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Mediastinal tumours

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Mediastinal tumours
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
Mediastinal tumours
CROSS SECTION
 Anterior - 1
 Middle - 2
 Posterior - 3
http://mywebpages.comcast.net/wnor/thor
axlesson3.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

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Mediastinal tumours

  • 2. 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)
  • 3. ANATOMY • Anteriorly : Sternum • Posteriorly : Thoracic vertebrae • Superiorly : Plane of thoracic inlet • Inferiorly : Diaphragm • Laterally : Mediastinal pleura
  • 5. CROSS SECTION  Anterior - 1  Middle - 2  Posterior - 3 http://mywebpages.comcast.net/wnor/thor axlesson3.htm
  • 6. 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
  • 7. 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
  • 8. MIDDLE MEDIASTINUM Pericardium Heart Descending aorta Lower half of SVC and azygous veins Right and left pulmonary veins Trachea Trachea Bifurcation Main Bronchi Phrenic Nerve Hilar Lymph Node Pericardiophrenic vessels
  • 9. POSTERIOR  Esophagus  Vagus  Splanchnic nerves  Thoracic duct  Thoracic descending Aorta  Azygos Vein and accessory hemiazygous vein  Hemi-azygos vein  Paravertebral Lymph node
  • 12. CLASSIFICATION - ANATOMICAL ANTEROSUPERIOR: - Thymic neoplasms - Cysts - Mesenchymal - Lymphomas - Germ cell tumours - Carcinoma - Endocrine  MIDDLE: - Cysts - Mesenchymal - Lymphomas - Carcinoma  POSTERIOR MEDIASTINAL: - Neurogenic - Mesenchymal - Cysts - Endocrine
  • 13. 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 )
  • 14. • 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
  • 15. 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%
  • 16. 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
  • 17. 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%)
  • 18. MALIGNANCY  All tumors 25% − Per Section  Anterior Superior 59%  Posterior 16%  Middle 16%
  • 19. Symptoms  Chest pain  Dyspnea  Cough  Fever  Weight loss  Fatigue  Dysphagia  Night sweats
  • 20. Clinical Features  SVC syndrome Myasthenia gravis, Immune deficiency, Aplastic anemia - Thymoma Sympathetic ganglion - Horner’s Syndrome In late stages : Diaphragmatic palsy Pleural Effusion Haemorrage (Erosion of major vessels)
  • 21. Malignant Tumors Invasion Structure  Tracheobronchial tree and lungs  Esophagus  Superior Vena Cava  Pleura and Chest Wall  Intrathoracic nerves
  • 22. CLINICAL MANIFESTATIONS OF ANATOMIC COMPRESSION OR INVASION BY NEOPLASMS OF THE MEDIASTINUM Vena caval obstruction  Pericardial tamponade Congestive heart failure Dysrhythmias Pulmonary stenosis Tracheal compression Esophageal compression Vocal cord paralysis Postobstructive pneumonitis  Horner's syndrome  Phrenic nerve paralysis  Chylothorax  Chylopericardium  Spinalcord compressive syndrome  Pancoast's syndrome
  • 23. DIAGNOSTIC EVALUATION  History and Physical examination  Radiology - Standard chest films ,Barium swallow, Fluroscopy ,Arteriography,Venography, CT,MRI,USG,Myelography Radioisotope scanning  Serology Endoscopy  Bronchoscopy  Needle aspiration and biopsy  Operative procedures – Mediastinoscopy, Mediastinotomy Thoracotomy
  • 24. 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
  • 25. Treatment  Thoracotomy and removal  If malignant - Adjuvant therapy like radiotherapy & chemotherapy  Sternotomy - Sup. and ant.tumours
  • 26. • 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.
  • 27. THYMOMAS  Most common tumour of the anterosuperior mediastinum in adults  Fifth to Sixth decade  Both sexes are equally affected
  • 29. Types  HISTOLOGY:  Epithelial cell - Poor prognosis  Lymphocytic  Mixed  Spindle - Better prognosis 50% THYMOMAS ARE MALIGNANT
  • 30. Clinical features:  ASYMPTOMATIC 50%  30 - 40% ASSOCIATED WITH MYASTHENIA GRAVIS  Chest pain  Dysphagia  Dyspnoea  SVC obstruction
  • 31. Modified Masaoka clinical staging of thymoma Stage Definition I Macroscopically and microscopically completely encapsulated IIA Microscopic transcapsular invasion IIB Macroscopic invasion into surrounding fatty tissue or grossly adherent to but not through mediastinal pleura or pericardium III Macroscopic invasion into neighboring organs (ie, pericardium, great vessels, or lung) IVA Pleural or pericardial dissemination IVB Lymphogenous or hematogenous metastasis
  • 32. 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
  • 34.  Treat myasthenia - Neostigmine  Thymectomy IS BENEFICIAL in:  Disease < 5yrs  Myasthenia without thymoma  In young females Treatment
  • 35.  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.
  • 36. RETROSTERNAL GOITRE  > 50 % goitre below the suprasternal notch.  PRIMARY : rare (1%) - SECONDARY : common
  • 37. 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
  • 38. 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
  • 39. CLINICAL FEATURES SYMPTOMS : Dyspnoea  Cough and stridor Dysphagia SIGNS : Engorgement of neck veins and superficial veins on the chest wall Lower border is not seen on inspection and not felt on palpation
  • 40.  PEMBERTON'S SIGN : is positive  Percussion :dull note over sternum  Can be nodular,toxic or malignant  Rarely – recurent larygeal nerve palsy
  • 41. INVESTIGATIONS  Chest X -ray  CT scan and MRI is diagnostic
  • 43. 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
  • 44. Neurogenic tumors Neurofibroma,Neurilemmoas Melanotic schwannoma Ganglioneuromas Chemodectoma Pheochromocytomas Benign Malignant Neurofibrosarcomas Ganglioneuroblastoma Neuroblastomas Malignant chemodectoma Malignant Pheochromocytoma
  • 45. Neurilemmomas Most common Arises from the Schwann cells of the nerve sheath Well encapsulated X-ray :dense homogenous mass in the posterior mediastinum Surgical excision
  • 46. Neurofibromas Arises from the nerve sheath and nerve fibres Poorly encapsulated Treatment : Difficult to excise due to its infiltrating nature Multiagent chemotherapy due to aggressive nature
  • 48. Ganglioneuromas Originates from sympathetic chain Composed of ganglionic cells and nerve fibres More common in children Mostly asymptomatic Usual location is paravetebral region INVESTIGATION: X ray- well marginated lesion on anterolateral aspect of spine TREATMENT: surgical resection
  • 49. Neuroblastomas Usually seen among young children Most poorly differentiated tumor Arising from the sympathetic nerves Highly invasive tumor 75% -children clinical feature is fever cough diarrhea vomiting At time of presentation – pain,neurological defects,Horners syndrome,respiratory distress ataxia -lymph node metastasis Metastasis to spinal cord Investigation : CT,MRI,radionucleide imaging Treatment : Radiotherapy and chemotherapy
  • 51. Germ cell tumors Benign Malignant Seminomatous Non seminomatous Choriocarcinomas Embryonal carcinomas Mixed tumors Teratocarcinomas Yolk sac carcinomas.
  • 52. 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
  • 53.  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
  • 54.  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
  • 55. Lymphomas Commonly situated in anterior mediastinum Commonest Hodgkin's lymphomas Common in 40-60 yrs Non 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 biopsy Treatment: Combination chemotherapy Surgery not usually done.
  • 57. Mediastinal cysts Primary cyst Pericardial cyst Bronchogenic cyst Enteric cyst Thymic cyst