Oncological Emergencies

1. Oncological Emergencies Dr. Gary Harding MD, FRCPC Medical Oncology Fellow CancerCare Manitoba

2. CASE 1…

3. Mr. SV • ID: 65 year old male with PMHx of CAD and emphysema • EC: present to clinic with one week history of increasing SOB • HPI: 3 month history of weight loss, decreased appetite, a change in his chronic cough, and intermittent hemoptysis

4. On Physical Examination • Inspection:

5. Respiratory Examination • Stridor • Dullness to percussion on right lower lung fields • Increased tactile fremitus to right lower lung fields • Decreased A/E to right lower lung fields

6. Chest X-Ray…

7. right pleural effusion

8. Thoracentesis • Exudate • Gram stain • Negative • AFB stain • Negative • Cytology • non-small cell lung cancer • Large cell type

9. T1-weighted axial MRI demonstrating paratracheal soft tissue mass that invades into the SVC

10. Superior Vena Cava Syndrome

11. Definition • Obstruction of blood flow in the superior vena cava results in signs and symptoms of SVC syndrome

13. Etiology • Caused by either invasion or external compression of the SVC by contiguous pathologic process • Right lung pathology, lymph nodes, other mediastinal structures, or thrombosis

14. Etiology • Before antibiotics the most common causes were from complications of untreated infection • Syphilitic thoracic aneurysms • fibrosing mediastinitis • Malignancy is presently the most common cause

15. Symptoms and Signs • As the obstruction develops venous collaterals are formed • Symptom onset depends on speed of SVC obstruction onset • Malignant disease can arise in weeks to months • Not enough time to develop collaterals • Fibrosing mediastinitis can take years to have symptoms

16. Symptoms and Signs • Central venous pressures remain high even in collaterals • High pressures cause the characteristic clinical picture • Shortness of breath is the most common symptom1 1. Parish, JM, Marschke, RF Jr, Dines, DE, Lee, RE. Etiologic considerations in superior vena cava syndrome. Mayo Clin Proc 1981; 56:407.

17. Signs and Symptoms • Facial swelling or head fullness • exacerbated by bending forward or lying down • Cough • Arm edema • Cyanosis

18. Facial swelling associated with SVC Syndrome in a patient with malignancy

19. Physical Findings • Venous distension • neck • chest wall • Pemberton’s Sign • Facial Edema

20. Patient who presented with progressively enlarging veins over the anterior chest wall. A diagnosis of a right-sided superior sulcus (Pancoast) tumor compressing the SVC was made.

21. Etiology: Malignancy • Lung cancer is the most common2 • Lymphoma is second most common • together represent 94% of cases 2. Escalante, CP. Causes and management of superior vena cava syndrome. Oncology (Huntingt) 1993; 7:61.

22. NSCLC • 2-4% of bronchogenic cancer patients develop SVC syndrome3 • extrinsic compression or direct invasion • primary tumor or by enlarging mediastinal nodes 3. Armstrong, BA, Perez, CA, Simpson, JR, Hederman, MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys 1987; 13:531.

23. Small Cell Lung Cancer • Greatest risk • 20% will develop SVC obstruction3 • more common because SCLC tends to occur centrally in contrast to other types

24. Lymphoma • 2-4% of patients • predominantly non-Hodgkin’s lymphoma4 • Hodgkin’s rarely causes SVC syndrome 4. Perez-Soler, R, McLaughlin, P, Velasquez, WS, et al. Clinical features and results of management of superior vena cava syndrome secondary to lymphoma. J Clin Oncol 1984; 2:260.

25. Lymphoma • Extrinsic compression caused by enlarging lymph nodes • subtypes of large B cell can be intravascular and cause occlusion (angiotropic) • diffuse large cell and lymphoblastic are most commonly associated with SVC syndrome

26. Other cancers • Thymoma • primary mediastinal germ cell neoplasm • solid tumors with mediastinal nodal metastases • breast cancer most common type

27. Other causes • Post radiation local vascular fibrosis can also be considered in oncology patients • Thoracic radiation treatment may predate syndrome by many years

28. Other causes • Thrombosis • Indwelling central venous catheters • Subcutaneous tunneled catheters have fewer thrombotic and infectious complications • Can also cause pulmonary embolism5 5. Sivaram, CA, Craven, P, Chandrasekaran, K. Transesophageal echocardiography during removal of central venous catheter associated with thrombus in superior vena cava. Am J Card Imaging 1996; 10:266.

29. Diagnosis • Timely identification of the cause is essential • Radiographic studies are useful • Up to 60% of patients with SVC syndrome related to neoplasm do not have a known diagnosis of cancer6 • Need a tissue biopsy for histologic studies 6. Schraufnagel, DE, Hill, R, Leech, JA, Pare, JA. Superior vena caval obstruction. Is it a medical emergency?. Am J Med 1981; 70:1169.

30. Radiographic Studies • Most patients have an abnormal chest x-ray at presentation • Most common findings are • Mediastinal widening • Pleural effusion

31. CT Chest • Preferred choice • IV contrast • defines the level of obstruction • Maps out collateral pathways • Can identify underlying cause of obstruction

32. Venography • Bilateral upper arm venograpy • superior to CT to define site of obstruction • Does not define cause unless thrombosis is solely responsible

33. Helical CT • With bilateral upper arm IV contrast injection • Best visualization of level of obstruction and cause

34. MRI • Can be useful in patients with IV contrast allergies

35. T1-weighted axial MRI demonstrating the primary tumor and the paratracheal soft tissue mass that invades into the SVC

36. Same patient’s MRI with different technique to further define the intramural mass

37. Histologic Diagnosis • Essential • Guides treatment • Aids in defining prognosis

38. Histologic Diagnosis • Sputum cytology, pleural fluid cytology, biopsy of enlarged peripheral nodes • Bone marrow biopsy for NHL • Bronchoscopy, mediastinoscopy, or thoracotomy are more invasive but sometimes necessary

39. Treatment of Oncologic Causes

40. Treatment • Aimed at underlying cause • Evolution of thought has occurred in recent years

41. Historically SVC syndrome was considered a potentially life-threatening emergency • Standard of care was immediate radiotherapy • Zap now • Ask questions later • The emergent approach is not appropriate for most patients

42. Newer strategies

43. Emergent to Urgent • Symptomatic obstruction is usually a prolonged process • Most patients are not in immediate danger at presentation • Most have time for a full diagnostic work up

44. Emergent to Urgent • Prebiopsy radiation can obscure the diagnosis • Current strategies aim at accurate diagnosis of underlying etiology before therapy

45. Exceptionto new rule • Stridor • Central airway obstruction or laryngeal edema • True medical emergency • Immediate action needed • Possible intubation and ICU admission • Immediate therapy to target obstruction needed

46. Prognosis… Linked to tumor histology and stage at presentation

47. Treatment Sensitive Tumors • NHLs, germ cells, and limited-stage small cell lung cancers usually respond to chemotherapy and or radiation • Can achieve long term remission with tumor specific directed therapy • Symptomatic improvement usually takes 1-2 weeks after start of therapy

48. Note: Corticosteroids • Controversial issue with regards to treatment benefit at presentation

49. Non-small cell lung cancer • SVC obstruction is a strong predictor of poor prognosis • Median survival around 5 months7 • Choice of therapy considers likelihood of response to each modality 7. Martins, SJ, Pereira, JR. Clinical factors and prognosis in non-small cell lung cancer. Am J Clin Oncol 1999; 22:453.

50. Non-small cell lung cancer • Goal usually directed to palliation rather than long term remission • Palliative radiation and chemotherapy can be used