Renal Tumors Hadeel Eissa

Yasmin Mansour Wafaa Hammodi

Benign Lesions of the Kidney

What is a Benign Lesion ? A benign lesion is a non-cancerous growth that does not spread (metastasize) to other parts of the body and usually is not life-threatening. Many researchers believe that most of the following types of kidney tumors are benign. However, others feel that some of these tumors have the potential to develop into renal cell carcinoma.

There are no diagnostic tests that can confirm if a benign tumor has the potential to become cancerous.

Benign tumors are sometimes found along with renal cell carcinoma when a removed kidney is examined by a pathologist. For these reasons, benign kidney tumors are treated like malignant tumors.

Clinical picture: Renal Cyst mostly asymptomatic , may be found as mass on routine clinical examination.If very large cyst may be present as aching pain in loin Usually initial imaging study that detects such a cyst is commonly ultrasonography, which is usually performed for reasons unrelated to the urinary tract

Bosniak classification

It is most useful and widely employed method for characterizing renal cystic lesions.

Cysts are graded according to bosniak grading system which attempt to predict their malignancy potential and to helpful in determine requirement for follow up or treatment.

Bosniak classification based on CT appearanceBased on evaluation of:

1 -wall of the lesion : its thickness and contour2 -septum : if present : number , thickness and

contour.3 -calcification : if present: amount , location

and character.4 -density of fluid in lesion .

5 -enhancement with contrast. 6 -margin of lesion : sharp , poorly defined.

7 -presence of solid component in lesion .

Category /Bosniak I – A benign simple renal cyst or multiple renal cystseach with a thin wall without septa, calcifications, or solid components .

The cyst has the density of water and does not enhanceMalignancy risk ~ 0%Work up : null – no need for follow up

Category /Bosniak II minimally complex Thin wallfew thin septa(<1mm)

the wall or septa may contain fine calcifications.

This category also includes uniformly high-attenuation lesions(hyperdense) that are <3 cm in diameter, well defined margin, and non enhancing.

Malignancy risk ~0%Null – no need for follow up Work up:

Category /Bosniak IIF Minimally complex but require follow up–

Cysts in this category are generally with wall :smooth thickening

Septa : multiple thin septa or smooth thickening which may contain calcification that may also be thick and nodular.This category also includes uniformly high-attenuation lesions(hyperdense) that are >3 cm in diameter, well defined margin, and non enhancing.malignancy risk : 5-20% Work up : follow up US/ CT

Category /Bosniak III – Indeterminate cystic masses that have thickened irregular or smooth walls or septa. With irregular or nodular calcification.

This category also includes uniformly high-attenuation lesions(hyperdense) that are >3 cm in diameter, with measurable enhancement.

Malignancy risk 40 to 60%.Treatment : partial nephrectomy or Radiofrequency ablation in elderly or poor surgical candidate.

Category / Bosniak IV –  cyst are clearly malignant and have all the

characteristics of category-III cysts, plus they contain enhancing soft-tissue components that are adjacent to and independent of the wall or septum

Malignancy risk 85 to 100% Treatment : partial or total nephrectomy

Many people with a benign kidney tumor are diagnosed when having a imaging test for other medical reasons.Approximately 25% of incidental found renal tumors are benign

Imaging tests cannot tell the difference between a benign kidney tumors and a malignant renal cell

carcinoma . With exception of Angiomyolipomas , which can be identified on non contrast CT Scan .

Benign Solid tumors

Clinical picture:

Most people with a benign kidney tumor do not have any signs and symptoms.

Symptoms may appear once the tumor grows into surrounding tissues and organs.blood in the urine (hematuria)pain in the back and side of the abdomen (flank)lump in the abdomen.

tests will be done to make a diagnosis. Tests may include:computed tomography (CT) scanultrasoundAny solid tumor that is >1 cm we deal with it as a malignant tumor until proven otherwise.

-if the solid tumor was <1 cm we can follow it up to

6 months till we are sure its benign.

biopsyA biopsy may be done if the doctor suspects that the tumors in the kidney is an oncocytoma, to rule out the presence of an aggressive malignant tumor.Biopsy is not done on angiomyolipoma, because of the possibility that the tumor may bleed.

TreatmentTreatment options for benign kidney tumors may include:active surveillance – for tumors causing no symptoms that are confirmed to be benignno treatment is offered, but the person is followed with imaging testssurgery – for tumors causing symptoms or tumors that grow after being watched with imaging testsradical nephrectomypartial (kidney-sparing) nephrectomy

Oncocytoma Histopathology● Oncocytomas are believed to originate from

intercalated tubular cells of the collecting tubules.

