RENAL TUMORS

Classification

Benign

• Cortical adenoma
• Angiomyolipoma

Malignant

• RCC
• Upper tract TCC
• Nephroblastoma
• Squamous CA of renal pelvis

Cortical adenoma

• Discovered incidentally, mostly found on autopsy
• Less than 1 cm without malignant features (pea like)
• Symptoms are unusual

Microscopically uniform basophilic or acidophilic with monotonous nuclear and cellular composition

Renal angiomyolipoma

• May occur alone or a part of tuberous sclerosis
• Hamartomas composed of fat, smooth muscle, and blood vessels
• AML may be found in the eyes, heart, lung and bone

• Malignant Tumors

Wilms tumor (nephroblastoma)

 Peak age:2-5 years

Risk factors

. WAGR syndrome-Wilms tumor, aniridia, genital anomalies, and mental retardation

. Beckwith-Wiedemann syndrome

.Tumor suppressor genes

 . WT-l (llp13)

 . WT-2 (llp15)

Gross: large solitary tan mass

Micro

1. Metanephric blastema

ii. Epithelial elements (immature glomeruli and tubules)

iii. Stroma

C/F:

Abdominal mass

Pyrexia

Hematuria

Metastasis occurs early, mainly to lungs.

Inv:

USG

Urography or

CT

Treatment: 

Nephrectomy followed by radiation with or without chemotherapy

If B/L tumor: Partial Nephrectomy

Prognosis: excellent; long-term survival rate of 90%

Renal Cell carcinoma

Epidemiology

- Male: Female  2:1

- 40-60 years of age

Risk factors

i. Cigarette smoking

ii. Chronic analgesic use

iii. Asbestos exposure

iv. Chronic renal failure and acquired cystic disease

v. Von Hippel- Lindau disease

Gross

. Large solitary yellow mass found in the poles (most commonly in the upper pole: most common site- PCT)

. Areas of necrosis and hemorrhage are commonly present

. The tumor often invades the renal vein and may extend into the vena cava and heart

Pathology

Clear cell types

• more than 80%
• polygonal or round cells with clear cytoplasm

Papillary variant

• 14% of RCC
• macroscopic feature-small, confined to cortex and nearly completely encapsulated
• Micro: papillae covered by small cells with scanty cytoplasm, arranged in a single layer on the papillary basement membrane with low nuclear grade

Sarcomatoid

• aggressive behavior and poor prognosis
• microscopic features- spindle cell pattern

Chromophobe variant

• 4% of RCC
• microscopic features light and transparent cytoplasm
• better prognosis

Signs and symptoms

Classic triad (pain, lump and hematuria) is found only in 10% cases

Weight loss, fever, hematuria, night sweats and sudden development of varicocele

Paraneoplastic syndromes from ectopic hormone production

   . Polycythemia(erythropoietin production)

   . Hypertension (renin production)

   . Cushing syndrome (corticosteroid synthesis)

   . Hypercalcemia (PTH-like hormone)

   . Feminization or masculinization (gonadotropin release)

High incidence of metastasis on initial presentation

May cause amyloidosis, a leukemoid reaction, or eosinophilia

Investigations

IVU

CT scan

• Renal Angiogram
• CX-Ray
• Bone scan

Treatment

• RCC is highly resistant to radio and chemotherapy
• Nephrectomy with removal of the perinephric fat

LUNG TUMORS

Bronchogenic carcinoma

•  Epidemiology
•  Leading cause of cancer death among both men and women
•  Increasing in women (increased smoking) in the past few decades
•  Occurs most commonly from 50-80 years of age

Major risk factors

i. Cigarette smoking

ii. Occupational exposure (asbestosis, uranium mining, radiation, etc.)

iii. Air pollution

Common genetic mutations

i. Oncogenes 

 L-myc: small cell carcinomas

 K-ras: adenocarcinomas

ii. Tumor suppressor genes p53 and the retinoblastoma gene

Clinical features

•  Cough
•  sputum production
•  weight loss
•  anorexia
•  fatigue
•  dyspnea
•  hemoptysis and
•  chest pain
•  hoarseness of voice
•  Obstruction may produce focal emphysema, atelectasis, bronchiectasis or pneumonia

Adenocarcinoma (35%)

.Males=females

.less closely associated with smoking than squamous cell

.Gross: peripheral gray-white mass with pleural puckering

.May develop in areas of parenchymal scarring (scar carcinoma)

.Micro: tumor forms glands and may produce mucin.

