Case presentation

1. Case presentation

2. 75 year old male No comorbities c/o exertional dysnea Insidious and gradual in progression No h/o PND /orthopnea No h/o chest pain,palpitation,decreased urine output,pedal oedema

3. No h/o DM,HTN, TB ,Bronchial asthma h/o contact to TB 20 years back Apetite- n/l 2 kg weight loss over 2 weeks No addictions

4. On Examination Conscious, not tachypnoeic PR - 75/min BP - 170/100mm Temp - 99 deg F Thyroid � n/l Skin - n/l No pallor, icterus, cyanosis,clubbing, LNE , pedal oedema

5. Systemic Examination Chest - tachea deviated to right stony dullness in left infraaxillary,infrascapular area Breath sounds decreased in left infrascapular ,infraaxillary area No rhonchi/crepitations CVS - S1S2 normal,no murmors P/A - soft non tender,no organomegaly NS - motor system-n/l sensory system-n/l Fundus - Normal

6. CBC �n/l, ESR-58 mm in 1st hr LFT-n/l RFT-n/l Sr. electrolytes-n/l ECG - n/l sinus rhythm, no ST-T wave changes Peripheral smear-normal S.uric acid and LDH-n/l

8. BF-protein-4.83 BF-LDH-366 S-LDH-290 BF- ADA-26.1 BF-TG-10 BF-amylase-normal

9. MEDIASTENUM Central space between two pleural cavities Bounded by sternum anteriorly Vertebral column posteriorly Thoracic inlet superiorly Diaphragm inferiorly

10. 65% of mediastinal masses arise from anterior/superior mediatenum 10% in middle 25% in the posterior mediastinum

11. ANTERIOR MEDIASTENUM The anterior compartment (also referred to as the anterosuperior compartment or retrosternal space) is anterior to the pericardium includes the thymus, the extrapericardial aorta and its branches, the great veins, and lymphatic tissue.

12. MIDDLE COMPARTMENT pericardium anteriorly, the posterior pericardial reflection, the diaphragm, and the thoracic inlet. compartment includes the heart, intrapericardial great vessels, pericardium, and trachea.

13. POSTERIOR COMPARTMENT posterior pericardial reflection to the posterior border of the vertebral bodies and from the first rib to the diaphragm. It includes the esophagus, vagus nerves, thoracic duct, sympathetic chain, and azygous venous system

14. anterior compartment Thymic tumors (thymomas , thymic carcinomas, thymic carcinoid tumors, and thymolipomas), thymic cysts germ cell tumors, Hodgkin and non-Hodgkin lymphomas, intrathoracic goiter thyroid tumors, parathyroid adenomas, pericardial cysts �

15. The four T's make up the mnemonic for anterior mediastinal masses:: Thymus Teratoma (germ cell) Thyroid Terrible Lymphoma

16. Middle compartment Lymphomas Aortic aneurysm Bronchogenic cyst Pericardial cyst Oesophageal diverticulum/ carcinoma

17. Posterior compartment Neurogenic tumors Diaphragmatic hernia Aortic aneurysm Meningocele Parasitic cysts-Hydratid cysts Potts disease

18. Cervicothoracic sign The anterior mediastinum stops at the level of the superior clavicle. Therefore, when a mass extends above the superior clavicle, it is located either in the neck or in the posterior mediastinum.�

19. Other causes of benign mediastenal lymphadenopathy -chronic infections -fungal infections-histoplasmosis,coccidiomycosis Non infectious causes-silicosis amyloidosis phenytoin

23. Age distribution Infants and children neurogenic tumors , enterogenous cysts Adults---- thymomas, and thymic cysts . Hodgkin's and non-Hodgkin lymphoma and germ cell tumors are most common between the ages of 20 and 40,

24. CLINICAL FEATURES Trachea/main bronchi-dysnea,stridor,cough Oesophagus-dysphagia SVC-engorged non pulsatile neck veins L recurennt laryngeal nerve-hoarseness of voice Phrenic nerve-hemi diaphragm palsy Sympathetic trunk-horners syndrome Chest pain-chestwall invasion,neural invasion

25. What next ? CT chest/MRI chest MRI �when preoperative determination of tumors invasion of vascular or neural structures is indicated In the evaluation of neurogenic tumours-to detect intraspinal component to nuerogenic tumours

28. CT CHEST with contrast Anterior mediastenal mass -invasive thymoma -and pleural mesothelioma

29. Trucut biopsy �suggestive of non hodgkins lymphoma

30. Non-Hodgkin lymphoma (NHL) consists of a diverse group of malignant solid tumors of the lymphoid tissues variously derived B cell progenitors T cell progenitors mature B cells, or mature T cells

31. Aggressive lymphomas commonly present acutely or subacutely with a rapidly growing mass, systemic B symptoms (ie, fever, night sweats, weight loss), elevated levels of lactate dehydrogenase and uric acid. include diffuse large B cell lymphoma, Burkitt lymphoma, adult T cell leukemia-lymphoma, and precursor B and T lymphoblastic leukemia/lymphoma.

32. Indolent lymphomas insidious slow growing lymphadenopathy, hepatomegaly, splenomegaly, or cytopenias. include follicular lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma

33. �20 percent of patients with NHL present with mediastinal adenopathy on clinical examination or chest radiograph Mediastinal involvement can be seen either as the sole site of disease (eg, primary mediastinal large B cell lymphoma) or as part of systemic disease Patients with mediastinal involvement can present with persistent cough, chest discomfort, or without clinical symptomatology but with an abnormal chest x-ray.

34. Other associated findings in the thorax include pleural and, less commonly, pericardial effusions. Chylothorax, alone or in combination with chylopericardium or chylous ascites, can be present if major obstruction (infiltration, compression) to lymphatic drainage has occurred, either in the chest (thoracic duct) . Pleural disease is seen in up to 10 percent of all patients with NHL at diagnosis

35. Pleural fluid�� Common findings include elevated protein concentration, a lymphocytic predominant pleocytosis, a normal or low glucose concentration. The presence of malignant lymphoid cells may be confirmed by cytology or flow cytometry.

36. Low grade -NHL Radiotherapy. This can be used for localised stage I disease, which is rare. Chemotherapy. This is the mainstay of therapy. Most patients will respond to oral therapy with chlorambucil, which is well tolerated.. Monoclonal antibody therapy. -The anti-CD20 antibody rituximab has been shown to induce durable clinical responses in up to 60% of patients Transplantation. Studies of autologous stem cell transplantation are in progress.

37. High grade-NHL Chemotherapy (> 90%) -The CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisolone) remains the mainstay of therapy. Radiotherapy.- indicated for a residual localised site of bulk disease after chemotherapy, and for spinal cord and other compression syndromes. Monoclonal antibody therapy. When combined with CHOP chemotherapy, rituximab (R) increases the complete response rates and improves overall survival. Transplantation. Autologous stem cell transplantation benefits patients with relapsed chemosensitive disease