Superior Vena Cava Syndrome with Malignant Causes

The new england journal of medicine

clinical practice

Superior Vena Cava Syndrome
with Malignant Causes

Lynn D. Wilson, M.D., M.P.H., Frank C. Detterbeck, M.D.,
and Joachim Yahalom, M.D.

This Journal feature begins with a case vignette highlighting a common clinical problem.
Evidence supporting various strategies is then presented, followed by a review of formal guidelines,

when they exist. The article ends with the authors’ clinical recommendations.

A 58-year-old man presents with a 2-week history of progressive dyspnea on exertion,
neck swelling, decreased appetite, and fatigue. There is no history of syncope or dys-
phagia. He smoked cigarettes until 5 years ago. The physical examination reveals a
heart rate of 105 beats per minute, a respiratory rate of 20 breaths per minute, and
superficial vascular distention over the neck, chest, and upper abdomen. Stridor is not
present. How should his case be evaluated and managed?

The Clinical Problem

From the Departments of Therapeutic The superior vena cava syndrome, which occurs in approximately 15,000 persons in
Radiology (L.D.W.) and Surgery (F.C.D.), the United States each year, encompasses a constellation of symptoms and signs re-
Yale University School of Medicine; and sulting from obstruction of the superior vena cava. The increased venous pressure in
the Yale Comprehensive Cancer Center the upper body results in edema of the head, neck, and arms, often with cyanosis,
(L.D.W., F.C.D.) — both in New Haven, plethora, and distended subcutaneous vessels (Fig. 1A). Edema may cause functional
CT; and the Department of Radiation On- compromise of the larynx or pharynx, manifested as cough, hoarseness, dyspnea, stri-
cology, Memorial Sloan-Kettering Cancer dor, and dysphagia. Cerebral edema may lead to headache, confusion, and coma. The
Center, and Weill Medical College of Cor- decreased venous return may result in hemodynamic compromise; this complication
nell University, New York (J.Y.). Address may be a consequence of obstruction of the superior vena cava (intrinsic or due to ex-
reprint requests to Dr. Wilson at the De- trinsic compression), compression of the heart by a large mass in the chest, or both.
partment of Therapeutic Radiology, Yale Symptoms develop over a period of 2 weeks in approximately a third of patients, and
University School of Medicine, HRT 132, over longer periods in other cases.1-5
333 Cedar St., New Haven, CT 06520, or
at [email protected]. Anatomy and Physiology
The superior vena cava carries blood from the head, arms, and upper torso to the heart;
N Engl J Med 2007;356:1862-9. it carries approximately one third of the venous return to the heart. Compression of
the superior vena cava may result from the presence of a mass in the middle or ante-
Copyright © 2007 Massachusetts Medical Society. rior mediastinum (generally to the right of midline), consisting of enlarged right para-
tracheal lymph nodes, lymphoma, thymoma, an inflammatory process, or an aortic
aneurysm, for example. Thrombosis of the superior vena cava without extrinsic com-
pression can also occur (Fig. 1B).

When the superior vena cava is obstructed, blood flows through a collateral vas-
cular network to the lower body and the inferior vena cava or the azygos vein. It
generally takes several weeks for the venous collaterals to dilate sufficiently to accom-
modate the blood flow of the superior vena cava.6,7 In humans with obstruction of
the superior vena cava, the cervical venous pressure is usually increased to 20 to
40 mm Hg (normal range, 2 to 8 mm Hg).8-10 The severity of the symptoms depends
on the degree of narrowing of the superior vena cava and the speed of the onset of
the narrowing.

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clinical practice

Figure 1. The Superior Vena Cava Syndrome.

Clinical findings in a patient with the superior vena cava syndrome, including facial edema, plethora, jugular venous distention, and
prominent superficial vascularity of neck and upper chest, are shown in Panel A. The vascular anatomy of the upper chest, including
the heart, superior vena cava, inferior vena cava, and subclavian vessels, is shown in Panel B. The tumor is shown compressing the
superior vena cava.

