A Case of Mesothelioma
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A Case of Mesothelioma

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A Case of Mesothelioma A Case of Mesothelioma Presentation Transcript

  • Dr. Prasanth Sankar Prof. Dr. E. Dhandapani’s Unit
    • Ramu
    • 38/m
    • Thiruvannamalai
      • C/o
        • Left sided chest pain
        • 6 mo
        • Breathlessness
    • H/o breathlessness – 6 mo
      • Progressively increasing
      • Initially on exertion, later on at rest
      • More on lying on right side
      • No PND, orthopnea
      • Severe for the last 6 days
    View slide
      • Left sided Chest Pain
      • Pleuritic type initially
      • Later on persistant, aching and pricking type
      • More on the lateral aspect of L chest
      • Not assoc with palpitation, sweating
      •  with coughing and lying on left side
    • Cough
      • Mostly non productive
      • Occasional expectoration of mucoid sputum
      • Non purulent; No hemoptysis
    View slide
    • Gradual restriction of movements of L chest
    • Painful Swelling -Lateral side of L chest-3 wks
    • H/o loss of weight -6-8 kg/6mo
    • H/o Loss of Appetite +
    • No h/o
      • Cyanosis
      • Leg swelling, Abdominal distension
      • Fever
      • Oliguria
      • Giddiness, LOC
      • Chest Trauma
    • Evaluated for symptoms at local hospital
    • Based on X ray findings – empirical ATT
    • Symptoms continued to progress
    • Discontinued ATT after 3 mo
    • Attended CT OP at GSH- adv CT chest
    • Severe dyspnea at rest -6 days
    • Referred from local hospital to GSH
    • No history S/o
      • Bronchial Asthma, COPD, TB,
      • Radiation Exposure
      • Heart disease/ HTN/DM/CKD
      • Trauma/interventions
      • Connective tissue disorders
      • Smoker – beedis – 25 pack yrs
      • Consumes alcohol once/twice per week
      • No high risk behaviour
      • Manual labourer – rice godown
    Personal History
    • G/E-
      • Conscious , Oriented
      • Moderate built & poorly nourished
      • Severely Dyspneic, Tachypneic, restless
      • Difficult to speak in sentences
      • No pallor, icterus, cyanosis, clubbing, edema
      • No significant lymph nodes
      • BP – 110/70
      • Pulse – 108/min, regular
      • RR – 34/min SpO2 – 88% (room air)
      • JVP – not raised
    • RS
      • Trachea – marked left shift
      • Marked scoliosis of thoracic spine- concavity to Left
      • Severe crowding of ribs left side, Dilated veins +
      • Chest movements markedly restricted on L side
      • 5X3X2cm tender firm swelling over Left 7,8,9 interspaces
        • Immobile
        • Tendernss of surrounding areas of chest wall
        • No sinuses,
        • non pulsatile
      • Dull/ stony dull percussion note - Left
      • Hyper-resonance - Right
      • VF/VR  - Left
      • BS markedly dimnished L hemithorax
      • No adventituous sounds
    • CVS
      • Apex not localised
      • S1, S2 +
    • Abdomen, CNS - WNL
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    • PT sequelae
    • Left pleural fibrosis
    • Malignancy
    • Shifted to IMCW
      • O2 inhalation/Propped up positioning
      • Bronchodilators
      • Antibiotics
      • Analgesics
      • IV Fluids
      • Supportives
    • With Treatment
      • Mild improvement of symptoms
      • SpO2 – 95-96%
    • Hb -10.5
    • PCV - 35
    • TC -9800
    • DC – P44/L53/E3
    • PLT – 2.1L
    • ESR-10/22
    • RBS – 121 Urea -24 Creat – 0.8
    • Na – 135 K – 3.4
    • LFT – WNL
    • BT/CT/INR – WNL
    • RUE – WNL
    • HIV/VDRL - negative
    • ECG – Sinus tachycardia, P pulmonale
    • Sputum
      • GS & AFB – negative
      • Culture – no growth
      • Cytology – no malignant cells.
