Superior Vena Cava Obstruction

IntroductionPresent case #1

Review imagesAbnormal radiological findingsWhat might this patient feel? (name 6) – clinical presentationDifferential diagnosis (name 4) – causes of SVCOWhat is the next step in diagnosis? – diagnosis of mediastinal mass

Clinical presentation (2-4)Facial edema 82%Dyspnea 54-66%Cough 50-54%Distended neck veins 38-63%Distended chest veins 53%Arm edema 46-68%Facial plethora 20%Hoarseness 17%

Syncope 10%Headache 9%Dizziness 6%Stridor 4%Confusion 4%Obtundation 2%Visual symptoms 2%

Causes of SVCO (6-7)Non-malignant

HistoricSyphilitic aortic aneurysmTBFibrosing mediastinitis (histoplasmosis)

CurrentThrombosis

MalignantNSCLC 50%SCLC 22%Lymphoma 12%

DLBCL and lymphoblastic lymphomas typicallyMetastatic 9%Germ-cell cancer 3%Thymoma 2%Mesothelioma 1%

Pathophysiology (8-10)What is the central venous pressure? 20-40 mm Hg (normal 2-8 mm Hg)Liver hot spot

Diagnosis (11-15)Contrast-enhanced CTPET

Diagnosis of mediastinal mass (16)Lung: sputum cytology, lymph node biopsy, pleural fluid cytology (50% diagnostic)More invasive techniques: CT-guided biopsy (75% diagnostic), EBUS, bronchoscopy (50-70% diagnostic), mediastinoscopy/mediastinotomy (90%), VATS, thoracotomy

Lymphoma: excisional lymph node biopsy > core needle biopsy > FNA. Also consider bone marrow biopsy.

Complications (17-18): Some studies suggest a higher rate of complications with mediastinal procedures among those with SVCO, but the absolute rate is quite low.

EvidenceAnn Thorac Surg. 1999;68(1):223: 80 patients undergoing cervical mediastinoscopy due to diagnostic uncertainty: 5 patients had significant bleeding, 1 patient required sternotomy, diagnosis obtained in all 80.Chest 2005;128:1551 (18): Case control study of 39 SVCO cases and 367 controls, all undergoing mediastinal procedures [cervical mediastinoscopy (CMDS) and anterior mediastinotomy (AMDT)]. Complication rate of SVCO cases (6 cases, 15.4%) > non-SVCO cases (1.1%), with p < 0.001.

The six complications were: major hemorrhage (2), airway obstruction (2), tracheal laceration (1), superficial wound infection (1)

Management (19)Is it a radiotherapeutic emergency? (20)Not necessarily. Rowell et al.: 107 cases reviewed, with many having a prolonged period between definitive therapy and symptom onset, showed no serious complications from SVCO or diagnostic procedures to workup the SVCO.

Jumping in may have consequences. J Clin Oncol. 1986;4(5):716: 19 cases of RT without pathologic diagnosis reviewed; 8 (42%) had non-diagnostic biopsies after RT commenced (thought to be lymphoma or seminoma)

True emergencies: stridor, laryngeal edema, coma from cerebral edema – require definitive treatment (stent and RT).

Obstruction (21)1. Supportive careElevate patient’s headSteroids? Dexamethasone 4 mg q6h: case reports only

Likely to help with lymphoma and thymomaUpToDate: May help with laryngeal edema associated with SVCO. For SVCO itself with non-responsive malignancies like NSCLC, steroids have not studied and are not indicated in these other malignanciesRowell et al.: No evidence, yet part of standard management.

Loop diuretics? Unclear whether lowering venous pressure distal to obstruction is of benefit

Evidence

Am J Med. 1981;70(6):1169: retrospective observation study of 107 patients showed rate of improvement (84% of patients) was similar between glucocorticoids, diuretics, or neither.