Oncocytoma cont'd

It is a relatively benign renal tumour.Unfortunately, the imaging appearance of

oncocytomas is difficult to distinguish from renal cell carcinoma.

The only reliable feature is evidence of metastasis or aggressive infiltration into adjacent structures, in which case the diagnosis of renal cell carcinoma can be safely made.

On angio, spoke-wheel apearance.

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Oncocytoma

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חל

Diagnosis

Pecrcutaneous Bx, core Bx, FNA.In gross appearance : Mass is tan to brown,

well-circumscribed, with pseudocapsule.Central stellate scar.

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Renal cortical adenoma

Imaging

● - Any solid tumor that is >1 cm we deal with it as a malignant tumor until proven otherwise. - if the solid tumor was <1 cm we can follow it up with 6 months till we are sure its benign.

They appear small, characterized by uniform basophilic or eosinophilic cells with benign appearing nuclear and cellular features.

Angiomyolipoma

(By Imaging) Diagnosis

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The cornerstone of diagnosis on all modalities is the demonstration of macroscopic fatHowever, in the setting of hemorrhage, or when lesions contain little fat, appearance may be difficult to distinguish from a renal cell carcinoma.Most lesions involve the cortex and demonstrate macroscopic fat.It is important to realise that a proportion of angiomyolipomas are fat-poor.Calcification is rare.

Angiomyolipoma

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Portal venous phaseHypodense mass proved to be fat by measuring the HU ( Hounsfield Unit) .

Histopathology

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Blood Vessels + Spindle Cells + Adipose tissue

Angio + Myo + Lipoma

Malignant Tumors

AdenocarcinomaTransitional cell carcimomaWilm’s tumorLymphomaSarcoma

Adenocarcinoma

Adenocarcinoma-RCC

Most common solid renal tumor -85%Originates from proximal renal tubular epitheliumMainly adults 40-60 of years of age, 3:1 male to female ratio; 5% of cancers overall in adults.

Etiology: like any other tumor is unknown.Risk Factors:Male sex, Smoking, or Family History.

Pathogenesis: this tumor arises from the parenchymal part of the kidney and mainlyfrom the proximal tubules. (And that’s why they used to call it hypernephroma).

Histological Types: the most common histological type is the clear cell carcinoma.Other types include papillary type, sarcomatoid type, chromophilic type, and chromophobic type.

History: age, sex, smoker or notClinical presenration:

incidental finding-most common

·Hematuria (40%) ·Flank pain (40%)

·Palpable mass in the flank or abdomen (25%)

Symptoms due to metastasisWhats the most common site?Lung History points

back pain jaundicecough, hemoptysis,dyspnea

Weight loss, fatigue, night sweats, fever, malaise

Paraneoplastic syndromePatient may be presented with Jaundice due to tumor factors affecting the functionof the liver (not due to liver metastasis), and when you remove the tumor the liverfunctions will back to normal. The tumor will secrete some mediators that mayaffect the liver and the patient will be presented with elevated liver enzymes and

this is all called "Stauffer syndrome."

Patient may be presented with Hypercalcaemia due to parathyroid hormone-like secretion from the tumor.

Patient may be presented with Anemia due to the supression of the bone marrow by the secretions of the tumor.

Patient may be presented Polycythemia due to the secretion of erythropoietin-like substances causing high levels of hemoglobin.

Patient may be presented with Hypertension, due to renin hypersecretion.

Patient may be presented with Cushingoid features due to secretion of ACTH like substances.

Patient may be presented with Diabetes.

Examination :Previously, anemic features, mass,

tenderness, and jaundice features might be found. However, nowadays usually you will find nothing

Investigations

Lab:Urine analysis & urine culture to rule out hematuria due to infection.CBCKFTLFT

Imaging Studies:

Ultrasound-most important To do staging order CT scan, MRIChest x-ray to detect if there is lung metastasisAngiography might be ordered

A: Ultrasound image of a simple renal cyst showing renal parenchyma (long arrows), cyst wall (arrowheads), and a strong posterior wall (short arrows). B: Ultrasound image of a solid renal mass (arrows

CT scan

Unenhanced CT scan shows solid, right posterior renal mass

Procedures: Biopsy?