Edema in the upper body as a result of obstruc- Etiologic Factors
tion of the superior vena cava is visually striking Infectious causes (especially syphilitic aortic aneu-
but often of little consequence. However, cerebral rysm and tuberculosis) accounted for the majority
edema, although rare, can be serious or fatal. The of cases of obstruction of the superior vena cava
upper respiratory tract may become narrowed by until about 50 years ago. These causes became rare,
nasal and laryngeal edema. Serious effects of ob- and malignant conditions accounted for more than
struction of the superior vena cava are rare; among 90% of cases approximately 25 years ago.1,12,13
1986 patients with obstruction of the superior vena Currently, obstruction of the superior vena cava
cava, only one death was documented.11 In case caused by thrombosis or nonmalignant conditions
reports of neurologic or laryngeal compromise, it accounts for approximately 35% of cases, reflect-
is unclear whether other contributing factors such ing the increased use of intravascular devices such
as brain metastases or tracheal compression were as catheters and pacemakers.14 The most common
present.10,11 malignant causes are non–small-cell lung cancer

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(approximately 50% of patients), small-cell lung is needed to evaluate the superior vena cava). Com-
cancer (approximately 25% of patients), lympho- plications, including excessive bleeding from the
ma, and metastatic lesions (each approximately venipuncture sites and reactions to contrast me-
10% of patients); the clinical features that may dium, are uncommon.11,14,19 Venography is gen-
suggest these diagnoses are summarized in Ta- erally warranted only when an intervention (place-
ble 1.1,4,5,13,15-17 ment of a stent or surgery) is planned.20 Magnetic
resonance imaging may be useful for patients who
Recognition of a nonmalignant cause of the cannot tolerate the contrast medium. Positron-
superior vena cava syndrome is typically straight- emission tomography (PET) is sometimes useful,
forward, particularly when the syndrome is associ- because it may influence the design of the radio-
ated with the use of an implanted intravascular therapy field (Fig. 2).21
device. An aortic aneurysm is easily recognized on
computed tomography (CT). The diagnosis of fi- The clinical history combined with CT imag-
brosing mediastinitis, although a rare cause, re- ing will generally differentiate between vena caval
quires a biopsy. thrombosis and extrinsic compression. A tissue
diagnosis is necessary to confirm the presence of
Strategies and Evidence malignant conditions. Clinical assessment is war-
ranted to determine whether a peripheral biopsy
Clinical Evaluation site (e.g., a palpable supraclavicular lymph node)
Clinical diagnosis of obstruction of the superior might be accessible before proceeding to an in-
vena cava is made on the basis of signs and symp- vasive procedure such as mediastinoscopy for tis-
toms (Table 2).1,4,5,13,15,18 The history taking should sue diagnosis. Cytologic examination of the spu-
attend to the duration of symptoms, previous di- tum may result in diagnosis in patients who have
agnoses of malignant conditions, or previous in- endobronchial cancer. Pleural effusion is common
travascular procedures. In most cases, symptoms (affecting about two thirds of patients with the
are progressive over several weeks, and in some superior vena cava syndrome); thoracentesis and
cases they may improve as collateral circulation cytologic analysis should be strongly considered
develops. The severity of the symptoms is impor- because they are simple to perform and expedient,
tant in determining the urgency of intervention. although they yield a diagnosis in only about 50%
of such patients.15 Bronchoscopy has a diagnostic
Imaging yield of 50 to 70% and transthoracic needle-aspi-
The most useful imaging study is CT of the chest ration biopsy has a yield of approximately 75%,
after the administration of contrast material (which whereas mediastinoscopy or mediastinotomy has

Table 1. Malignant Causes of the Superior Vena Cava Syndrome.*

Tumor Type Proportion Suggestive Clinical Features
% (range)
Non–small-cell lung cancer 50 (43–59) History of smoking; often age >50 yr
Small-cell lung cancer 22 (7–39) History of smoking; often age >50 yr
Lymphoma 12 (1–25) Adenopathy outside the chest; often age <65 yr
Metastatic cancer† 9 (1–15) History of malignant condition (usually, breast cancer)
Germ-cell cancer 3 (0–6) Usually, male sex and age <40 yr; elevated levels of β human chorionic

Thymoma 2 (0–4) gonadotropin or alpha-fetoprotein are common
Characteristic radiographic appearance on the basis of the location of
Mesothelioma 1 (0–1)
Other cancers 1 (0–2) the thymus; frequently associated with the parathymic syndromes
(e.g., myasthenia gravis and pure red-cell aplasia)
History of asbestos exposure

* Data are from Armstrong et al.,1 Yellin et al.,4 Schraufnagel et al.,5 Chen et al.,13 Rice et al.,15 Nicholson et al.,16 and
Detterbeck and Parsons.17

† Approximately two thirds of the patients who have metastatic cancers have breast cancer.

