38. Renal parenchymal neoplasms Flashcards
what are the big differences of renal parencymak tumors between children and adults
Adults
most common renal parenchymal tumour Renal cell carcinoma AKA :Grawitz tumour, adenocarcinoma of the renal cortex, hypernephroma is inaccurate term
Children
most common renal parenchymal tumour is Wilms tumour or nephroblastoma
epidemiology of renal paranchymal tumors
- 1/3 is at advanced stage at the time of diagnosis
- e.g Rcc= metastatic
- Lcoally advanced tumor?
- 1/3 only develop metastasis during follow up period
RF’s of renal parenchymal tumours
GO & SHOC your kidneys to death
which is most dramatic
- Smoking î risk 3x
- Hypertension
- Obesity
- Cyclophosphemide→ chemotherapeutic that suppresses the immune usystem
- Genetic predisposition: Birt Hogg Dube &, Von Hippel-Lindau
- Occupational risk factors
Histogenesis of renal cell carcinoma
where do they originate
what is the histological type of RCC
Renal cell carcinoma originate from
- Proximal Convoluted Tubules
- very rarely from Collecting duct of Bellini.
Histology of renal cell carcinoma is adenocarcinoma
Gross appearance of renal cell carcinoma
cut section = is yellow due to lipid cytoplasm
origin of nephroblastoma
histological types of nephroblastoma
gross section of nephroblastoma
prognosis of mixed tumor
named after german surgeon max wilms
Nephroblastoma is from mesodermal origin,
has many histological types so is a mixed tumour;
Therefore tumour contains: bones, cartilage, muscles, nerves, vessels and connective tissue
gross section of nephroblastoma
Cut section of the tumour - tumour is like brain
it is a Mixed tumour has usually unfavourable prognosis
what is The new WHO histological classification of renal tumours 2016 - NB.
types of adenocarcinoma
a histological classification by who in 2016 referring to the histlogical subtype of renal cell carcinoma
RCC histological type in adult is adenocarcinoma - which adenocarcinoma according to the new classification may present several histological subtypes:
- Clear cell RCC (80%),
- chromophobe RCC,
- papillary RCC,
- Bellini duct carcinoma (malignant cells in collecting duct system)
most tumours are heterogenous =there are many histological subtypes and they have differences in their biological behavior
what does familial RCC mean
what does true heredity of rcc mean
what are the 2 genetic syndromes of heriditary RCC
how does Hereditary rcc present
what is the mc type of tumour of BHD
what is indicative of Genetic RCC
Familial RCC is when there is significant high risk for occurrence and development of RCC in first line relatives
True heredity RCC is based on 2 specific genetic syndromes
- Von hippel Lindau syndrome — clear cell RCC
- Birt-Hogg-Dube syndrome — chromophobe RCC,
- NB this genetic syndrome, very often has tumours containing several histlogical subtypes.
- Most common is a hybrid tumour w/ more than 1 histological subtype
- NB this genetic syndrome, very often has tumours containing several histlogical subtypes.
Hereditary RCC show a tendency to be
- multiple and bilateral location,
- present at earlier age of onset → indicates genetic investigation
- bad prognosis
what is a REnal Oncocytoma
what is it’s associating symptom
what cells are found
- a benign renal epithelial tumour,
- usually manifests with hypertension ,
- contains eosinophilia cells
what is the histological grading system of RCC
Fuhrmann grading system
- defines the degree of malignancy of RCC
- it is based on nuclear changes according to the degree of nuclear atypism
G1: well differentiated (L_ow grade)_
G2: moderately differentiated (L_ow grade)_
G3: Poorly differentiated/ anaplastic (High grade)
when is the clincal and pathological staging required for RCC
how is RCC staged
clinical and pathological staging is done for each malignancy
done using TNM classification 2017
expand on the clinical staging of RCC
what does it do
when is it used
- stages the tumour using TNM
- determines the type and extent of the treatment
- Radical or Palliative
expand on pathological staging
when is it used
what is it’s purpose
- used to define TNM after surgery
- it determines the final histological report after surgery
how does RCC present ( 3 MAIN SX)
what is the LHP classic triad (11%) and what do they suggest
what is a paraneoplastic syndrome and why are they important
give 2 examples of paracrine effects
- 11% of pts present w/ classic triad symptoms
- 1) Loin pain, 2) Hematuria, 3)Palpable mass
- Clinical interpretation of these symptoms =
- Advanced renal tumour w/ poor prognosis
-
Paraneoplastic syndrome = specific form
- caused by systemic toxic effect of the tumour
- Components include: -fever, -unexplained anaemia, -hypertension, -elevated ESR, -paracrine effects
- Paracrine effects: RCC is a hormone acting tumour producing substances similar to 1) parathormone leading to hypercalcemia 2) erythropoietin leading to polycythaemia
- Paraneoplastic symptoms are non specific symptoms, so they present in early stages which us important as For each malignancy is important to detect it at this early stage
-
Scrotal pain from Varicocele of RIght Testicle
- caused by compression of the right testicular vein that drains directly into the IVC
- clinical interpretation = locally advanced tumor
- caused by compression of the right testicular vein that drains directly into the IVC
Paraneoplastic syndrome components aka specific sx of RCC
clinical presentation
- fever,
- unexplained anaemia,
- hypertension,
- elevated ESR,
- paracrine effects
clinical indication of early stage of disease
Wilms tumour clinical presentation
which tissues are affected and why
what is the main sx of wilm’s
- Histogenesis from mesoderm origin thus may affect any tissue with mesodermal origin e.g.
- bones, (CT)
- cartilage,
- nerves
- Main symptom is huge palpable abdominal mass or asymmetric abdomen,
