies of 7 and 17 and possibly deletion of Y in males
This type of Renal cell carcinoma involves trisomies of 7 and 17 and possibly deletion of Y chromosome in males?
Papillary Carcinoma
Papillary Carcinomas have trisomies of 7 and 17 commonly. What mutations does that cause to arise?
MET mutations that promote HGF activity
Papillary Carcinomas arise from where in the nephron and what do they look like?
Arise from DCT
– look hemorrhagic
What 2 things are commonly seen in Papillary Carcinomas?
Foam cells
Psammoma bodies
Chromophobe Carcinomas have what types of cells?
Pale eosinophilic cells with a halo around the nucleus
Pale eosinophilic cells with a halo around the nucleus is likely what Renal Cell Carcinoma?
Chromophobe Carcinoma
This Renal Cell Carcinoma is commonly hypodiploidy?
Chromophobe Carcinoma
Xp11 Translocation Carcinomas occur in what age patients and have what overexpression?
Young patients with overexpression of TFE3
Xp11 Translocation Carcinomas occur in what age patients and have what overexpression?
Young patients with overexpression of TFE3
What are 3 common symptoms of Renal Cell Carcinomas?
Costovertebral pain
Palpable mass
Hematuria
Renal Cell Carcinomas often go unnoticed, so they are usually large when discovered. Besides hematuria, what other changes may be common?
Abnormal hormones, HTN, and other molecular changes
Renal Cell Carcinomas commonly metastasize before they produce any clinical signs. What 2 locations are common sites of metastasis?
Lungs
Bone
Urothelial Carcinomas of the renal pelvis are clinically apparent within a short time. Why?
Produce hematuria and can block urine outflow
What are some possible symptoms of a Urothelial Carcinoma?
Hematuria
Flank pain
Hydronephrosis
Urothelial Carcinomas of the renal pelvis are associated with what things?
Bladder tumors
Lynch Syndrome
Urothelial Carcinomas of the renal pelvis are small on discovery. Why do they have poor prognosis?
Invade calyces and renal pelvis wall
Wilms tumors affect children between 2-5 years old. What are 3 common syndromes that they present with?
WAGR
Denys Drash
Beckwith Wiedemann
WAGR
Wilms tumor
Aniridia
Genitourinary Abnormalities
Retardation
What genes are deleted with WAGR?
WT1 and PAX6
Denys - Drash Syndrome
Gonadal Dysgenesis
Early onset renal failure
– wilms tumor
Beckwith - Wiedemann Syndrome
Organomegaly
Macroglossia
Hemihypertrophy
Large cells in adrenal cortex
What cell types are present with Wilms tumors?
Sheets of small blue cells
- blastemal, stromal, epithelial cells present