RCC

Renal parenchymal neoplasm

Benign tumor
1-Renal adenoma
-most common benign renal parenchymal lesion.
-These are small well differentiated glandular tumor of renal cortex.
-they are usually asymptomatic & discovered incidentally
-No clinical, histological, or immune histochemical criteria differentiate adenoma from carcinoma.
-renal tumors less than 3cm usually were considered adenoma and had little propensity for metastasis
2-Renal oncocytoma
-Has spectrum of behavior ranging from benign to malignant.
-Composed of large epithelial cell with eosinophilic cytoplasm (oncocyte cell).
-Gross hematuria or flank pain occur in less than 20% of patients.
-No characteristic features of the tumor appear on CT, U/S, IVU, or MRI.
-Angiographic features including (spoke wheel) appearance of the arterioles.
3-Angeomyolipoma(AML) or renal hamertoma
-They are characterized by 3 histologic component fat cell, smooth muscle & blood vessels.
-Usually (about 45-80% of AML) associated with tuberous sclerosis.
-Negative density -20 to -80 hounsfield units in CT pathognomonic for AML.
-Treatment depend on
1-size
2-tuberous sclerosis
3-size of lesion by CT (4cm more or less)


4-Other rare tumor like
-leiomyoma,
-hemangioma,
-renal lipoma &
-juxtaglomerular cell tumor (renin secreting tumor) which is always benign
Adenocarcinoma of the kidney
Renal cell ca
-most commonly in the 5th-6th decade (m:f ratio 2:1)
-The cause of RCC remain unknown.
-It originate from the proximal convoluted tubule of the cortex & tend to grow out into the perinephric tissue.
Histologically
-most often mixed adenocarcinoma containing clear cells, granular cells, and occasionally, sarcomatoid appearing cells
RCCs are vascular tumors
Spread
direct invasion through renal capsule
direct extension into the renal vein.
-25-30% of patient have evidence metastatic disease
at presentation.
Risk Factores
1-smoking is only definitive risk factor
2-occupational
3- genetic
4-Acquired renal cystic disease
Tumor grading & staging
The ultimate goal of staging is to select appropriate therapy & obtain prognostic information.
Stage 1—tumor is confined within the renal parenchyma.
Stage 2—tumor is confined within the gerota fascia
(including perinephric fat & adrenals).
Stage 3a—tumor involve main renal vein or IVC.
3b—tumor involve regional LN.
3c—tumor involve both local vessel & regional LN.
Stage 4a—tumor involve adjacent extragerotal organs
(colon, pancreas, etc).
Stage 4b—distant metastases.
Grading are 4 grades from well differentiated to undifferentiated
Symptoms & signs
1-The classical triad of gross hematuria, flank pain, &palpable mass occur in 7-10 % of patients & frequently manifestation of advance disease.
2-60% of pt present with gross or microscopic hematuria.
3-Pain abdominal mass or both occur in 40% of pt.
4-Symptoms secondary to metastases dyspnea, cough, seizure, headache, or bone pain.
-Renal tumor increasingly discovered incidentally due to the use of CT more than 50%
Paraneoplastic syndrome
occur in 10-40% of RCC patient.
It include erthrocytosis, hypercalcaemia, hypertension and non metastatic hepatic dysfunction
It does not indicate apoor prognosis
Usually relieve after nephractomy
Laboratory finding
1-Anemia 30%
2- increse ESR.
Imaging
U/S : it highly accurate in distinguishing simple cyst from solid lesion
CT :
It more sensitive than U/S and IVP
show renal mass that enhanced with contrast
CT is method of staging
MRI :to evaluate vascular invasion
Angiography
Radionuclide imaging
Management
Mainly depend on the stage of the tumor
Localized—Radical nephrectomy when kidney, perirenal fat & adrenal gland removed .
Disseminated—30% of pt present with metastases usually aggressive & rapidly progressive.
Palliative surgery
Radiotherapy,
Hormonal therapy,
Chemotherapy & biologic response modifier like interferon & interleukin
Observation.
Notce prognosis is mainly depend on performance state


Nephroblastoma
(Wilms tumor)

Most common solid renal tumor of childhood

peak age for presentation is the 3rd year of life
there is no sex predilection
10% have congenital malformation like aniridia and genitourinary abnormalities.
Pathology
Tumour precursor lesion is nephrogenic rests
Typically consist of blastemal,epithelial,and stromal
It of 2 types :Favorable contain no anaplasia and
Un favorable containing anaplasia
Metastases
Diract Hematogenouse Lymphatic
Clinical finding
Asymptomatic mass is the most common presentation discovered by the family member or physician.
abdominal pain, distension, nausea, vomiting, anorexia, fever.
The most common sign is abdominal mass
Hypertension
Hematuria
laboratory—hematuria & anemia
Imaging
U/S—is the current initial study of choice to evaluate palpable abdominal masses.
CT—useful in providing tumor extension, state of contralateral kidney &LN involvement.
IVU—to evaluate renal masses, but had been replaced by newer modality.
Chest x-ray—to evaluate the presence of lung metastases


*Needle biopsy—indicated if
Tumor too large for resection
For which chemotherapy or radiotherapy is planned.
D.Dx
Hydronephrosis.
Cystic kidney.
Neuroblastoma.
Treatment
1-surgical measure, radical nephrectomy
2-chemptherapy, wilms tumor is chemosesitive.
3-radiotherpy, its also radiosensitive