- suspecting lymphoma - If the patient is inoperable or refusing the

operation

MRI of a renal cell carcinoma (long arrows) with vena caval tumor thrombus (short arrows

Obstruction of the Inferior Vena Cava

.1Bilateral lower extremity edema.2Nonreducing or right-sided varicocele

StagingTNM classification

T: size or direct extent of the primary Tumor

N: spread to regional lymph NodesM: presence of Metastasis

TTX Primary tumor cannot be assessedT0 No evidence of primary tumorT1 Tumor <7.0 cm and confined to the kidneyT1a Tumor <4.0 cm and confined to the kidneyT1b Tumor >4.0 cm and <7.0 cm and confined to the kidneyT2 Tumor >7.0 cm and confined to the kidneyT2a Tumor >7.0 cm and <10.0 cm and confined to the kidneyT2b Tumor >10.0 cm and confined to the kidney

TT3 Tumor extends into major veins or perinephric tissues but not into the ipsilateral adrenal gland and not beyond the Gerota’s fasciaT3a Tumor grossly extends into the renal vein or its segmental (muscle containing) branches or tumor invades perirenal and/or renal sinus fat but not beyond the Gerota fasciaT3b Tumor grossly extends into the vena cava below the diaphragmT3c Tumor grossly extends into the vena cava above the diaphragm or invades the wall of the vena cavaT4 Tumor invades beyond the Gerota fascia (including contiguous extension into the ipsilateral adrenal gland)

N, MN: Regional Lymph NodesNX: Regional lymph nodes cannot be assessedN0: No regional lymph nodes metastasisN1: Metastasis in regional lymph node(s)N2: extraregional lymph nodes are involvedM: Distant MetastasesMX Distant metastasis cannot be assessedM0 No distant metastasisM1 Distant metastasis present

If you found bilateral RCC , what do you suspect?Familal forms

Treatmentdepends on

Stage of RCC, Distant organ involvement

Type of RCC

Overall health

Surgical options

Radical NephrectomyKidney with perirenal fat ,Gerota's fascia and the supra-renal gland , without lymphadenectomy.Modified Radical NephrectomySuprarenal gland is preserved

Nephron sparing : Tumor Inoculation , partial nephrectomy

Boundaries of radical nephrectomy

Lymphadenectomy :Remains contraversal

Palliative nephrectomy :We remove the kidney to reduce the symptoms (pain, mass, hematuria ...) without the intent to cure the patient.Adjuvant therapy :

*Adenocarcinoma are Chemo-resistant and Radio-resistant

Immunotherapy : better benefits and fewer side effects.Immunotherapy include IL-2 (Interleukins) ,

IFN (Interferons)Tyrosine kinase inhibitors ( Sunitinib, Sorafenib)

Prognosis-: Staging (TNM) is the most consistent factor that

influence the prognosis in patients with renal cell carcinoma.

The 5-year survival rate among T1 patients is around 75 percent , while it drops among

progressing stages to 3 percent among T4.

Follow up-: In the first 6 monthes we do history , PE , lab

investigations (LFT ..) , CXR , US , CT. After the first 6 months we do yearly labs , CXR ,

CT.

Screening

The primary factor that limits the widespread implementation of screening for RCC is the relatively low incidence of RCC in the general population

For now, however, the focus of screening for RCC must be on well-defined target populations, such as patients with end-stage renal disease and acquired renal cystic disease, tuberous sclerosis, and familial RCC

Malignant renal tumors

Primary:Renal cell carcinoma (RCC)Transitional cell carcinoma (TCC)Wilm’s tumorSarcomaLymphomaSecondary (Mets)

Transitional cell carcinoma (TCC)

EPIDEMIOLOGYage range of 75 - 79 yearsMale-to-female ratio 2:1Whites are twice as likely as African-

Americans to develop upper tract tumors.

Etiology and Risk Factors

Smoking: the most important of the modifiable risk factors for upper urinary tract cancer, producing an incidence 3x that seen in nonsmokersOccupation chemical, dyes, petroleum, plastic industries and coal miners.tumors can occur at long intervals (up to 15 years or more) after exposureCoffee ConsumptionAnalgesics abuseArsenic

Pathogensisarises from the urothelium

papillary or sessile lesions, unifocal or multifocal

spread in several different ways, including:.1direct invasion .

.2lymphatic invasion..3vascular invasion.

.4seeding or direct extension.

Clinical presentationThe most common presenting symptom is

hematuria, either gross or microscopic (56% - 98%)

Flank pain ;symptoms of advanced disease, flank or

abdominal mass, weight loss, anorexia, and bone pain

Only 15% of patients are asymptomatic at presentation and are diagnosed when an

incidental lesion is found on radiologic evaluation.

