mediastinal tumours
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INTRODUCTION
MEDIASTINUM is the central compartment in the thoracic cavity between the two lungs Any age group-both sexes (often on routine x ray) (50% are asymptomatic)
ANATOMY
• Anteriorly : Sternum• Posteriorly : Thoracic vertebrae• Superiorly : Plane of thoracic inlet• Inferiorly : Diaphragm• Laterally : Mediastinal pleura
CROSS SECTION
Anterior - 1 Middle - 2 Posterior - 3
http://mywebpages.comcast.net/wnor/thoraxlesson3.htm
SUPERIOR MEDIASTINUM MUSCLES : Origins of sternohyoid ,sternothyroid,lower ends of
longus colli ARTERIES : aortic arch,brachiocephalic artery,thoracic portions
of left common carotid, and left subclavian artery VEINS : brachiocephalic vein,upper part of SVC,left highest
intercostal vein NERVES : vagus,superficial and deep cardiac plexus,phrenic
nerve,left recurrent larnygeal nerve Trachea Oesophagus Thoracic duct Remains of thymus lymph glands
ANTERIOR MEDIASTINUM Loose areolar tissue Some lymphatic vessels which arise from the
convex surface of the liver 2 or 3 mediastinal lymph nodes small mediastinal branches of internal mammary
artery Thymus
MIDDLE MEDIASTINUM
PericardiumHeartDescending aortaLower half of SVC and azygous veinsRight and left pulmonary veinsTracheaTrachea BifurcationMain BronchiPhrenic NerveHilar Lymph NodePericardiophrenic vessels
POSTERIOR Esophagus Vagus Splanchnic nerves Thoracic duct Thoracic descending Aorta Azygos Vein and accessory hemiazygous vein Hemi-azygos vein Paravertebral Lymph node
CLASSIFICATION - ANATOMICALANTEROSUPERIOR: - Thymic neoplasms - Cysts - Mesenchymal - Lymphomas - Germ cell tumours - Carcinoma - Endocrine MIDDLE: - Cysts - Mesenchymal - Lymphomas - Carcinoma POSTERIOR MEDIASTINAL: - Neurogenic - Mesenchymal - Cysts - Endocrine
CLASSIFICATION- PATHOLOGICAL NEUROGENIC TUMOURS - MOST COMMON : NEUROFIBROMA, NEURILEMOMA, GANGLIONEUROMA, NEUROBLASTOMA, NEUROSARCOMA OTHERS : CHEMODECTOMA, PARAGANGLIOMA
• THYMOMA - BENIGN, MALIGNANT• LYMPHOMA – HODGKINSDISEASE , • T AND B IMMUNOBLASTIC SARCOMA,
SCLEROSING FOLLICULAR CELL• GERM CELL TUMOURS- ,
TERATODERMOID(BENIGN AND MALIGNANT) SEMINOMA, NONSEMINOMA (EMBRYONAL,CHORIOCARCINOMA,ENDODERMAL )
• MESENCHYMAL TUMOURS – • FIBROMA,/FIBROSARCOMA,LIPOMA/ LIPOSARCOMA,
LEIOMYOMA/LEIOMYOSARCOMA, RHABDOMYOSARCOMA,MYXOMA, MESOTHELIOMA,XANTHOGRANULOMA, HEMANGIOMA,HEMANGIOENDOTHELIOMA,HEMANGIOPERICYTOMA LYMHANGIOMA,LYMPHANGIOMYOMA,,LYMPHANGIOPERICYTOMA
• ENDOCRINE – INTRATHORACIC THYROID,PARATHYROID ADENOMA/CARCINOMA CARCINOID
• CYSTS – BRONCHOGENIC, PERICARDIAL, ENTERIC, THYMIC, THORACIC DUCT NONSPECIFIC
• GIANT LYMPH NODE HYPERPLASIA – CASTLEMAN'S DISEASE• CHONDROMA• EXTRAMEDULLARY HEMATOPOIESIS
Common Pathologies• ANTEROSUPERIOR MEDIASTINUM - THYMIC NEOPLASM-33% LYMPHOMA-19% GERM CELL TUMOUR-17%• MIDDLE MEDIASTINUM - CYSTS-61%,LYMPHOMA-21%• POSTERIOR MEDIASTINUM - NEUROGENIC-53%,CYST-32%
CHILDREN
Posterior Mediastinum Most often benign 2/3 of tumors symptomatic
Neurogenic tumors(40%)Lymphoma(18%)Cysts(18%)Germ cell tumors(11%)Mesenchymal tumors(9%)Thymomas -Rare
ADULTS
Anterior Mediastinum Often Malignant Ages 30 – 50 1/3 of tumors are symptomatic
Neurogenic tumours(21%)Cysts(20%)Thymomas(19%)Lymphoma(13%)Germ cell tumours(11%)Mesenchymal tumours(7%)Endocrine tumours(6%)
MALIGNANCY
All tumors 25% Per Section
Anterior Superior 59% Posterior 16% Middle 16%
Symptoms Chest pain Dyspnea Cough Fever Weight loss Fatigue Dysphagia Night sweats
Clinical Features