Bronchioloalveolar carcinoma (5%)

. Subset of adenocarcinoma

. Arises from terminal bronchioles or alveolar walls

. Gross: peripheral mucinous gray-white nodules

Micro:

     columnar tumor cells grow along the walls of pre-existing alveoli

Squamous cell carcinoma (30%)

. Males> females

. strongly related to smoking

Gross: usually centrally located, gray-white bronchial mass

            Arises from bronchial epithelium after a progression:

metaplasia ~dysplasia ~ carcinoma in situ ~ invasive carcinoma

Micro:

. Invasive nests of squamous cells

. Intercellular bridges (desmosomes)

. Keratin production ("squamous pearls")

Small cell (oat cell) carcinoma (20%)

. Males> females

. strong association with smoking

 Very aggressive: rapid growth and disseminate  early

Gross: central, gray-white masses

Micro:

small round or polygonal cells in clusters

EM: cytoplasmic dense-core neurosecretory 

granules

Commonly associated with paraneoplastic syndromes.

Also most common cause of venacaval obstruction syndromes.

Large cell carcinoma (10%)

In early stages, is associated with cavitation

Gross: peripherally located lesion

Micro: 

          large anaplastic cells without evidence of differentiation

Intrathoracic spread

i. Lymph nodes (50%):

         hilar, bronchial, tracheal, and mediastinal

ii. Pleural involvement (adenocarcinoma)

iii. Pancoast tumor (apical tumor) causing Horner syndrome

iv. Superior vena cava syndrome

     Obstruction of SVC by tumor 

     .Distended head and neck veins

     .Plethora

     .Facial and upper arm edema

v. Esophageal obstruction: dysphagia

vi. Recurrent laryngeal nerve involvement: hoarseness

vii. Phrenic nerve damage: diaphragmatic paralysis

Extrathoracic sites of metastasis:

   adrenal (>50%)

    liver

    brain and

    bone

Paraneoplastic syndromes

i. Endocrine/metabolic syndromes

    . ACTH ~ Cushing syndrome 

    . ADH~ SIADH

    . PTH ~ hypercalcemia (squamous cell carcinomas)

ii. Eaton-Lambert syndrome

iii. Acanthosis  nigricans

iv. Hypertrophic pulmonary osteoarthropathy 

. Periosteal new bone formation

. Clubbing 

. Arthritis

Investigations

Sputum cytology

Bronchoscopy:

                         Best for centrally located lesions

Fine Needle Aspiration Bx

                         for peripheral lesions

Pleural Bx in all patients presenting with pleural effusion.

CX-Ray: common features-

     a. U/L hilar lymphadenopathy

c. Lung, lobe or segmental collapse

d. Pleural effusion

b. Peripheral pulmonary opacity

c. Lung, lobe or segmental collapse

d. Pleural effusion

e. Broadening of mediastinum, enlarged cardiac shadow, elevation of hemidiaphragm.

f. Rib destruction


CT Chest

Others:
CT head
Liver ultrasound
Bone marrow biopsy

Treatment

1.Surgical resection
   Symptoms that suggest unresectable lesion: Wt. loss >10%
                                                                               

• Bone pain

other extrathoracic metastases
CNS symptoms
Tumor involving trachea, esophagus, pericardium and chest wall.

Small cell CA

2. Radiotherapy:
    palliation of distressing complications like SVC obstruction, rec. hemoptysis, pain caused by chest wall invasion.
Used in adjunct to Chemothrapy for small cell ca.
 

3. Chemotherapy

   
esp. used in small cell CA along with RT.
 
I.v Cyclophophamide, doxorubicin and vincristine
                              OR
 
I.v  Etoposide and Cisplatin are used.

4. Neoadjuvant and adjuvant CT
   CT given  surgery for down staging the disease in non small cell CA.
 Post-op CT used if lymph node is involved

5. Effusion sclerosed with tetracycline.

Prognosis:
Poorest for small cell carcinoma
Best after surgical resection of squamous cell CA.

Secondary lung tumors

Blood borne metastatic deposits from many primary tumors: part. Breast
                                                                                                         kidney
                                                                                                         uterus
                                                                                                         ovary
                                                                                                         testes
                                                                                                         thyroid
Deposits are usually multiple and bilateral.
Often there are no respiratory symptoms and diagnosis is made by radiological examination.

Malignant mesotheliomas

. Rare highly malignant neoplasm affecting the pleura.
. Occupation exposure to asbestos in 90% of cases
. Presents with recurrent pleural effusions, dyspnea, chest pain

. Gross: encases and compresses the lung

. Micro: carcinomatous and sarcomatous elements (biphasic pattern)

. EM: long, thin microvilli
.No curative treatment and chain pain is often difficult to control.
. Poor prognosis.