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clinical practice

a diagnostic yield of more than 90%.9,22 Particu- Table 2. Symptoms and Signs Associated with the Supe-
larly in the case of lymphoma, adequate tissue is rior Vena Cava Syndrome.*
needed to characterize the nodal architecture and
cell type, and also for immunohistochemistry in Sign or Symptom Frequency Range
order to confirm the subtype.
Facial edema percent
Although some studies suggest a higher rate of Arm edema
complications from mediastinal procedures among Distended neck veins 82 60–100
patients who have the superior vena cava syndrome Distended chest veins
than among those who do not, other studies re- Facial plethora 46 14–75
port low rates of complications even in the pres- Visual symptoms
ence of the superior vena cava syndrome.9,11,14,22,23 Dyspnea 63 27–86
A review involving 319 patients with the superior Cough
vena cava syndrome found major hemorrhage (not Hoarseness 53 38–67
specifically defined) in 3% of patients undergoing Stridor
mediastinoscopy or mediastinotomy. Bronchosco- Syncope 20 13–23
py (both fiberoptic and rigid) was associated with Headaches
low risk (risk of bleeding, 0.5%; and risk of re- Dizziness 2 0–3
spiratory distress, 0.5%).11,22 Confusion
Obtundation 54 23–74
Management
Management of the superior vena cava syndrome 54 38–70
associated with malignant conditions involves both
treatment of the cancer and relief of the symp- 17 15–20
toms of obstruction. Most data regarding man-
agement of the superior vena cava syndrome are 4 0–5
from case series; randomized trials are scarce. The
median life expectancy among patients with ob- 10 8–13
struction of the superior vena cava is approximate-
ly 6 months; but estimates vary widely according 9 6–11
to the underlying malignant conditions.4,5,24-26 Sur-
vival among patients presenting with obstruction 6 2–10
of the superior vena cava associated with malig- 4 0–5
nant conditions does not appear to differ signifi- 2 0–3
cantly from survival among patients with the same
tumor type and disease stage without obstruction * Data are from Armstrong et al.,1 Yellin et al.,4
of the superior vena cava. In some patients, treat- Schraufnagel et al.,5 Chen et al.,13 Rice et al.,15 and
ment of the superior vena cava syndrome and Urruticoechea et al.18
their malignant conditions results in the cure of
both.3,11,27-29 are somewhat less than in patients with lympho-
ma, small-cell lung cancer, or germ-cell tumors.
Management is guided by the severity of the
symptoms and the underlying malignant condi- Supportive Care and Medical Management
tions as well as by the anticipated response to An obvious therapeutic maneuver is to elevate the
treatment. For example, in patients with lympho- patient’s head to decrease the hydrostatic pressure
ma, small-cell lung cancer, or germ-cell tumors, and thereby the edema. There are no data docu-
the clinical response to systemic chemotherapy menting the effectiveness of this maneuver, but
alone typically is rapid. In the majority of patients it is simple and without risk. Glucocorticoid ther-
with non–small-cell lung cancer, relief of symp- apy (dexamethasone, 4 mg every 6 hours) is com-
toms of obstruction of the superior vena cava re- monly prescribed, although its effects have not
sults from treatment of the cancer (chemotherapy been formally well studied, and there are only case
for patients with stage IV disease, and chemo- reports to suggest the benefit. Glucocorticoids re-
therapy with radiotherapy for those with stage III duce the tumor burden in lymphoma and thymo-
disease), but the degree and rapidity of response ma and are therefore more likely to reduce the ob-
struction in patients with lymphoma or thymoma
than in those with other types of tumor.3,30 Loop
diuretics are also commonly used, but it is unclear
whether venous pressure distal to the obstruction
is affected by small changes in right atrial pres-
sure. In an observational study involving 107 pa-
tients with the superior vena cava syndrome due
to various causes, the rate of clinical improvement
(84% overall) was similar among patients receiv-
ing glucocorticoids, diuretics, or neither therapy.5

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AB

C

Figure 2. Chest Radiograph and PET–CT Scans of a Patient with the Superior Vena Cava Syndrome.

