Lots of mitochondria abundant and distorted tan color granular eosinophilic cytoplasm Eosinophilic cells

Well encapsulated STELLATE SCAR May reach 12 cm in size NOTE: 5% of ressected tumors

Benign Angio→blood vessels Myo→smooth muscle Lipoma→adipose tissue 25-50% of pt with tuberous sclerosis (CM3) Radiological diagnosis

A. Familial Clear Cell Carcinoma 50% risk B. Von Hippel Lindau VHL disease AD cancer syndrome Cerebellar haemangioblastomas Retinal angiomas Phaeochromocytoma Cysts in various organs C. Hereditary Papillary RCC Multiple bilateral papillary tumors

\<10% Hematuria (occurs in 50% ie most common symptom) Flank pain Mass

Kidney Tumors Flashcards by Ahmed Alyaseen

Classification of Primary Tumors

A. Benign

Papillary Adenoma (<5mm)
Oncocytoma
Angiomyolipoma

B. Malignant

Renal cell carcinoma
Wilms tumor (children)
Urothelial carcinoma (pelvis)

C. Others

Lymphoma
METASTATIC -RARE
- Blood borne from lungs breast and stomach

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Renal Papillary Adenoma

Origin

Renal tubules; within cortex
7-22% found at autopsy

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Renal Papillary Adenoma

Gross appearance

Small encapsulated tumor
Within cortex as pale yellow discrete nodules
<2cm→benign
>2cm→malignant

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Renal Papillary Adenoma

Histopathology

Branching papillomatous glands
Resembles low grade papillary renal carcinoma therefore all papillary tumors are seen as malignant until proven otherwise
Share immunohistochemical and cytogenic features with papillary carcinomas →trisomies 7 and 17

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Oncocytoma

Type and origin

Type: Benign epithelial tumor

Origin: From intercalated cells of the collecting ducts

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Oncocytoma

Histo

Lots of mitochondria
- abundant and distorted
- tan color
- granular eosinophilic cytoplasm
Eosinophilic cells

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Oncocytoma

Gross

Well encapsulated
STELLATE SCAR
May reach 12 cm in size

NOTE: 5% of ressected tumors

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Angiomyolipoma

Type

Benign
- Angio→blood vessels
- Myo→smooth muscle
- Lipoma→adipose tissue
25-50% of pt with tuberous sclerosis (CM3)
Radiological diagnosis

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Malignant tumors of kidney

Classification

A. Cortex

Epithelial
- Renal cell carcinoma
Mesenchymal
- Sarcoma
- Lymphoma
- others
Blastema
- Wilms tumor/nephroblastoma (Peds)

B. Renal pelvis

Urothelial carcinoma
Squamous cell carcinoma

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Renal Cell Carcinoma

Hypernephroma, clear cell carcinoma, adenocarcinoma

Epidemiology

1-3% of visceral cancers
85% of adult renal cancers
60-70
Male to female 3:1
Mostly sporadic
AD in younger pts
Familial variant in 4%
Mostly sporadic with 5% inherited

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Renal Cell Carcinoma

Origin

Tubular epithelium

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Renal Cell Carcinoma

Gross morphology

Yellow color

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Renal Cell Carcinoma

Risk Factors

30X increase in pt with aquired cystic disease in CRF
Familial syndromes
Tobacco
Obesity
HTN
Unopposed estrogen therapy
Exposure to asbestos
Petroleum
Heavy metals

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Heridetary RCC

Types

A. Familial Clear Cell Carcinoma

50% risk

B. Von Hippel Lindau VHL disease

AD cancer syndrome
Cerebellar haemangioblastomas
Retinal angiomas
Phaeochromocytoma
Cysts in various organs

C. Hereditary Papillary RCC

Multiple bilateral papillary tumors

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RCC

Classical Triad

<10%

Hematuria (occurs in 50% ie most common symptom)
Flank pain
Mass

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RCC

Metastasis

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Hematogenous

Local ie abdominal

RCC

Paraneoplastic Syndromes

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PTH, PG→Hypercalcemia
Renin→HTN
GC→CUSHING
Gonadotropins→Feminisation or Masculinisation
EPO→Polycythemia

