Download Oncological emergencies: Superior vena cava syndrome

PRACE POGLĄDOWE
Justyna Bochenek-Cibor
Mirosława Püsküllüoğlu
Aneta Zygulska
Oncological emergencies: Superior vena cava
syndrome
Stany nagłe w onkologii: Zespół żyły głównej górnej
Klinika Onkologii
Szpitala Uniwersyteckiego w Krakowie
Kierownik:
Doc. dr hab. Krzysztof Krzemieniecki
Dodatkowe słowa kluczowe:
zespół żyły głównej górnej
stan nagły w onkologii
radioterapia
chemioterapia
chirurgia
stentowanie
Additional key words:
superior vena cava syndrome
oncologic emergency
radiation therapy
chemotherapy
surgery
stenting
Adres do korespondencji:
ul. Śniadeckich 10, IIp., 31-531 Kraków
tel. 124248912, fax 124248910
e-mail: [email protected]
Przegląd Lekarski 2014 / 71 / 12
Superior vena cava obstruction
leads to the venous hypertension of
the head, neck, upper extremities and
upper part of the truncus. Its clinical
manifestation, known as the superior
vena cava syndrome, is a complication
of malignancy, mainly of lung cancer
and lymphoma. As it usually affects
patients with advanced disease, the
prognosis is poor. This article stresses
the importance of early detection and
adequate management of this oncological emergency. The diagnostic
tools as well as treatment possibilities
are discussed.
Upośledzenie drożności żyły głównej górnej doprowadza do nadciśnienia w obrębie głowy, szyi, kończyn
górnych oraz górnej części tułowia.
Rozwija się zespół objawów znany jako
zespół żyły głównej górnej. Należy on
do powikłań choroby nowotworowej,
szczególnie raka płuca i chłoniaków.
Dotyka zwykle pacjentów w zaawansowanym stadium choroby, dlatego
rokowanie jest zazwyczaj złe. Niniejszy
artykuł został przygotowany w celu
podkreślenia wagi wczesnej diagnozy
i odpowiedniego leczenia tego onkologicznego stanu nagłego. Przedstawione zostały narzędzia diagnostyczne
oraz możliwości leczenia.
What is the Superior Vena Cava
Syndrome?
The term superior vena cava syndrome
(SVCS), introduced in 1865 by Trousseau,
encompasses a range of signs and symptoms arising from obstruction or direct invasion of the superior vena cava (SVC) [1,2].
The SVC carries blood from the head, arms
and upper torso to the heart. The blocked
venous drainage leads to pleural effusions
and edema of the face, neck, trachea and
arms. Severe obstruction may cause cerebral edema with focal neurologic signs
and unconsciousness as well as impaired
cardiac filling [3].
Among other malignancies causing the
SVC obstruction metastases from different
locations, germ cell cancers, thymoma and
mesothelioma should be mentioned. Due
to their anatomic proximity to the SVC, rightsided lesions are related to a higher risk than
left-sided tumors [6].
Etiology
Over 90% of SVCS cases are caused
by malignancies and are therefore cancerrelated. Other causes include mediastinal
fibrosis, vascular diseases (for example
aortic aneurysm), infections (such as histoplasmosis, tuberculosis, syphilis, and
actinomycosis) and benign mediastinal
tumors. Among these the most frequent are:
teratoma, cystic hygroma, thymoma, and
dermoid cyst. SVCS may also be a result of
thrombosis from central venous catheters or
cardiac pacemakers [4]. Over the last two
decades, the benign causes have been reported to stand for more cases of SVCS than
previously when the intravascular devices
were uncommon [5].
Among the patients with malignancies,
SVCS most often affects males aged 5070 with primary or metastatic tumors of the
mediastinum [6]. Advanced small-cell lung
carcinoma and – less frequently – advanced
non-small-cell lung carcinoma stand for 75%
of all cases of SVCS. Next most common
cause of the SVCS lymphoma (usually
non-Hodgkin) with perihilar lymphadenopathy, that accounts for around 12% cases.
Patophysiology
The SVC is located inside the rigid
walls of the thoracic cavity, surrounded by
inflexible structures such as the sternum,
ribs, vertebral bodies, and aorta with its
high intravascular pressure. Figure 1. shows
the anatomic drainage of the SVC. The
SVC is usually situated close to the right
main bronchus. As a thin-walled structure
with low intravascular pressure in a tight,
non-expanding compartment, SVC can get
easily compressed by extraluminal tumor
or enlarged lymph nodes. SVCS may also
be a result of intraluminal obstruction by
thrombosis or tumor. The symptoms can
occur rapidly or gradually and there can be
a complete or partial obstruction. In some
cases, the collateral venous drainage may
develop. The blockage develops gradually
and a minimal blood flow remains in most
SVCS cases [1].