SVC syndromeMyasthenia gravis, Immune deficiency, Aplastic anemia - ThymomaSympathetic ganglion - Horner’s SyndromeIn late stages : Diaphragmatic palsyPleural EffusionHaemorrage (Erosion of major vessels)
Malignant Tumors Invasion Structure
Tracheobronchial tree and lungs Esophagus Superior Vena Cava Pleura and Chest Wall Intrathoracic nerves
CLINICAL MANIFESTATIONS OF ANATOMIC COMPRESSION OR INVASION BY NEOPLASMS OF THE MEDIASTINUM
Vena caval obstruction Pericardial tamponadeCongestive heart failureDysrhythmiasPulmonary stenosisTracheal compressionEsophageal compressionVocal cord paralysisPostobstructive pneumonitis
Horner's syndrome
Phrenic nerve paralysis
Chylothorax
Chylopericardium
Spinalcord compressive syndrome
Pancoast's syndrome
DIAGNOSTIC EVALUATION History and Physical examination Radiology - Standard chest films ,Barium swallow, Fluroscopy ,Arteriography,Venography, CT,MRI,USG,Myelography Radioisotope scanning SerologyEndoscopy Bronchoscopy Needle aspiration and biopsy Operative procedures – Mediastinoscopy, Mediastinotomy Thoracotomy
ULTRASTUCTURAL CHARACTERISTICS OF MEDIASTINAL TUMOURS
Carcinoid : Dense core granules,fewer tonofilaments and desmosomes
Lymphomas : Absence of junctional attatchments and epithelial features
Thymoma : Well formed desmosomes ,bundles of tonofilaments
Germ cell : Prominent nucleoli ,even chromatin, scant desmosomes, rare tonofilaments
Neuroblastoma :Neurosecretory granules ,synaptic endings
Treatment Thoracotomy and removal If malignant - Adjuvant therapy like radiotherapy & chemotherapy Sternotomy - Sup. and ant.tumours
Thymic cancers require surgery, followed by radiation or chemotherapy. Types of surgery include thoracoscopy (a minimally invasive approach), mediastinoscopy (minimally invasive) and thoracotomy (a procedure performed through an incision in the chest).
Lymphomas are recommended to be treated with chemotherapy followed by radiation.
Neurogenic tumors found in the posterior (back) mediastinum are treated surgically.
THYMOMAS Most common tumour of the anterosuperior mediastinum in adults Fifth to Sixth decade Both sexes are equally affected
Types HISTOLOGY: Epithelial cell - Poor prognosis Lymphocytic Mixed Spindle - Better prognosis
50% THYMOMAS ARE MALIGNANT
Clinical features: ASYMPTOMATIC 50% 30 - 40% ASSOCIATED WITH MYASTHENIA GRAVIS Chest pain Dysphagia Dyspnoea SVC obstruction
Modified Masaoka clinical staging of thymoma
Stage DefinitionI Macroscopically and microscopically completely encapsulatedIIA Microscopic transcapsular invasionIIB Macroscopic invasion into surrounding fatty tissue or grossly adherent to but not through
mediastinal pleura or pericardiumIII Macroscopic invasion into neighboring organs (ie, pericardium, great vessels, or lung)IVA Pleural or pericardial disseminationIVB Lymphogenous or hematogenous metastasis
Investigations
Chest Xray : Lateral view - Opacity in mediastinum
Mediastinoscopy & biopsy. Tensilon Diagnostic test : Injecting 10mg
edrophonium chloride iv. Myasthenia is relieved within 1 min temporarily
CT scan
Treat myasthenia - Neostigmine
Thymectomy IS BENEFICIAL in: Disease < 5yrs Myasthenia without thymoma In young females
Treatment
Surgical removal of the tumor is the preferred treatment. Surgery is often the only treatment required for stage I tumors. Treatment of thymoma often relieves the symptoms caused by paraneoplastic syndromes. Stages II, III, and IV thymomas are often treated with surgery and some form of adjuvant therapy.
