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Benign renal parenchymal tumors Adenoma Oncocytoma Angiomyolipoma Incidence The most common parenchymal lesion 7-22% at autopsy 3-5% of renal tumors. < 1% of renal tumors. Histology Small, well diff. glandular tumors of renal cortex. Difficult to be diff. from RCC. Composed of oncocytes “Large epithelial cells with fine granular eosinophilic cytoplasm”. 3 major components: 1. Angio: blood vessels. 2. Myo: Smooth ms. 3. Lipoma: Fat cells. Diagnosis Usually incidental. Predominately pathological. Predominately radiological Treatment Nephron sparing surgery (NSS). Nephron sparing surgery (NSS). 2 types: 1. With Tuberous sclerosis (T.S.) Embolization. 2. Without T.S. NSS Specimen (gross picture) Histological picture Prognostic factors of renal cell carcinoma Anatomic a) Tumors size smaller better b) Tumor extension to the surroundings. c) Venous and caval involvement. d) Lymph node affection. e) Distant metastasis. *smaller better, presence of metastasis poor prognosis* Histologic Cell type from good to bad: 1. Chromophobe. 2. Papillary. 3. Conventional. 4. Collecting duct. 5. Unclassified. Fuhrman grade. Sarcomatoid changes. Microvascular invasion. Tumor necrosis. Collecting duct invasion. Clinical Patient’s performance status. Anemia. Cachexia. Thrombocytopenia Degree of localized symptoms Molecular 1. Vascular endothelial growth factors “VEGF”. 2. Hypoxya inducible factors “HIF”. 3. P53. 4. Gene expression.

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Ben ign rena l parenchymal tumors Adenoma Oncocytoma Ang iomyo l ipoma

Inc idence

• The most common parenchymal lesion • 7-22% at autopsy

3-5% of renal tumors. < 1% of renal tumors.

H is to logy

• Small, well diff. glandular tumors of renal cortex.

• Difficult to be diff. from RCC.

• Composed of oncocytes • “Large epithelial cells with fine

granular eosinophilic cytoplasm”.

3 major components: 1. Angio: blood vessels. 2. Myo: Smooth ms. 3. Lipoma: Fat cells.

D iagnos is Usually incidental.

Predominately pathological.

Predominately radiological

Treatment

Nephron sparing surgery (NSS).

Nephron sparing surgery (NSS).

2 types: 1. With Tuberous sclerosis (T.S.) →

Embolization. 2. Without T.S. → NSS

Spec imen (gross p ic ture)

H is to log ica l p ic ture

 Prognost ic fac tors o f rena l ce l l carc inoma

Anatomic

a) Tumors s ize à sma l le r bet ter b) Tumor ex tens ion to the surround ings . c) Venous and cava l invo lvement .

d) Lymph node a f fec t ion . e) Dis tant metastas is .

*smal le r bet ter , presence o f metastas is poor prognos is*

H is to log ic

Ce l l type f rom good to bad: 1. Chromophobe. 2. Pap i l la ry . 3. Convent iona l . 4. Co l lec t ing duct . 5. Unc lass i f ied .

q Fuhrman grade. q Sarcomato id changes. q Microvascu lar invas ion . q Tumor necros is . q Co l lec t ing duct invas ion .

C l in ica l • Patient’s performance status. • Anemia. • Cachexia.

• Thrombocytopenia • Degree of localized symptoms

Mo lecu lar

1. Vascular endothelial growth factors “VEGF”. 2. Hypoxya inducible factors “HIF”.

3. P53. 4. Gene expression.

                     

  Rena l ce l l carc inoma Wi lms’ tumor Tumor of renal pelvis

Inc idence

General à In USA o 30k new cases/year o 11k deaths/ year o RCC à 85% of primary malignant renal tumors in adults

Age à 5th to 5th decade of life Sex à M:F = 2:1 Race à Hispanic > Americans

General: § The most common solid renal tumor of

childhood. § In USA: 460 new cases / year.

Age: The peak age is the 3rd year of life Sex: Male: female = 1:1 Bilaterality: In 5% of cases Wilms’ tumor is bilateral.

Genera l à 7% of a l l k idney tumors . Age à 7 th decade o f l i fe . Sexà Ma le : female = 2:1 .

