1) The document outlines various treatments for superior vena cava syndrome (SVCS) including radiation therapy (RT), chemotherapy, stenting, and surgery. 2) RT is effective at relieving symptoms in 80% of cases and works rapidly with initial high doses, while chemotherapy can also effectively palliate SVCS in lung cancers and lymphomas. 3) Stenting provides rapid and effective relief in 95% of cases and should be considered for life-threatening presentations or where other treatments are limited. Surgery has a limited role and is mainly used for refractory cases or certain malignancies.

2. Out line of presentation
• Introduction
• Anatomy
• Definition of SVCS
• Etiology of SVCS
• Epidemiology of SVCS
• Clinical features of SVCS
• Diagnostic modalities of SVCS
• Management of SVCS

3. Introduction
• Oncologic emergency is a clinical condition
resulting from a metabolic, neurologic,
cardiovascular, hematologic or infectious
change caused by cancer or its treatment that
require immediate intervention to improve
quality or prevent loss of life

4. Introduction

5. Anatomy & patho-physiology
• The SVC is formed by the junction of the
brachiocephalic veins, which in turn are
formed by the union of the internal jugular
and subclavian veins.
• Thus, the SVC represents the major drainage
system of venous blood from the head, neck,
arms, and upper thorax.

6. Anatomy & patho-physiology
• Rt &Lt brachiocephalic veins join at the level
of the sternal angle to form SVC.
• The SVC descends on the Rt side of ascending
aorta & empties into the Rt atrium.
• It measures 6-8cm with physiologic width of
1.5-2cm
• Normal cervical venous pressure is 2-8 mm
Hg but in SVCS increased to 20-40 mm Hg

8. Anatomy & patho-physiology
• It is Surrounded with rigid structures (sternum,
trachea,pulmonary artery,Rt main bronchus&
LNs)
• its thin vascular walls &low intravascular pressure
contribute to the ease of obstruction.
• SVC obstruction can be caused by:
-External compression
-Invasion
-Intraluminal Thrombus

9. Anatomy & patho-physiology
• The azygous vein represents an important
collateral system of SVC & is formed by the
junction of Rt subcostal & Rt ascending
lumbar veins.
• Additional routes of collateral flow include:
-mammary -vertebral
-lateral thoracic -Paraspinous
-esophageal vessels.

10. Anatomy & patho-physiology
• Therefore, an obstruction of the SVC above
the orifice of the azygous vein is better
tolerated than is blockage below this level.
• If the SVC is obstructed between the azygous
vein & heart, the only route of blood return is
via the IVC.

12. Definition
• Superior vena cava syndrome (SVCS)
encompasses a range of signs and symptoms
resulting from external compression or
intrinsic obstruction of :
-SVC it self
-Associated greater veins drain to SVC
- Superior cavo-atrial junction,
• resulting in reduced blood flow & can be acute
or sub acute process.

13. Etiology
• First recorded description of SVCS was reported -
1757 when William Hunter described the entity
in a pt with a syphilitic aortic aneurysm.
• For nearly two centuries- nonmalignant
processes were the predominant etiologic factors
like:
-aortic aneurysms
-syphilitic aortitis
-chronic mediastinitis due to tuberculosis

14. Etiology
• However, in the post antibiotic era, cancer
became the leading cause of SVC obstruction.
• In recent years, nonmalignant causes of SVCS
has again risen, largely driven by the increased
number of intravascular devices and
associated thrombi

15. Etiology
• The two most frequent lung cancer histologic
types associated with SVCS are SCLC & SCC.
• NHL is the second most common cause of SVC
obstruction and the common NHL types are:
-lymphoblastic lymphoma
-diffuse large cell lymphoma
-Primary mediastinal lymphoma

16. Etiology
• Metastatic cancers account for approximately
5-10% of SVC obstructions.
• In order of frequency, the most common
primary tumor sites are breast ca, GCT &GI ca.
• Virtually any cancer capable of metastasizing
to the mediastinum can result in SVC
obstruction

17. Etiology
• Nonmalignant causes of SVC obstruction now
account for approximately one-third of cases.
• Common benign cause of SVC obstruction is
central venous catheter–induced thrombosis,
which may occur with :
-cardiac pacemakers
-dialysis

18. Etiology
• In children, SVCS is most frequently related to
iatrogenic causes resulting from
cardiovascular surgery for congenital heart
disease or ventriculoatrial shunts for
hydrocephalus.