    • CXR-
    • CT chest-
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    • CTS opinion
    • imp: Pleural Mesothelioma
    • Adv: CT guided biopsy
    • Medical oncology:
    • imp: Mesothelioma Stage IV
    • Adv: CT guided/open Biopsy
    • Review with results
    • CT guided biopsy was fixed in GH
    • Patients dyspnea and chest pain persisted
    • SpO2 – 92-94% with O2
    • On 4 th day of admission..patient went into sudden cardiac arrest
    • Resuscitated and recovered.
    • Intubation-Mechanical Ventilation given
    • Again went into cardiac arrest & expired..
    • Malignant Mesothelioma - Stage IV
    • Uncommon but no longer be considered rare.
    • 3000 cases/year diagnosed in the US alone.
    • In countries where control of asbestos was delayed by several decades, the “epidemic” of mesothelioma will also be delayed by several decades
    • Asbestos continues to be mined, and its use is actually increasing in many developing countries
    • Asbestos industry
    • Insulators
    • Pipefitters
    • Shipyard workers
    • Brake mechanics
    • Railroad workers
    • Construction trades
    • Carpenters
    • Plumbers
    • Electricians
    • Painters
    • Non-asbestos miners
    • Welders
    • Machinists
    • Manufacturers of mineral products
    • Maintenance and repair in buildings with asbestos insulation.
    • Women with asbestos-induced mesothelioma -only clear asbestos exposure was from exposure to their spouses' contaminated clothing.
    • Children incidentally exposed -develop mesothelioma in early adult life.
    • Incidence may rise - dust that settled after the collapse of the World Trade Center
    • Of nanoparticles and nanotubes raises concerns
    • Simian virus (SV40) – animal studies
    • Oil refinery workers-petroleum oil and its products ?
    • Cigarette smoking and Silica- - not associated with increased incidence of mesotheliomas
    • Radioactive contrast medium thorotrast
    • Therapeutic radiation for of lymphoma or breast Ca.
    • In turkey, -exposure to erionite dust, a non-asbestos crystalline fibrous form of the mineral zeolite.
    • Chronic inflammation of the pleura as in familial mediterranean fever
    • Although millions exposed, only a few develop mesothelioma
    • High degree of aneuploidy, but no single oncogene or tumor suppressor gene found culprit
    • p16 INK4A -p14 ARF (9p21), (NF2) gene (22q12)
    • P53 & Kras – not mutated
    • 1p, 3p, 6q, 9p, 15q, and 22q
    • Growth-promoting genes – PDGF, EGFR
    • long latency period-multiple genetic abnormalities
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    • Constitutional symptoms
      • Weight loss (30%), cough (10%) and fatigue are not common in the early presentation.
      • HPOA and intermittent hypoglycemia are unusual
    • Serous effusions
      • Most common presentation - 95%
      • Dyspnoea (40–70%)
      • Non-pleuritic chest pain (60%)
    • Local invasion
      • Direct invasion of adjacent structures is characteristic of malignant mesothelioma.
        • SVC obstruction, Spinal cord compression,
        • Horner’s syndrome, oesophageal compression,
        • Chest wall masses, Malignant pericardial disease.
      • Metastasis along tracks of previous invasive procedures (2 – 51 %)
      • Chest wall, rib or intercostal nerve- pain.
      • Pericardial invasion- pericardial effusion, cardiac tamponade and/or arrhythmias,
      • Invasion into c/l hemithorax or peritoneal cavity
    • Distant spread
      • Extrathoracic spread- 54–82% PM
      • Often clinically silent and rarely cause of death
      • Hilar or mediastinal lymph node metastasis -44%
      • Intracranial metastases-3%
      • ‘ Miliary mesothelioma’- rare.
    • Pleural effusion or pleural mass
    • Large effusions or tumour masses-m ediastinal displacement.
    • Tumour may erode through chest wall and cause localised tenderness and/or palpable masses
    • Spread within the pleural cavity - ‘ fixed’ hemithorax , with reduced chest expansion.
    • Signs of compression or invasion of mediastinal structures.