E t io logy or pre

d ispos ing fac tors

1) Hered i tary à Von H ippe l L indau Syndrome o Cerebellar hemangioblastoma o Retinal angioma o Bilateral RCC

2) Acqu ired rena l cyst ic d isease o f the k idney

o Patients with ESRD + dialysis. 3) C igaret te smok ingà at least 2-fold increase in risk. 4) Ana lges ic abuse

o Phenacitin-containing products → analgesic nephropathy → RCC.

5) Occupat iona l q Shoe workers. q Leather tanners. q Workers exposed to Cadmium., Petrolium, Asbestos

1. Fami l ia l . § 15-20% of all tumor. § Typically younger and with bilateral

tumors. § Short arm of chromosome 11. § Autosomal dominant (AD).

2. Sporad ic .

1. Occupat iona l : a. Chemical b. Printing c. Rubber d. Plastic e. Leather f. Gasworks g. Sewage works

2. Balkan nephropathy . 3. Ana lgest ic nephropathy . 4. C igaret te smok ing. 5. Chron ic in f lammat ion .

Patho logy

1. Convent iona l type(70%) • C lear ce l l + granu lar ce l l .

2. Papi l la ry type (10-15%) 3. Chromophobe type (5%.) 4. Co l lec t ing duct (1%) 5. Unc lass i f ied (5%)

3 components in vary ing proport ions : 1. Blastemal . 2. Epi the l ia l . 3. Stromal

Spread

1. Direct invas ion à Through renal capsule to the surrounding structures.

2. Direct ex tens ion to rena l ve in & IVC à 10% → Tumor thrombus.

3. Lymphat ic spread. 4. Hematogenous (D is tant metast . )

• 1/3 of patients have metastasis at time of presentations. • The most common site is the lung.

1. Direct ex tens ion through rena l capsu le .

2. Direct ex tens ion v ia rena l ve in & IVC . 3. Lymphat ic . 4. Haematogenous

• Metastatic disease is present at diagnosis in 10-15% of patients

• The most common site is the lung

1. Benign à Pap i l loma. 2. Mal ignant

§ TCC (The most common). § SCC. § Adenocarcinoma.

S tag ing

Tx: not assessed. To: no Tumor. T1: Tumor 7 cm or less limited to the kidney:

• T1a : ≤ 4 cm. • T1b: > 4cm ≤ 7cm

T2: Tumor > 7cm limited to kidney. T3: Tumor outside the kidney but still within Gerota’s fascia :

• T3a : Adrenal or perinephric fat. • T3b : Renal vein or IVC below the diaphragm. • T3c : IVC above the diaphragm.

T4: Tumor invades beyond Gerota’s fascia. Nx : Not assessed. N0: No LN metastasis. N1: metastasis in a single LN. N2: metastasis in more than one LN Mx : Not assessed. M0: No distant metastasis. M1: Distant metastasis.

NWTS Nat iona l W i lms’ Tumor Stag ing System

Stage I à Tumor limited to kidney and completely excised. Stage II à Tumor extends beyond the kidney but is completely removed. Stage III à Residual non hematogenous tumor confined to abdomen. Stage IV à Haematogenous metastasis. Stage V à Bilateral renal involvement at diagnosis.

Tx: not assessed T0: no tumor Ta: mucosa T1: submucosa T2: muscles T3: surrounding fat T4: surrounding organs Nx: not assessed N0: no L.N. metastasis N+: L.N. metastasis Mx: not assessed M0: no distant metastasis M+: Distant metastasis

C l in ica l p ic ture

Symptoms 1. Triad of Sx à hematuria, mass, pain. Can also presented by

only one symptom 2. Paraneoplastic syndrome àsystemic manifestation 3. CP of metastasis àbone ache, enlarged LN, hemotypsis

Signs

1. Mass enlarged and can be felt 2. Virchow’s LN if metastasis 3. Irreducible varicocele on left = pathognomonic

Most commonly à mass observed by mother on the f lank

Symptom • Hematuria is common • CP of metastasis

Treatment

• T1,T2 à NSS or partial nephrectomy

• Beyond kidney à radical nephrectomy

• Metastasis à nephrectomy + targeted therapy

• If presence of comorbidity à radiofrequency ablation, cryosurgery

Chemosens i tve , rad iosens i t i ve Nephroureterectomy + excision of

bladder cuff