(cPthCaaenTncesweul riAp.thePsorahiuonotrewclvsoBennastacrhahcosaewtvs)sataar—fatPedEraionTtgh–iredCaeppTnhastticoifeaIERCifCncMEAnMaGatSat(EiphFliCoaaTntdiFATewtuIIUnhGTinTLttaUhdEHtwRoeOiEisrutRghtcohctnhoaeenllFe5tsWirnguawiglsp2seitaoen)e-Lnrgckioinoswferotihvtfheesonysuasa4ttmc-eCcamoevnaipcRtEsarcTaytRAhineseKvetdnEimserteo.ndomPhthaae231nnesnrdtcdrecaael pumCys.eesTnhdhtob.ewysasrmaroPawEll-Ticd–eeClnlTtluifsnicegasn
Enon ARTIST: mleahy H/T SIZE
H/T
FILL Combo 33p9
In patients with obstruction of the supAUeTrHioOrR, PLpEaAStiEeNnOtTsE:and partial relief in 23% of the patients.
vena cava resulting from intravascFuigluarer rhteahmsrbooeemPvnlaebrlaesuodesrfcahwencIoknanncadlayruteyf1tpuo4lelpy%h.saysobsfeteutnhdreieespset,a. tcioemntpsleatnedpaptaerntciaylwpaastefnocuyndwains
associated with an indwelling catheter,
the catheter should be consideredJO.BR: em35o6v1a8l of the foundISiSnUE1:0%5-o03f-0t7he patients, despite reported re-
catheter is performed in conjunction with antico- lief of symptoms in 85% of the patients.11 These
agulation therapy (see Areas of Uncertainty). findings suggest that the development of collat-
eral circulation may contribute to improvement of
symptoms and underscore the uncertain value of
Radiotherapy

Radiotherapy is often used to treat symptomatic urgent initiation of radiotherapy before chemo-
patients with malignant obstruction of the supe- therapy is initiated in those patients with chemo-
rior vena cava; its use requires a tissue diagnosis. therapy-sensitive tumors.
The majority of the tumor types causing the su- If radiation is given as the initial treatment, the
perior vena cava syndrome are sensitive to radio- fields should encompass gross disease and the ad-
therapy. A systematic review found complete relief jacent nodal regions, taking into account the
of the symptoms of obstruction of the superior volume of pulmonary and cardiac tissue to mini-
vena cava in 78% of patients with small-cell lung mize complications. CT-based simulation (for de-
cancer and 63% of those with non–small-cell lung signing radiotherapy fields) and irradiation in
cancer at 2 weeks. Improvement is often apparent daily fractions of 1.8 to 2.0 Gy are recommended
within 72 hours.1,3-5,11,16,31-35 for the majority of lymphomas. The total dose of
However, objective measures of the change in radiation should be based on a multidisciplinary
vena caval obstruction have not paralleled mea- plan that incorporates systemic chemotherapy, ei-
sures of symptomatic improvement based on pa- ther from the beginning of treatment or after a
tients’ reports. In a case series of patients receiving brief initial course of radiotherapy. A similar ini-
radiotherapy (in most patients as the sole therapy), tial course of radiotherapy is often used to treat
complete relief of vena caval obstruction as mea- small-cell and non–small-cell lung cancer, with
sured on serial venograms was noted in 31% of the higher daily fractions of 2.0 to 3.0 Gy. The size and