19. Etiology

20. Epidemiology
• 90% SVCS is due to malignancy with 85%
accounted by lung ca & NHL
• Intra thoracic malignancies causes 60-85% SVCS
where by:
-50% of cases are due to NSCLC
-25% case due to SCLC
-10% due to NHL
• 2-4% of lung ca develop SVCS and is more in
SCLC(10%) than NSCLC (2%)
• 2-4% of NHL is associated with SVCS

21. Clinical features
• Duration of symptoms range from a few days
to several weeks, most pts have symptoms of
4 weeks duration
• Fullness in the head, facial swelling & dyspnea
are common presenting symptoms.
• Rarely do pts present with life-threatening
symptoms such as confusion, obtundation,
stridor

22. Clinical features
• Symptoms may be aggravated by positional
changes, particularly those associated with
lowering of the head (e.g lying down and
bending to put on shoes).

23. Clinical Features

26. Diagnosis
• SVCS was considered medical emergency,
now pts with SVC obstruction rarely
experience immediate, life-threatening
complications.
• So it is appropriate to first proceed with a
diagnostic test before any therapy because :
1st definitive Dx is necessary to plan therapy
2nd even a brief course of RT make future
histologic diagnosis difficult or impossible

27. Diagnosis
CXR
• mediastinal widening
• pleural effusion
• hilar/ mediastinal mass
• lung mass
• normal findings on CXR
does not R/O Dx of SVC
obstruction

28. Diagnosis
CT scan
• The most useful imaging study is a contrast-enhanced
chest CT.
• CT scan can define:
- level & extent of venous blockage
-identify collateral venous drainage
-identify the underlying cause obstruction.
• The presence of collateral vessels on CT is a strong
indicator of SVCS with
-Specificity -96 %
-sensitivity -92%

30. Diagnosis
Venography
• Bilateral upper extremity venography is the gold
standard for identification of SVC obstruction &
the extent of associated thrombus formation
• It is superior to CT for defining the site and
extent of SVC obstruction and for visualizing
collateral pathways
• However, it does not identify the cause of SVC
obstruction unless thrombosis is the sole
etiology.

31. Diagnosis
• Magnetic resonance venography can be
helpful in patients with contrast allergy

32. Diagnosis
• Pathologic confirmation of a thoracic malignancy can
be obtained via:
-sputum cytology
-thoracentesis
-Bronchoscope ± endobronchial fine-needle aspiration
-CT-guided needle biopsy
-mediastinal lymph node biopsy
-video-assisted thoracotomy
-Mediastinoscopy
-Thoracoscopic biopsy or thoracotomy-if all other failed

33. Diagnosis
• As lymphoma, carcinoma& GCT are DDx
adequate tissue should be obtained for IHC
• laboratory studies(β-HCG & AFP) useful in
selected cases
• Bone marrow biopsy provide diagnostic &
staging data for lymphoma
• Brain CT/MRI if SCLC

35. Treatments
• although general recommendations
supporting the consideration of RT, CT & stent
placement have been made by NCCN & ACCP
specific professional guidelines are lacking.
• Goal of therapy
-alleviating symptoms
-Treatment of the underlying malignancy

36. Treatments
• The intent and mode of treatment used for SVC
obstruction associated with malignancy depends
on :
-the type of cancer
-stage and extent of disease
-history of prior treatment
-overall prognosis
-urgency of the presenting symptoms
→It should be MDT based

37. Managements
• Conventional measures(head elevation &
oxygen supplementation )are important while
obtaining investigations.
• Glucocorticoids -can be effective in steroid-
responsive malignancies such as lymphoma or
thymoma
• Anxiolytics/morphine can also be used in the
initial supportive management.

38. Treatments
• Conditions which need immediate
intervention are these associated with very
severe to life-threatening presentations:
-significant cerebral edema causing
obtundation and confusion;
-major hemodynamic compromise leading to
hypotension, syncope, and renal insufficiency;
-significant laryngeal edema manifested by
stridor

40. RT
• RT has been considered an integral
component of SVC obstruction management
for palliative or curative intent.
• With an efficacy rate of up to 80% across
tumor types, localized radiation is effective at
reducing tumor mass with relatively rapid
responses.