Investigations 1 .Urine Cytology: specific but not sensitive enough .

the sensitivity is directly related to tumor grade.

2 .Radiologic Evaluation:IVU has been the traditional means for diagnosis

In CT : filling defects

obstruction or incomplete filling nonvisualization of the collecting system

Evaluation of the contralateral kidney is important??

possible bilaterality of the disease determination of the functionality of the contralateral kidney

Computed tomography scan showing the presence of a renal pelvic tumor (arrow).

2 .Cystoscopy:To exclude coexistent bladder lesions.

3 .Ureteroscopic Evaluation and Biopsy:If diagnosis remains in question Increases diagnostic accuracyAllows visualization of the tumor and more accurate biopsy of suspected area.

Endoscopic view of a 2-

cm transitional

cell carcinoma in the left renal

pelvis (A) with

intravenous pyelogram (B)

andcomputed

tomography scan

appearance (C).

STAGING (TNM)Primary Tumor (T)TX Primary tumor cannot be assessedT0 No evidence of primary tumorTa Papillary noninvasive carcinomaTis Carcinoma in situT1 Tumor invades subepithelial connective tissue.T2 Tumor invades the muscularis.T3 Tumor invades periureteral fat (for renal pelvis only) or Tumor invades beyond muscularis into perinephric fat or the renal parenchyma.T4 Tumor invades adjacent organ or through the kidney into the perinephric fat.

(Ta -T1 ) are Superficial Tumors .(T2-T4 ) are Invasive Tumors.

Lymph Nodes (N)NX Regional lymph nodes cannot be assessed.N0 No regional lymph node metastasesN1 Metastasis to a single lymph node, 2 cm or less in greatest dimensionN2 Metastasis in a single lymph node, more than 2 cm but not more than 5 cm in greatest dimension; or multiple lymph nodes, none more than 5 cm in greatest dimensionN3 Metastasis in a lymph node, more than 5 cm in greatest dimension

Distant Metastasis (M)MX Distant metastasis cannot be assessedM0 No distant metastasisM1 Distant metastasis

Treatment

based on stage, grade, position, and multiplicity.

Renal function and anatomy should be assessed.

Surgery-: 1 -superficial tumors (Ta , T1 ) usually treated by

Resection especially if the tumor was in the bladder but in the kidney its difficult.

2 -Radical nephroureterectomy:Complete removal of kidney and all parts of

the ureter It’s the standard therapy with excision of a

bladder cuff owing to the possibility of multifocal disease within the ipsilateral

collecting system .*A field of growth tumor*

Advnaced tumorsChemotherapy .oplatinum-based.

Nephroureterectomy .oThere are no benefits of RNU in metastatic (M+)

disease, although it can be considered a palliative option.

Wilm’s Tumor

Wilms’ tumor (Nephroblastoma)

mixed tumour containing blastemal, stromal and epithelial elements arising from embryonic nephrogenic tissue .

The tumours are usually discovered during the first 5 years of life, usually in one pole of one kidney .

Pathology:The cut surface of the tumour is tan coloured .

Clinical features

grows rapidly while the general well-being of the child deteriorates .The mass may be enormous compared with the tiny patient .Abdomenal pain , fever , nausea , vomiting. Some patients are hypertensive.Haematuria denotes extension of the tumour into the renal pelvis and the prognosis is worse.

Imaging by ultrasonography, CT or magnetic resonance imaging (MRI) confirms a solid space-occupying lesion in the kidneyMetastasis to the lungs occurs early.Liver, bone and brain metastases are rare. Lymphatic spread is uncommon.

Treatment: Most unilateral tumors are treated by chemotherapy followed by nephrectomy .

Partial nephrectomy may be possible in patients with bilateral disease.

Prognosis:With modern chemotherapy and surgery, long-term survival can be expected in more than 80% of patients .

The prognosis is worse in those with metastases and in older children

SARCOMA OF THE KIDNEY

Rare5th decade slight male predominance .

Flank or abdominal pain and weight loss are the most frequent presenting symptoms .They are typically of renal capsular origin.They present with symptoms analogous to those of other renal masses and tend to exhibit aggressive local spread with distant metastases to lung and liver as late findings.

Radical nephrectomy for localized disease is the only effective therapy .

Adjuvant radiotherapy has been demonstrated to decrease the incidence of local recurrence in patients with resectable retroperitoneal sarcomas; however, there is no improvement in overall survivalVarious chemotherapeutic agents (e.g. doxorubicin and ifosfamide) have activity in the treatment of metastatic disease, but responses are typically partial and of brief duration.