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configuration of the field may be altered after the rable benefit from angioplasty alone.38,39 Place-
administration of several fractions, as symptoms ment of an intravascular stent results in more
begin to subside and the staging and plans for prompt relief of symptoms than does radiation
subsequent management are organized. When the or chemotherapy (although the usually rapid re-
radiotherapy is palliative, the course of treatment sponse to radiation or chemotherapy in patients
is typically over a period of 1 to 3 weeks, with with tumors sensitive to these therapies means
daily fractionation. that stent placement is not typically warranted).
After stent placement, cyanosis is usually relieved
Systemic Chemotherapy within hours, and edema resolves within 48 to 72
Complete relief of symptoms of vena caval obstruc- hours in most series (response rate, 75 to 100%).
tion is achieved with chemotherapy in approxi- However, in one prospective series, symptoms re-
mately 80% of patients with non-Hodgkin’s lym- solved completely in only 17% of cases. This out-
phoma or small-cell lung cancer and in 40% of come may have been due to the fact that not all the
those with non–small-cell lung cancer.5,27,30,32 associated symptoms actually resulted from caval
A review of 2 randomized studies and 44 observa- obstruction.26
tional studies concluded that among patients with
lung cancer, there was no clinically significant dif- Complications of stent placement have been
ference in the rate of relief from the superior vena reported in 3 to 7% of patients with the superior
cava syndrome whether chemotherapy, radiothera- vena cava syndrome, including infection, pulmo-
py, or chemotherapy with radiotherapy was used.30 nary embolus, stent migration, hematoma at the
In the two randomized trials, there were no sig- insertion site, bleeding, and, very rarely, perfora-
nificant differences in the rates of relief of symp- tion. Late complications include bleeding (1 to
toms, relapse, or survival with initial chemotherapy 14% of patients) and death (1 to 2% of patients)
alone, as compared with either sequential chemo- due to anticoagulation, a treatment often recom-
therapy with radiotherapy among patients with mended after stent placement (see Areas of Un-
small-cell lung cancer or immediate (concurrent) certainty).16,18,24,25,38-40
chemotherapy and radiotherapy among those with
non–small-cell lung cancer.32,33 In observational Surgery
studies, manifestations of the superior vena cava Surgical bypass grafting is infrequently used to
syndrome caused by other chemotherapy-sensitive treat the superior vena cava syndrome. The sur-
malignant conditions such as germ-cell tumors gery, which involves a subcutaneous jugular–fem-
have also been reported to improve rapidly with oral graft, for example,41 can be performed with
systemic therapy alone. relatively few complications. The more common
approach is sternotomy or thoracotomy with ex-
Placement of an Intravascular Stent tensive resection and reconstruction of the supe-
Percutaneous placement of an intravascular stent rior vena cava; case series indicate an operative
to bypass the obstruction of the superior vena cava mortality of approximately 5% and patency rates
is another possible intervention. Because the stent of 80 to 90%.28,42-46 Thymomas are relatively re-
can be placed before a tissue diagnosis is available, sistant to chemotherapy and radiation, as compared
it is a useful procedure for patients with severe with lymphomas, and surgery is therefore often
symptoms such as respiratory distress that require appropriate when the superior vena cava syndrome
urgent intervention. Stent placement should also is caused by thymoma. A curative approach gener-
be strongly considered for patients with mesothe- ally involves preoperative chemotherapy, surgical
lioma, which tends not to respond well to chemo- resection and reconstruction, and postoperative ra-
therapy or radiation, and may also be particularly diotherapy.17
useful when obstruction of the superior vena cava
is caused by a thrombus associated with an in- Durability of Response
dwelling catheter.36,37 The durability of various treatment strategies ap-
pears to be relatively similar and may primarily re-
Angioplasty for the narrowing of the superior flect the underlying malignant conditions. A sys-
vena cava is generally performed only in prepara- tematic review found that symptomatic recurrence
tion for stent placement because of a lack of du- of the superior vena cava syndrome occurred in

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nearly 20% of patients with either small-cell or Guidelines from
non–small-cell lung cancer after chemotherapy, ra- Professional Societies
diotherapy, or both.32 The rate of relapse after stent There are no formal professional guidelines ad-
placement was 11%, although 78% of these relaps- dressing the management of obstruction of the
es were successfully managed by repeat intravas- superior vena cava. A general recommendation sup-
cular interventions. Relapse rates ranging from porting consideration of radiotherapy, stent place-
9 to 20% after stent placement have been report- ment for symptomatic obstruction of the superior
ed by others.10,16,18,24,38 Rates of occlusion of the vena cava due to lung cancer, or both has been
superior vena cava of 10% have been reported af- made by both the American College of Chest Phy-
ter surgical reconstruction.42 sicians48 and the National Comprehensive Cancer
Network.49
Areas of Uncertainty