NOTE: 5 year survival→40%

RCC

Histological classification

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Clear cell type
- 77%
- Clear cytoplasm with lipid and glycogen
Papillary RCC
- 15%
- Papillary structures
Chromophobe cell type
- 5%
- Solid growth
- Perinuclear halo
- Granular cytoplasm

Clear Cell Carcinoma

Epidemiology

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Most common type (70-80% or renal cell cancers)
Mostly sporadic→few familial
Loss of chromosomal segment 3p14 to 3p26
Associated with Hippel-Lindau VHL disease

Papillary Carcinoma

characteristics

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10-15% of RCC
Multifocal, bilateral
Sporadic → trisomies of ch7,16,17
Familial→Trisomy of ch7

Papillary Renal Cell Carcinoma

Types

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Morphological overlap but distinct molecular pathway and clinical picture

Type 1
- Papillae lined by single layer of cells
- Scanty basophilic cytoplams
- Low nuclear grade
- Trisomies 7,17
- MET mutation
- better prognosis
Type 2
- Lined by pseudostratified layers
- Abundant eosinophilic cytoplasm
- Higher nuclear grade
- Multiple genetic changes
- WORSE prognosis

Chromophobe RCC

characteristics

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5%
Arise form Cortical collecting duct or intercalated cells
Loss of entire chromosomes: 1,2,6,10,13,17,21
HYPOPLOIDY
Good prognosis

Renal Cell Carcinoma

Staging

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Stage 1: <7cm; 5 yr survival more than 95%

Stage 2: > 7cm; 88%?

Stage 3: Gerota’s fasia or 1 regional lymphnode involved; 5 yr survival 59%

Stage 4: Beyond gerota’s fascia or > 1 lymph node involved; 5 yr survival 20%

Urothelial Carcinoma of Renal Pelvis

Etiology

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5-10% of primary renal cancers

Analgesic abuse
dye
rubber

Urothelial Carcinoma Clinical presentation

* Hematuria within a short time * Small when discovered→not plapable * May block urine flow * May affect pelvis, urter and bladder * **Poor prognosis**

Urothelial Carcinoma of renal pelvis Grossly

May infiltrate wall of pelvis Papillary urothellial carcinoma enter to urterer

Wilms Tumor/Nephroblastoma Epidemiology

* Childhood malignant neoplasm * Commonest solid abdominal tumor in children 2 to 5 yrs * Prevalence 1 in 10 thouand * Sporadic and unilateral in 90% * Familial and bilateral in 20%

Wilms Tumor Triphasic Pattern

Embryonic microscopic nephrogenic elements that are a mixture of: * Blastema * Epithelial structures- immature glomeruli or abortive tubules * Stroma Nephrogenic rests

Wilms Tumor Clinical Presentation

* Palpable abdominal mass * Fever * Abdominal pain * Hematuria

Wilms tumor Morphology

* Large * Solitary * Lobulated * Well circumscribed * 10% bilateral or multicentric

Syndromes associated with increased risk and often bilateral Wilms tumor

1. WAGR SYNDROME * **W**ilms (30% develop it) Wilms Tumor-1 associated gene on ch**11p13** * **A**niridia * **G**enital anomalies * **R**etardation (mental)

Syndromes associated with increased risk and often bilateral Wilms tumor

2. Denys Drash Syndrome * Gonadal Dysgenesis (male pseudohemaphrodite) * Nehropathy (RF) * WT-1 on **Ch 11p13**

Syndromes associated with increased risk and often bilateral Wilms tumor

3. Beckwith Weidemann Syndrome * Enlarged body organs * Hemihypertrophy * Renal Medullary Cysts * Adrenal cytomegaly **WT-2 gene on Ch 11p15.5**

Wilms Tumor Course

* Initially spreads locally thru renal capsule * Propensity to extend into **renal vein and IVC** * Metastasizes prefretially to **regional LN and Lung** * **Unusual to involve bone marrow**

Metastatic tumors to the kidney

* Uncommon * From * Lung * Breats * Stomach

Diagnostic tests

* CBC * Renal FT * Tumor markers * EPO * PTH * Urine * Rouine microscopic * Cytologic exam * Radio * US * CT * others * Uretroscopy, cytoscopy * Biopsy * FNA * Core needle biopsy

Staging, screening tests

**STAGING** * LFT * CXR * BONE SCAN * imaging **Screening** * **US** every 6-8 months in children at high risk

Kidney Tumors Flashcards

(37 cards)