    • Signs of extrathoracic involvement are uncommon
    • Clubbing of fingers -not a feature
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      • Unilateral pleural effusion with or without evidence of pleural thickening
      • Occasionally-large visible mass at presentation
      • Pleural thickening and encasement of the underlying lung
        • may neutralise the mediastinal shift
        • contracture of the affected hemithorax.
      • Pleural plaques and asbestosis - (20%)
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    • Rind-like tumor extension on pleural surfaces - 70%
    • Circumferential encasement by multiple nodules -28%
    • Pleural thickening with an irregular margin between the lung and the pleura - 26%
    • Pleural thickening;pleural-based nodules - 20%
    • Invasion of soft tissues & chest wall with rib erosion
    • Lung encased with tumor, volume loss with a shift of mediastinum toward side of primary tumor
    • Signs of lymphatic metastasis – late
    • Mediastinal adenopathy – very rare
    • CT features that favor diagnosis of malignant mesothelioma over metastatic pleural disease
      • Rind like pleural involvement,
      • Mediastinal pleural involvement, and
      • Pleural thickness more than 1 cm
    • MRI may demonstrate extent of disease and in particular chest wall and diaphragmatic invasion better than CT
    • FDG-PET and particularly PET/CT -differentiate benign from malignant disease and as adjunctive tools for staging
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    • No specific haematological or biochemical test
    • Anemia, thrombocytosis, high ESR, hypergammaglobulinaemia.
    • Only serum biomarker clinically useful is serum mesothelin
      • High specificity (>90%).
      • But only a 50% sensitivity for the diagnosis.
    • Thoracocentesis - difficult to distinguish between reactive mesothelial cells and malignant ones.
    • Aspirate cytology of effusions - 33% to 54%
    • Pleural fluid Mesothelin – useful biomarker.
    • Closed pleural biopsy – sensitivity of 55%
    • CT-guided biopsy – sensitivity of 88%
    • Accuracy - morphologic appearance and results of tumour marker staining using light microscopy
    • Preferred technique -surgical biopsy via pleuroscopy
      • Large samples
      • Drainage of effusions
      • Freeing up of a trapped lung
      • Talc pleurodesis if lung not trapped
    • Bronchoscopy, BAL and Ga scan-no useful role
    • Median survival- 4 -12 mo from the time of diagnosis
    • Epithelial cell type do best and those with the sarcomatous cell type the worst
    • Poor Prognosis
      • Age,
      • male gender,
      • performance status,
      • leukocytosis, and
      • chest pain
      • microvessel density
      • tumor necrosis
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    • Surgical Therapy - Debulking
      • Pleurectomy with Decortication (P/D)
      • Extrapleural Pneumonectomy (EPP)
      • Epithelial cell type, clean margins after resection, and negative lymph nodes
      • Tumor debulking using EPP followed by chemotherapy and high-dose radiation therapy
    • Chemotherapy
      • Pemetrexed – Cisplatinum
      • Gemcitabine with a platinum agent
    • Radiation Therapy
      • Limited to adjunctive therapy
    • Drugs
      • Gefitinib and Imatinib
      • Thalidomide
      • Superoylanilide hydroxamic acid (SAHA)- histone deacetylase inhibitor
      • Proteasome inhibitors
      • Bevacizumab
    • Immunotherapy
      • Intrapleural interferon- γ
      • Infusion of interleukin-2
    • Gene Therapy
    • Palliative Therapy
      • Pain management
      • Pleurodesis
      • Placement of a tunneled pleural catheter
    • Chemoprevention and Screening
      • Screening of high-risk populations
      • Sensitivity of serum mesothelin is not sufficient for use as a marker
      • Daily vitamin A (retinol) or β-carotene - trial
      • Routine low-dose CT scanning - trials
    • 2010 – Murray and Nadel's Textbook of Respiratory Medicine, 5th ed
    • 2010 – Pleural Disease- Second Edition
    • 2008 – Fishman’s Pulmonary Diseases and Disorders-Fourth Edition
    • 2002 – Mesothelioma
    • 2002 – Crofton And Douglas’s Respiratory Diseases – Fifth Edition