Standardized criteria to grade the severity of symp- Conclusions and
toms in the superior vena cava syndrome are lack- Recommendations
ing. The benefit of either short-term or long-term
anticoagulation therapy for this syndrome is un- The superior vena cava syndrome is often clinically
clear, although thrombolytic agents have been used striking but rarely requires emergency intervention.
effectively in patients with vena caval thrombosis. The majority of cases are due to malignant condi-
Most experts recommend anticoagulation after tions; a tissue biopsy is warranted to guide diag-
thrombolysis (to prevent disease progression and nosis and therapy and is generally safe when per-
recurrence) and aspirin after stent placement in the formed by experienced practitioners. Treatment
absence of thrombosis, but data to inform these planning should be multidisciplinary. In patients
recommendations are limited.16,24,39 with life-threatening symptoms or signs of ob-
struction of the superior vena cava, the placement
Whether the presence of brain metastasis of an intravascular stent can provide rapid relief.
should affect management of the superior vena In other patients, such as the patient described in
cava syndrome is unclear. Patients with brain me- the vignette, information on the tumor type and
tastasis may undergo stent placement because of stage of the cancer should be used to guide the
the potential of the superior vena cava syndrome therapy (i.e., chemotherapy or radiotherapy or both
to exacerbate cerebral edema, but at least tempo- or, in occasional cases, surgery alone or in combi-
rary anticoagulation is needed and associated cere- nation with other therapies); these types of therapy
bral hemorrhage has been reported. The care of can relieve the symptoms of obstruction of the su-
patients with both the superior vena cava syn- perior vena cava in the vast majority of patients. The
drome and significant airway obstruction is also presence of the superior vena cava syndrome does
unclear. Some authors suggest resection of the not reduce the likelihood of cure of the underly-
tumor mass (complete or subtotal resection) in ing malignant condition and should not compro-
such patients to provide immediate relief of both mise the choice of appropriate therapy.
clinical problems.45-47 The optimal management
of recurrent obstruction of the superior vena cava No potential conflict of interest relevant to this article was
is also controversial. Placement of a stent is often reported.
considered because of the limited benefit or the
risk of excessive toxic effects from repeat chemo- We thank Marilyn L. Powers for assistance in the preparation
therapy or radiation, but data to guide decision of the manuscript, Roy H. Decker for assistance in the prepara-
making are limited. tion of the figures, and Waldo Greenspan for his encouragement
and support.

References 3. Ostler PJ, Clarke DP, Watkinson AF, 5. Schraufnagel DE, Hill R, Leech JA,
1. Armstrong BA, Perez CA, Simpson JR, Gaze MN. Superior vena cava obstruction: Pare JA. Superior vena caval obstruction:
Hederman MA. Role of irradiation in the a modern management strategy. Clin On- is it a medical emergency? Am J Med 1981;
management of superior vena cava syn- col (R Coll Radiol) 1997;9:83-9. 70:1169-74.
drome. Int J Radiat Oncol Biol Phys 1987; 4. Yellin A, Rosen A, Reichert N, Lieber- 6. Kim HJ, Kim HS, Chung SH. CT diag-
13:531-9. man Y. Superior vena cava syndrome: the nosis of superior vena cava syndrome: im-
2. Abner A. Approach to the patient who myth — the facts. Am Rev Respir Dis portance of collateral vessels. AJR Am J
presents with superior vena cava obstruc- 1990;141:1114-8. Roentgenol 1993;161:539-42.
tion. Chest 1993;103:Suppl 4:394S-397S.