The development of SVCS depends on
several factors, such as the hypercoagulable
state in patients with cancer, the growth rate,
extent and localization of the tumor, flow in
the azygos vein and the ability to form collateral circulation [1]. Blood from the upper
thoracic venous system may be redirected
to the azygos vein, internal mammary veins,
thoracic venous system, and vertebral veins
[6]. The subcutaneous veins may also play a
role in the formation of collateral circulation
[7]. The extent of SVCS depends on the level
of obstruction – if the drainage is impaired
above the azygos vein, the pressure in the
697
SVC is lower and the symptoms are less
significantly demonstrated.
Symptoms
The severity of symptoms of SVCS depend on the speed of the SVC obstruction
development. With a gradual progression
the clinical manifestation is mild [8]. Patients
complain of swelling of the face, neck and
arms, especially in the morning after sleeping in the supine position or upon position
changes such as bending forward [1]. Distended neck and chest veins and facial plethora are often striking, but generally of little
consequence. Figure 2 shows the dilatation
of the vessels of the upper chest due to the
development of the collateral drainage. If
the development of the disease is acute,
patients suffer from neurological and respiratory distress. Dyspnea and cough are the
most frequently reported symptoms among
patients with SVCS. They also complain of
hoarseness and stridor. Severe edema of
the larynx may be life-threatening as it makes intubation impossible. Neurologic symptoms include headache, syncopes, dizziness
and confusion. Cerebral edema may also
be observed and lead to cerebral ischemia,
herniation or even death. Typical symptoms
of SVCS are listed in Table I [9,7].
Diagnosis
Patient evaluation should begin with a
history and a thorough physical examination,
which play a fundamental role in establishing
a tentative diagnosis of SVCS. In most cases, symptoms of the SVCS progress over
several weeks and get better over time. Further investigation allow the identification of
SVCS causes. Chest computed tomography
scans (CT) with contrast enhancement and
chest magnetic resonance imaging (MRI)
are the preferred radiological tools used to
Figure 1
Venous drainage of the SVC.
Drenaż żylny SVC.
SVC – superior vena cava
RBC – right brachiocephalic vein
LBC – left brachiocephalic vein
AV – azygos vein
HAV – hemiazygos vein
AHAV – accessory hemiazygos vein
Yellow – normal variants of venous connections
698
confirm SVC obstruction [9,11]. Chest X-rays
may sometimes also be useful showing
a lung or mediastinal mass and superior
mediastinal widening [10].
The histological diagnosis is crucial.
Invasive procedures are performed to collect
tumor samples. These procedures are usually connected with a high risk of bleeding
because of increased venous pressure in the
head and neck [12]. The procedure should
be adjusted to the type of tumor (e.g. mediastinoscopy is well established in the diagnosis of lung cancer) [13]. Bronchoscopy with
brushings and biopsy of lymph nodes is also
valuable [1]. In the case of pleural effusion,
thoracocentesis should be considered [12].
If the underlying malignancy tends to involve
bone marrow, like in Hodgkin’s lymphoma or
non-small-cell lung carcinoma, bone marrow
biopsy may prove useful [7].
Management
The way of treatment depends on the
reason for SVC obstruction. In the case of
a malignancy-related syndrome, management includes treatment of the neoplasm
itself and relievement of the symptoms of
SVCS. It should also be adjusted to the
patient’s performance status, the severity of
symptoms, the presence of thrombosis and
comorbidities. Radiotherapy, chemotherapy
and surgery are the main therapeutic options
[6]. Radical treatment may be attempted
especially in such cases as the small-cell
lung carcinoma, Hodgkin’s lymphoma and
germ cell tumors [1].
In life threatening cases emergency
treatment should be started as soon as
possible [3].
Radiotherapy is the most common
treatment modality for the patients suffering from lung cancer [14]. The irradiated
field should include the tumor mass with
adequate margins and the mediastinal and
hilar lymph nodes [15]. Daily radiation dose
is 400 cGy administered for 5 consecutive
days [16]. Radiotherapy is associated with
significant clinical improvement, noticeable
much sooner than the objective signs in radiological examinations [3]. Symptoms could
be reduced after only several days [6]. The
side effects depend on the irradiated field,
the total dose of the radiation therapy and
the patient’s general condition [17]. Fatigue,
dyspnoea, cough, pneumonitis, pharyngitis,
esophagitis, skin changes are the most
common side effects [18].
The histological diagnosis of malignancy
Table I
Typical symptoms of the SVCS.