RETROSTERNAL GOITRE
> 50 % goitre below the suprasternal notch. PRIMARY : rare (1%) -SECONDARY : common
PRIMARY Arises from ectopic thyroid tissue from mediastinum. It gets it blood supply from mediastinum itself,not from the neck.Not related to existing thyroid tissue in the neck
SECONDARY
Extension from an enlarged thyroid from the neck Arises from lower pole of a nodular goitre usually. Commonly seen in short neck or obese individuals Nodule gets drawn into the superior medistinum due To negative intrathoracic pressure
TYPES Substernal type : part of the nodule is palpable in
the lower neck Plunging goitre : an intrathoracic goitre is
occasionally forced into the neck by increased intrathoracic pressure
Intrathoracic goitre: neck is normal
CLINICAL FEATURES SYMPTOMS :Dyspnoea Cough and stridorDysphagiaSIGNS :Engorgement of neck veins and superficial veins on the chest wallLower border is not seen on inspection and not felt on palpation
PEMBERTON'S SIGN : is positive Percussion :dull note over sternum Can be nodular,toxic or malignant Rarely – recurent larygeal nerve palsy
INVESTIGATIONS
Chest X -ray CT scan and MRI is diagnostic
TREATMENT Surgical removal Commonly through incision in neck Large goitre or malignant type median sternotomy
is required rarely
Radiodine therapy is not not accepted Stridor –due to compression of tracheobronchial
tree it is very dangerous, as often it is not possible to clear airway either by intubation or tracheostomy
Surgical removal should be complete : recurrence – very difficult to operate
Neurogenic tumors
Neurofibroma,NeurilemmoasMelanotic schwannoma
Ganglioneuromas
Chemodectoma Pheochromocytomas
Benign Malignant
Neurofibrosarcomas
GanglioneuroblastomaNeuroblastomas
Malignant chemodectomaMalignant Pheochromocytoma
Neurilemmomas
Most common
Arises from the Schwann cells of the nerve sheathWell encapsulated
X-ray :dense homogenous mass in the posterior mediastinumSurgical excision
Neurofibromas
Arises from the nerve sheath and nerve fibres Poorly encapsulated
Treatment :Difficult to excise due to its infiltrating natureMultiagent chemotherapy due to aggressive nature
Ganglioneuromas
Originates from sympathetic chain Composed of ganglionic cells and nerve fibresMore common in children Mostly asymptomaticUsual location is paravetebral regionINVESTIGATION: X ray- well marginated lesion on
anterolateral aspect of spine TREATMENT: surgical resection
Neuroblastomas
Usually seen among young childrenMost poorly differentiated tumor Arising from the sympathetic nervesHighly invasive tumor 75% -children clinical feature is fever cough
diarrhea vomitingAt time of presentation – pain,neurological defects,Horners
syndrome,respiratory distress ataxia-lymph node metastasis Metastasis to spinal cordInvestigation : CT,MRI,radionucleide imagingTreatment :Radiotherapy and chemotherapy
Germ cell tumors
Benign Malignant
Seminomatous Non seminomatous
Choriocarcinomas
Embryonal carcinomas
Mixed tumors
Teratocarcinomas
Yolk sac carcinomas.
LAB STUDIES
Beta human chorionic gonadotropin
Alpha-fetoprotein
IMAGING
Chest radiography
CT scan of the chest and mediastinum
DIAGNOSTIC PROCEDURES
Transthoracic needle biopsy
Cervical mediastinoscopy and substernal extended mediastinoscopy
Anterior mediastinotomy
Video-assisted thoracoscopy
Sternotomy and thoracotomy
TERATOMA is the commonest. Symptoms: usually asymptomatic, cough, dyspnoea and chest pain can
occur. Investigations : Chest Xray, CT, MRI Treatment: complete surgical excision and chemotherapy.
SEMINOMA: 25- 50% of GCT Men 20- 40 yrs Symptoms: dysnoea, substernal pain, weakness, gynaecomastia, SVC
syndrome Investigations: chest Xray, CT, Treatment: radiotherapy
Non Seminoma: Embryonal cell carcinoma, endodermal thymus
tumours, choriocarcinoma, yolk sac tumours with multiple cellular components.
Symptoms: chest pain, hemoptysis, cough, fever, wt loss, gynaecomastia
Investigation: AFP, beta HCG are increased. Treatment: chemotherapy with bleomycin,
etoposide and cisplatin If there is residual tumour then = surgery
Lymphomas
Commonly situated in anterior mediastinumCommonest Hodgkin's lymphomasCommon in 40-60 yrsNon Hodgkin’s affects any age. Symptoms: fever, wt loss, night sweats, compressive
symtoms like pain, dyspnoea, wheezing, SVC syndrome and pleural effusion.
Investigations : x-ray , CT scan, cervical lymph node biopsyTreatment: Combination chemotherapySurgery not usually done.
Vascular and Lymphatics
Hemangiomas Lymphangiomas HaemangimyomasLymphangimyomas
Commonest is lymphangioma(cystic hygroma)Seen in ant mediastinum
Mediastinal cysts
Primary cystPericardial cyst
Bronchogenic cystEnteric cystThymic cyst
Thank You…
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