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clinical practice

7. Trigaux JP, Van Beers B. Thoracic col- mediastinoscopy in superior vena cava cancer. Int J Radiat Oncol Biol Phys 1997;
lateral venous channels: normal and patho- obstruction. Chest 2005;128:1551-6. 38:513-20.
logic CT findings. J Comput Assist Tomogr 23. Porte H, Metois D, Finzi L, et al. Supe- 36. Fine DG, Shepherd RF, Welch TJ.
1990;14:769-73. rior vena cava syndrome of malignant ori- Thrombolytic therapy for superior vena
8. Gonzales-Fajardo JA, Garcia-Yuste M, gin: which surgical procedure for which cava syndrome. Lancet 1989;1:1200-1.
Florez S, Ramos G, Alvarez T, Coca JM. diagnosis? Eur J Cardiothorac Surg 2000; 37. Kee ST, Kinoshita L, Razavi MK, Ny-
Hemodynamic and cerebral repercussions 17:384-8. man UR, Semba CP, Dake MD. Superior
arising from surgical interruption of the 24. Marcy PY, Magne N, Bentolila F, vena cava syndrome: treatment with cath-
superior vena cava: experimental model. Drouillard J, Bruneton JN, Descamps B. eter-directed thrombolysis and endovascu-
J Thorac Cardiovasc Surg 1994;107:1044-9. Superior vena cava obstruction: is stent- lar stent placement. Radiology 1998;206:
9. Mineo TC, Ambrogi V, Nofroni I, Pis- ing necessary? Support Care Cancer 2001; 187-93.
tolese C. Mediastinoscopy in superior vena 9:103-7. 38. Courtheoux P, Alkofer B, Al Refai M,
cava obstruction: analysis of 80 consecu- 25. Greillier L, Barlesi F, Doddoli C, et al. Gervais R, Le Rochais JP, Icard P. Stent
tive patients. Ann Thorac Surg 1999;68: Vascular stenting for palliation of superi- placement in superior vena cava syndrome.
223-6. or vena cava obstruction in non-small-cell Ann Thorac Surg 2003;75:158-61.
10. Kishi K, Sonomura T, Mitsuzane K, et lung cancer patients: a future ‘standard’ 39. Uberoi R. Quality assurance guide-
al. Self-expandable metallic stent therapy procedure? Respiration 2004;71:178-83. lines for superior vena cava stenting in
for superior vena cava syndrome: clinical 26. Tanigawa N, Sawada S, Mishima K, et malignant disease. Cardiovasc Intervent
observations. Radiology 1993;189:531-5. al. Clinical outcome of stenting in supe- Radiol 2006;29:319-22.
11. Ahmann FR. A reassessment of the rior vena cava syndrome associated with 40. Smayra T, Otal P, Chabbert V, et al.
clinical implications of the superior vena malignant tumors: comparison with con- Long-term results of endovascular stent
caval syndrome. J Clin Oncol 1984;2:961- ventional treatment. Acta Radiol 1998;39: placement in the superior vena caval ve-
9. 669-74. nous system. Cardiovasc Intervent Radiol
12. Parish JM, Marschke RF Jr, Dines DE, 27. Sculier JP, Evans WK, Feld R, et al. 2001;24:388-94.
Lee RE. Etiologic considerations in supe- Superior vena caval obstruction syndrome 41. Dhaliwal RS, Das D, Luthra S, Singh J,
rior vena cava syndrome. Mayo Clin Proc in small cell lung cancer. Cancer 1986;57: Mehta S, Singh H. Management of supe-
1981;56:407-13. 847-51. rior vena cava syndrome by internal jugu-
13. Chen JC, Bongard F, Klein SR. A com- 28. Magnan PE, Thomas P, Guidicelli R, lar to femoral vein bypass. Ann Thorac
temporary perspective on superior vena Fuentes P, Branchereau A. Surgical recon- Surg 2006;82:310-2.
cava syndrome. Am J Surg 1990;160:207- struction of the superior vena cava. Car- 42. Bacha EA, Chapelier AR, Macchiarini
11. diovasc Surg 1994;2:598-604. P, Fadel E, Dartevelle PG. Surgery for in-
14. Rice TW, Rodriguez RM, Light RW. 29. Maddox AM, Valdivieso M, Lukeman vasive mediastinal tumors. Ann Thorac
The superior vena cava syndrome: clinical J, et al. Superior vena cava obstruction in Surg 1998;66:234-9.