Special cases:Avoid arms for injection due to venous stasis in upper extremitiesThrombosis: Anticoagulation, ± removal of catheter, ± thrombolysis

2. Definitive treatment (22)Need tissue diagnosis

a. ChemotherapyNHL (complete relief of symptoms in 80%)SCLC (complete relief of symptoms in 40-77%)Germ cell tumours

b. RadiationSCLC (complete relief of symptoms in 63%)NSCLC (complete relief of symptoms in 78%)Improvement often within 72 h; degree of radiologic improvement is less than clinical improvement. Patients feel better faster than their cancer actually shrinks. This is attributed to collateral drainage, and has led some to question the value of emergent RT.

SVCO patients may still be curable; keep this in mind before urgent RT.

Radiotherapy techniques:Gross disease and adjacent nodesFractionation as per histology (lymphoma or lung cancer)

SCLC: 40 Gy/15, 50 Gy/25, 60 Gy/30, or 45 Gy/1.5 Gy bid x 3 weeksNSCLC: 60-66 Gy/30-33

Palliative: 30 Gy/10, 20 Gy/5, 37.5 Gy/15 (Roach), 12 Gy/2 (poor PS)

c. Stent (self-expanding) (23)Does not need tissue diagnosisGood for immediate palliation of symptoms, especially for radioresistant mesothelioma (not as important for SCLC/lymphoma); resolves within hours-daysTechnical success rate: 95-100%

Inserted via IJ, SC, or FV; kissing stents (serial) are sometimes usedTotal occlusion: use thrombolysis or angioplasty first

Relief of symptoms: 95%

Complications: 3-7% (infection, PE, migration, hematoma, bleeding, perforation)Anticoagulation is controversial but generally suggested

Warfarin 1 mg po daily (INR ≤1.6) or clopidogrel 75 mg po daily + ASADuration is controversialUse of thrombolytics increases complication rate

d. Surgery (24)Thymoma – neoadjuvant chemotherapy, surgery, adjuvant RT

Evidence (25)Clinical Oncology (2002) 14: 338–351: A systematic review of 2 RCT and 44 non-randomized studies.

SCLC: Chemotherapy 76.9% relief; CRT 83.3% relief; RT (no prior chemo) 94.4%; RT (prior chemo) 70.4%, RT overall (77.6%); any treatment overall 77.4% (Table 3 - 26)NSCLC: Chemotherapy 59% relief, CRT 31.3% relief; RT 63% relief; any treatment overall 59.8% relief (Table 4 - 27)Stent: 95% overall (Table 5 - 28)Rapidity of response: chemo/RT: 7-21 d; stent 24-72 h (29)Relapse rates: lower with stents (11% vs 17-19% with chemo/RT); also better because stents better at relieving symptoms to begin with; easy to re-stent

PrognosisNatural history of SVCO

Usually gets better over hours-weeks due to collateral drainageMalignancy-related

Survival does not differ significantly among patients with same tumour type and disease stage, but without SVCO

Applying knowledgePresent case #2 (SCLC 2007, continued smoking, NSCLC 2012)

Review images (early)Review images (later)

Collateral drainageReview images (stent)Review treatment plan

60 Gy/30 fractions, 4 fieldOARs: cord (50 Gy), plexus (70 Gy), lung, esophagus

SVCO

In distressStridor, laryngeal

edema, cerebral edema

Urgent steroids, stent, radiotherapy

Symptomatic

StentProceed to "No

distress"

No distress

Obtain pathologic diagnosis

Lymphoma

Chemotherapy, radiotherapy, ±steroids

SCLC

Chemotherapy, radiotherapy

NSCLC

Radiotherapy, chemotherapy

Thymoma

Chemotherapy, surgery, then radiotherapy

Tailored treatment depending on diagnosis

Key references1. Wan J.F., Bezjak A. Superior vena cava syndrome. Hematol Oncol Clin N Am 24 (2010)

501–5132. Drews et al. Malignancy-related superior vena cava syndrome. UpToDate 2012.3. Rowell et al. Steroids, radiotherapy, chemotherapy and stents for superior vena caval

obstruction in carcinoma of the bronchus: a systematic review. Clinical Oncology 2002; 14:338-351.

4. Wilson et al. Superior Vena Cava Syndrome with Malignant Causes. N Engl J Med 2007;356:1862-9.