Radiation Oncology Presentation

Superior
Vena Cava
Syndrome
Kaviya Kalyanasundaram and Lucy Xiong
 Definition and aetiology

Clinical features

Agenda Diagnosis

What we'll discuss today Classifications

Management
Definition
Partial or complete obstruction of the SVC
causing venous congestion of the head,
neck and upper extremities
Aetiology

Malignant 60-85% Nonmalignant

Most common: - Thrombosis from

>80% caused by intravasvular devices 20-40%
- NSCLC 50% - Fibrosis from RT, infection,
- SCLC 25-35% autoimmune, nocardiosis,
- NHL 10-15% agenesis of SVC, etc.
Less common: metastases, Historically: syphilitic arotic
ferm cell tumours, thymoma, aneurysm, TB mediastinal
mesothelioma adenopathy
Clinical Features
Haemodynamic: Raised ICP:
Oedema Headache
Prominent veins Dizziness
JVP Confusion
Orthostatic hypotension Visual impairment
Syncope
Renal failure

Neck congestion:
Dyspnoea
Cough
Vocal hoarseness
Stridor
Dysphagia
Chronic vs acute
Chronic
- Collateral venous dilation
- Relieves pressure, delays symptom onset
- Takes several weeks
- 4 collateral venous systems: azygos, internal
mammmary, lateral thoracic, vertebral

Acute
- More severe clinical features
Diagnosis

Stable
- CT with venogram
- US

Unstable
- Digital subtraction venography with/without
stent: Gold standard
- Second line: MR venography
Proposed grading system
Based off clinical findings
Anatomical
Classification
Stanford Classification

Type I: partial obstruction SVC with patency and

antegrade flow in azygos vein
Type II: near-complete obstruction SVC with patency and
antegrade flow in azygos vein
Type III: complete obstruction SVC with reversal of azygos
blood flow
Type IV: complete obstruction SVC and azygos systems
with development of chest-wall and internal mammary
collaterals
General Management
Principles
Goals: Alleviate symptoms and treat the underlying
disease.

Factors that affect treatment choice:

- Type of cancer
- The extent of disease,
- Overall prognosis, which is closely linked to
histology and whether or not prior therapy has been
administered.
Patients with Life-
Threatening Symptoms

Symtoms of emergency First line Immediate intervention

Central airway obstruction, Initial stabilization, secure Endovenous recanalization ±

Severe laryngeal edema, airway, support breathing, SVC stent placement
Coma from cerebral edema and circulation
Stent>RT in life threatening situations
Endovascular recanalization with or without stenting is a faster way to relieve
symptoms.

RT given prior to biopsy may obscure the histologic diagnosis.

If RT is needed, it can be deferred until after severe symptoms have been relieved
through endovascular techniques, and a biopsy is secure, without posing a hazard
to a clinically stable patient.

Patients Without Life Threatening Symptoms

Stenting
For tumours that are not Chemo
or radiation-sensitive eg Non-
small cell lung cancer and
pleural mesothelioma.
Radiotherapy
For radiosensitive tumors in patients
with other less-chemotherapy-
sensitive malignancies

For those who have not been
For those with recurrent SVC previously irradiated
syndrome who have previously
received systemic therapy or RT.
Patients Without Life Threatening Symptoms
Chemotherapy Surgery
Rarely considered
For Chemotherapy-sensitive

malignancies such as small cell For thymoma and thymic carcinoma as a

lung cancer (SCLC), non-Hodgkin component of a multimodality approach to
treatment
lymphoma (NHL), or germ cell

cancer and possibly breast cancer

For patients with residual masses after
treatment of a germ cell tumor.

Thank you!
 References
Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore) 2006; 85:37.
Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med 2007; 356:1862.
Yellin A, Rosen A, Reichert N, Lieberman Y. Superior vena cava syndrome. The myth--the facts. Am Rev Respir Dis 1990; 141:1114.
Schraufnagel DE, Hill R, Leech JA, Pare JA. Superior vena caval obstruction. Is it a medical emergency? Am J Med 1981; 70:1169.
Markman M. Diagnosis and management of superior vena cava syndrome. Cleve Clin J Med 1999; 66:59.
Rowell NP, Gleeson FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic
review. Clin Oncol (R Coll Radiol) 2002; 14:338.
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.
Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990; 160:207.
Mose S, Stabik C, Eberlein K, et al. Retrospective analysis of the superior vena cava syndrome in irradiated cancer patients. Anticancer Res 2006; 26:4933.
Kee ST, Kinoshita L, Razavi MK, et al. Superior vena cava syndrome: treatment with catheter-directed thrombolysis and endovascular stent placement.
Radiology 1998; 206:187.
Ahmann FR. A reassessment of the clinical implications of the superior vena caval syndrome. J Clin Oncol 1984; 2:961.
Dombernowsky P, Hansen HH. Combination chemotherapy in the management of superior vena caval obstruction in small-cell anaplastic carcinoma of the
lung. Acta Med Scand 1978; 204:513.
Spiro SG, Shah S, Harper PG, et al. Treatment of obstruction of the superior vena cava by combination chemotherapy with and without irradiation in small-cell
carcinoma of the bronchus. Thorax 1983; 38:501.
Sculier JP, Evans WK, Feld R, et al. Superior vena caval obstruction syndrome in small cell lung cancer. Cancer 1986; 57:847.
Urban T, Lebeau B, Chastang C, et al. Superior vena cava syndrome in small-cell lung cancer. Arch Intern Med 1993; 153:384.
Würschmidt F, Bünemann H, Heilmann HP. Small cell lung cancer with and without superior vena cava syndrome: a multivariate analysis of prognostic factors
in 408 cases. Int J Radiat Oncol Biol Phys 1995; 33:77.
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.
Kvale PA, Selecky PA, Prakash UB, American College of Chest Physicians. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd
edition). Chest 2007; 132:368S.
Drews, R. and Rabkin, D., 2021. Malignancy-related superior vena cava syndrome. [online] Uptodate. Available at:
<https://www.uptodate.com/contents/malignancy-related-superior-vena-cava-syndrome?search=https:%2F%2Fwww-uptodate-
com.proxy.library.adelaide.edu.au%2Fcontents%2Fmalignancy-related-superior-vena-cava-
syndrome%3Fsearch%3Dsvc%2520syndrome%26source%3Dsearch_result%26selectedTitle%3D1~150%26usage_type%3Ddefault%26display_rank%3D1%23H2
363630142&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1> [Accessed 4 October 2021].
Loeffler JS, Leopold KA, Recht A, Weinstein HJ, Tarbell NJ. Emergency prebiopsy radiation for mediastinal masses: impact on subsequent pathologic diagnosis and
outcome. J Clin Oncol. 1986 May;4(5):716-21. doi: 10.1200/JCO.1986.4.5.716. PMID: 3701390.
Ameku, K., Higa, M. and Ganaha, F., 2020. Stanford type IV venous collateral blood flow following complete chronic occlusion of the superior vena cava in a
patient with lung cancer. Radiology Case Reports, 15(3), pp.254-258.
JACC: Cardiovascular Interventions, 2020. Superior Vena Cava Syndrome. 13(24), pp.2896-2910.
Yu, J., Wilson, L. and Detterbeck, F., 2008. Superior Vena Cava Syndrome—A Proposed Classification System and Algorithm for Management. Journal of Thoracic
Oncology, 3(8), pp.811-814.