characteristics and evolving etiology. Med- small cell bronchogenic carcinoma: clini- 43. Chen KN, Xu SF, Gu ZD, et al. Surgical
icine (Baltimore) 2006;85:37-42. cal parameters and survival. Cancer 1983; treatment of complex malignant anterior
15. Rice TW, Rodriguez RM, Barnette R, 52:2165-72. mediastinal tumors invading the superior
Light RW. Prevalence and characteristics 30. Rowell NP, Gleeson FV. Steroids, ra- vena cava. World J Surg 2006;30:162-70.
of pleural effusions in superior vena cava diotherapy, chemotherapy and stents for 44. Kalra M, Gloviczki P, Andrews JC, et
syndrome. Respirology 2006;11:299-305. superior vena caval obstruction in carci- al. Open surgical and endovascular treat-
16. Nicholson AA, Ettles DF, Arnold A, noma of the bronchus: a systematic re- ment of superior vena cava syndrome
Greenstone M, Dyet JF. Treatment of ma- view. Clin Oncol (R Coll Radiol) 2002;14: caused by nonmalignant disease. J Vasc
lignant superior vena cava obstruction: 338-51. Surg 2003;38:215-23.
metal stents or radiation therapy. J Vasc 31. Anderson PR, Coia LR. Fractionation 45. Inoue M, Minami M, Shiono H, et al.
Interv Radiol 1997;8:781-8. and outcomes with palliative radiation ther- Efficient clinical application of percuta-
17. Detterbeck FC, Parsons AM. Thymic apy. Semin Radiat Oncol 2000;10:191-9. neous cardiopulmonary support for peri-
tumors. Ann Thorac Surg 2004;77:1860-9. 32. Spiro SG, Shah S, Harper PG, Tobias operative management of a huge anterior
18. Urruticoechea A, Mesia R, Domin- JS, Geddes DM, Souhami RL. Treatment of mediastinal tumor. J Thorac Cardiovasc
guez J, et al. Treatment of malignant su- obstruction of the superior vena cava by Surg 2006;131:755-6.
perior vena cava syndrome by endovascu- combination chemotherapy with and with- 46. Detterbeck FC, Jones DR, Kernstine
lar stent insertion: experience on 52 patients out irradiation in small-cell carcinoma of KH, Naunheim KS. Lung cancer: special
with lung cancer. Lung Cancer 2004;43: the bronchus. Thorax 1983;38:501-5. treatment issues. Chest 2003;123:Suppl 1:
209-14. 33. Pereira JR, Martins SJ, Ikari FK, Ni- 244S-258S.
19. Schwartz EE, Goodman LR, Haskin kaedo SM, Gampel O. Neoadjuvant che- 47. Takeda S, Miyoshi S, Omori K, Oku-
ME. Role of CT scanning in the superior motherapy vs radiotherapy alone for supe- mura M, Matsuda H. Surgical rescue for
vena cava syndrome. Am J Clin Oncol 1986; rior vena cava syndrome (SVCS) due to life-threatening hypoxemia caused by a
9:71-8. non-small cell lung cancer (NSCLC): pre- mediastinal tumor. Ann Thorac Surg 1999;
20. Stanford W, Jolles H, Ell S, Chiu LC. liminary results of randomized phase II 68:2324-6.
Superior vena cava obstruction: a veno- trial. Eur J Cancer 1999;35:Suppl 4:260. 48. Kvale PA, Simoff M, Prakash UBS.
graphic classification. AJR Am J Roent- abstract. Lung cancer: palliative care. Chest 2003;
genol 1987;148:259-62. 34. Wurschmidt F, Bunemann H, Heil- 123:Suppl 1:284S-311S.
21. Khimji T, Zeiss J. MRI versus CT and mann HP. Small cell lung cancer with and 49. NCCN Clinical Practice Guidelines in
US in the evaluation of a patient present- without superior vena cava syndrome: Oncology: non-small cell lung cancer. V.
ing with superior vena cava syndrome: a multivariate analysis of prognostic fac- I.2007. National Comprehensive Cancer
case report. Clin Imaging 1992;16:269- tors in 408 cases. Int J Radiat Oncol Biol Network, 2007. (Accessed April 9, 2007,
71. Phys 1995;33:77-82. at http://www.nccn.org/professionals/
22. Dosios T, Theakos N, Chatziantoniou 35. Chan RH, Dar AR, Yu E, et al. Superi- physician_gls/PDF/nscl.pdf ).
C. Cervical mediastinoscopy and anterior or vena cava obstruction in small-cell lung
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