41. RT-fractionation
• Pts who are rapidly progressing & distressing
SVCS & potential cured, 300-cGy fractions for
the first 2 to 3 days is recommended.
• Thereafter, the daily fraction size can be safely
reduced and the total dose adjusted to
account for the initial high-dose fractions

42. RT-dose
• Based on
-specific cancer type & its extent
-prognosis and performance status
-whether it is with concurrent chemotherapy
-goal of treatment(curative Vs palliative )
• In palliative, particularly in pts with poor
prognosis, total radiation dose 3000 cGy at
300 cGy/fraction/day.

43. RT-volume
• Radiation treatment volume is determined by:
-tumor type
-extent of disease
-baseline pulmonary reserve
-type of chemotherapy
• In curative intent, treatment volume, dose,
and fractionation should be according to the
malignancy

44. B. A. ARMSTRONG et al.
1) Goal –to determine role of RT in malignancy
associated SVCS
2)Methods - records of 4100 pts with
bronchogenic carcinoma & 952 pts with
lymphoma treated at the Mallinckrodt
Institute of Radiology
Of these 125 pts were treated for SVCS

45. B. A. ARMSTRONG et al.
3) Results
-median age 55 yrs
M:F=2.8:1
Most causes of SVCS
-bronchogenic ca79%
-lymphoma 14%
-Others-6%

46. B. A. ARMSTRONG et al.
4)Symptomatic response to therapy:
• Pts who received initial high dose RT(300-
400cGy/≠) have 83% response while pts on
conventional fraction had 78% response
• High initial dose produce rapid initial response
than conventional(70% vs 56%, p-0.09) with in
2 weeks

47. B. A. ARMSTRONG et al.

48. Chemotherapy
SCLC
• Patients with limited-stage disease, good
performance status, and no contraindication
to cisplatin are best treated with cisplatin plus
etoposide and concurrent chest irradiation.

49. Chemotherapy
• NSCLC
• Chemotherapy alone also has been used for
the treatment of SVC obstruction in patients
with NSCLC with positive results.
• but due to their innate chemotherapy
resistance, RT or combined-modality therapy
remains the preferred initial treatment

50. Chemotherapy
• NHL
• Chemotherapy can effectively palliate the
symptoms of SVC obstruction in persons with
NHL.
• Local recurrences tend to occur in pts with
large mediastinal masses & large cell
histologic types(so consolidation RT should be
considered)

51. Chemotherapy
• When chemotherapy is the initial
intervention of choice and the SVC
obstruction is unrelieved, chemotherapy
should be administered through a dorsal foot
vein!!

52. stent
• Stenting can be an effective initial treatment
in SVC obstruction of neoplastic origin.
• Morbidity and complications are minimal.
• Clinical relief of symptoms is rapid & clinical
decision for subsequent therapy not affected
• Venous access is accomplished through a
femoral, jugular or subclavian veins.

53. stent
• Stenting should be strongly considered in:
1)Patients presenting with life-threatening
symptoms such
-haemodynamic compromise
-laryngeal oedema
-cerebral oedema
2) In pts where other effective treatment
approaches are limited

56. Stent –Complications

57. Surgery
• plays a limited role in the management of pts
with SVC obstruction
• Surgery should considered
1) obstruction is refractory to RT, chemo &
stenting provided that anticipated survival
approaches 6 months
2) malignant thymoma & thymic ca as part of
multimodality therapy

58. • Objective
→To conduct a systematic review to determine
the relative effectiveness of treatments
currently employed in the management of
SVC obstruction

59. • Data collection and analysis:
→ There were three randomized and 98 non-
randomized studies of which two and 44
respectively met the inclusion criteria

60. • Main results:
1) SVCS was present at Dx in 10% of pts with
SCLC & 1.7% of pts with NSCLC
2) In one small randomized trial in SCLC, the
rate of SVCO relapse was not significantly
↓by giving RT on completion of chemo
3) addition of induction chemo to chemo-RT
not provide greater relief of SVCO

61. • Main results:
4) In SCLC
chemo± RT relieved SVCO in 77%
recurrence 17%
5) In NSCLC
Chemo ± RT relief of SVCO -60%
recurrence -19%
6) Insertion of an SVC stent
relieved SVCO in 95%
recurrence 11%
long-term patency rate of 92%.

62. • Reviewers’ conclusions:
1) Chemo and RT are effective in relieving SVCO
in a proportion of pts whilst stent insertion
provide relief in a higher proportion & more
rapidly.
2)The effectiveness of steroids and the optimal
timing of stent insertion remain uncertain.

63. Treatments

64. References