A Case of Mesothelioma
-
Upload
stanley-medical-college-department-of-medicine -
Category
Health & Medicine
-
view
1.412 -
download
2
description
Transcript of A Case of Mesothelioma
Dr. Prasanth Sankar
Prof. Dr. E. Dhandapani’s Unit
Ramu38/m Thiruvannamalai
C/oLeft sided chest pain
6 moBreathlessness
H/o breathlessness – 6 mo
Progressively increasingInitially on exertion, later on at
restMore on lying on right sideNo PND, orthopneaSevere for the last 6 days
• Left sided Chest PainPleuritic type initiallyLater on persistant, aching and pricking
typeMore on the lateral aspect of L chestNot assoc with palpitation, sweatingwith coughing and lying on left sideCough Mostly non productiveOccasional expectoration of mucoid
sputumNon purulent; No hemoptysis
Gradual restriction of movements of L chestPainful Swelling -Lateral side of L chest-3
wksH/o loss of weight -6-8 kg/6moH/o Loss of Appetite +No h/o
CyanosisLeg swelling, Abdominal distensionFeverOliguriaGiddiness, LOCChest Trauma
Evaluated for symptoms at local
hospitalBased on X ray findings – empirical
ATTSymptoms continued to progressDiscontinued ATT after 3 moAttended CT OP at GSH- adv CT chestSevere dyspnea at rest -6 daysReferred from local hospital to GSH
No history S/oBronchial Asthma,
COPD, TB, Radiation ExposureHeart disease/
HTN/DM/CKDTrauma/
interventionsConnective 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 , OrientedModerate built & poorly nourishedSeverely Dyspneic, Tachypneic, restlessDifficult to speak in sentencesNo pallor, icterus, cyanosis, clubbing,
edemaNo significant lymph nodesBP – 110/70Pulse – 108/min, regularRR – 34/min SpO2 – 88% (room air)JVP – not raised
RSTrachea – marked left shiftMarked scoliosis of thoracic spine- concavity
to LeftSevere crowding of ribs left side, Dilated
veins +Chest movements markedly restricted on L
side5X3X2cm tender firm swelling over Left 7,8,9
interspacesImmobileTendernss of surrounding areas of chest wallNo sinuses,non pulsatile
Dull/ stony dull percussion note - LeftHyper-resonance - Right VF/VR - Left BS markedly dimnished L hemithoraxNo adventituous sounds
CVSApex not localisedS1, S2 +
Abdomen, CNS - WNL
PT sequelaeLeft pleural fibrosisMalignancy
Shifted to IMCW
O2 inhalation/Propped up positioningBronchodilatorsAntibioticsAnalgesicsIV FluidsSupportives
With TreatmentMild improvement of symptomsSpO2 – 95-96%
Hb -10.5PCV - 35TC -9800DC – P44/L53/E3PLT – 2.1LESR-10/22RBS – 121 Urea -24 Creat –
0.8Na – 135 K – 3.4
LFT – WNLBT/CT/INR – WNLRUE – WNLHIV/VDRL - negativeECG – Sinus tachycardia, P pulmonaleSputum
GS & AFB – negativeCulture – no growthCytology – no malignant cells.
CXR-CT chest-
CTS opinion
imp: Pleural Mesothelioma Adv: CT guided biopsy
Medical oncology:imp: Mesothelioma Stage IVAdv: CT guided/open Biopsy
Review with results
CT guided biopsy was fixed in GHPatients dyspnea and chest pain
persistedSpO2 – 92-94% with O2On 4th day of admission..patient went
into sudden cardiac arrestResuscitated and recovered.Intubation-Mechanical Ventilation
givenAgain 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 industryInsulators PipefittersShipyard workers Brake mechanics Railroad workersConstruction
trades CarpentersPlumbersElectricians
PaintersNon-asbestos
minersWeldersMachinistsManufacturers of
mineral productsMaintenance 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
mesotheliomaHigh degree of aneuploidy, but no single
oncogene or tumor suppressor gene found culprit
p16INK4A-p14ARF(9p21), (NF2) gene (22q12)P53 & Kras – not mutated1p, 3p, 6q, 9p, 15q, and 22qGrowth-promoting genes – PDGF, EGFRlong latency period-multiple genetic
abnormalities
Constitutional symptoms
Weight loss (30%), cough (10%) and fatigue are not common in the early presentation.
HPOA and intermittent hypoglycemia are unusual
Serous effusionsMost common presentation - 95%Dyspnoea (40–70%)Non-pleuritic chest pain (60%)
Local invasionDirect 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% PMOften clinically silent and rarely cause of
deathHilar or mediastinal lymph node
metastasis -44%Intracranial metastases-3%‘Miliary mesothelioma’- rare.
Pleural effusion or pleural mass Large effusions or tumour masses-
mediastinal 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
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%)
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
erosionLung encased with tumor, volume loss with a shift
of mediastinum toward side of primary tumor Signs of lymphatic metastasis – lateMediastinal 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
No specific haematological or biochemical test
Anemia, thrombocytosis, high ESR, hypergammaglobulinaemia.
Only serum biomarker clinically useful is serum mesothelinHigh 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 pleuroscopyLarge samplesDrainage of effusionsFreeing up of a trapped
lungTalc pleurodesis if lung not
trappedBronchoscopy, BAL and Ga
scan-no useful role
Median survival- 4 -12 mo from the time of
diagnosisEpithelial cell type do best and those with
the sarcomatous cell type the worstPoor Prognosis
Age, male gender, performance status, leukocytosis, and chest painmicrovessel densitytumor necrosis
Surgical Therapy - Debulking
Pleurectomy with Decortication (P/D)Extrapleural Pneumonectomy (EPP)Epithelial cell type, clean margins after
resection, and negative lymph nodesTumor debulking using EPP followed by
chemotherapy and high-dose radiation therapyChemotherapy
Pemetrexed – CisplatinumGemcitabine with a platinum agent
Radiation TherapyLimited to adjunctive therapy
Drugs
Gefitinib and ImatinibThalidomideSuperoylanilide hydroxamic acid (SAHA)-
histone deacetylase inhibitorProteasome inhibitorsBevacizumab
ImmunotherapyIntrapleural interferon-γInfusion of interleukin-2
Gene Therapy
Palliative Therapy
Pain managementPleurodesisPlacement of a tunneled pleural catheter
Chemoprevention and ScreeningScreening of high-risk populations Sensitivity of serum mesothelin is not sufficient
for use as a marker Daily vitamin A (retinol) or β-carotene - trialRoutine low-dose CT scanning - trials
1. 2010 – Murray and Nadel's Textbook of Respiratory Medicine, 5th ed
2. 2010 – Pleural Disease- Second Edition
3. 2008 – Fishman’s Pulmonary Diseases and Disorders-Fourth Edition
4. 2002 – Mesothelioma
5. 2002 – Crofton And Douglas’s Respiratory Diseases – Fifth Edition