Typowe objawy zespołu żyły głównej górnej.
Symptoms of SVCS
Cardiologic
Neurologic
Pulmonologic
Other
Chest pain
Seizures
Dyspnoea
Edema of the face, neck, upper thorax,
breasts, arms and fingers
Tachycardia
Stupor, coma
Orthopnoea
Dilatation of the vessels of the upper chest
Jugular vein distention
Cyanosis of the upper
part of the body
Headache
Tachypnoea
Dysphagia and hoarseness
Visial disturbances
Pleural effusion
Periorbital edema and engorged conjunctivae
Dizziness
Nonproductive
cough
Psychiatric changes
Syncope
Facial plethora
Figure 2
Dilatation of the vessels of the upper chest (collateral drainage).
Poszerzenie żył widoczne w górnej części klatki piersiowej (krążenie oboczne).
J. Bochenek-Cibor i wsp.
is required to initiate chemotherapy which is
used to treat chemosensitive tumors, such
as small-cell lung carcinoma or lymphoma.
Systemic treatment of small-cell lung carcinoma is based on cisplatin with etoposide.
Taxanes, anthracyclines, alkylating agents
and nucleoside analogs may also be used
[19]. In the case of non-Hodgkin’s lymphoma,
the schedule of chemotherapy depends on
the immunohistological diagnosis. In some
cases, monotherapy with an alkylating agent
or nucleoside analog may prove sufficient. In
other cases, polychemotherapy with alkylating agents, anthracyclines, vinca alkaloids
and immunomodulators is administered [20].
Symptoms relief is usually reported in 1–2
weeks after the first cycle of chemotherapy
[7]. Combined chemoradiotherapy may also
be considered provided that the tumor is
radiosensitive [21].
Chemotherapy is also applied to patients
with non-small cell lung carcinoma who have
already received the maximum dose of radiotherapy to the mediastinum [22].
Surgery can be either minimally invasive
like coronary intravascular stent placement,
or an open-chest operation. Intravascular
stenting is safe and provides the most immediate relief. It can be accomplished even if
there is complete SVC obstruction or thrombosis. That is why percutaneous placement
of the intravascular stent is the preferred
first-line intervention. Anticoagulation is
usually recommended after intravascular
stent placement [23]. Angioplasty is typically
performed in preparation for stenting as itself
it does not prevent recurrences [24]. Complications of stenting such as occlusion, infection, pulmonary embolus, stent migration,
haematoma at the insertion site, bleeding
and perforation have been reported in 3–7%
cases. Surgical bypass is rarely performed
due to the success of endovascular devices,
but when it is the treatment of choice, it is
associated with few complications and good
effectiveness. Sometimes, SVC gets locally
resected and a reconstruction with a venous
patch or graft replacement is carried out.
Grafts are made of Dacron and polytetrafluoroethylene or they are autologous from
saphenous vein. Surgical thrombectomy,
sternotomy and thoracotomy with or without
extensive resection and reconstruction of
the SVC are also performed. The operative
mortality of 5% and the patency of 80-90%
are reported [25].
Pharmacotherapy is helpful in relieving
symptoms. Corticosteroids should be administered in order to reduce edema and
inflammation around the tumor. However,
their effectiveness is controversial [26]. Diuretics may also prove useful, but they need
to be administered very carefully for the risk
of decreased vascular volume and hypovolemic shock [27]. Medical management of
Przegląd Lekarski 2014 / 71 / 12
SVCS includes also the thrombolytic therapy
used to treat intraluminal thrombosis. Tissue
plasminogen activators can effectively lyse
clots. In the case of catheter-induced thrombosis, the intravascular devices should be
immediately removed [28]. Anticoagulants
should also be prescribed to the patients
with cancer-related SVCS as the presence
of malignancy is a potential risk factor for
thrombosis [29]. Attention must be paid to
the risk of hemorrhage.
Authors want to stress the importance of
early detection of the first signs of SVCS. It
allows time for the final histological diagnosis which determines adequate treatment
modality. In fact, the entire medical staff
should assume a proactive role and ask
patients at risk about the typical symptoms
of SVCS. Diagnosing this syndrome is a
challenge not only for general practitioners,
medical oncologists, palliative care specialists and radiologists but also the remaining
personnel.
Summary
SVCS is an emergency in oncology,
resulting from compromised blood flow
through the SVC. The treatment modality
depends on the histological diagnosis. Radiation therapy, chemotherapy and surgery
are the treatment options. Unfortunately,
there are no evidence-based guidelines on
how to manage SVCS. Most data come from
case series. The prognosis is poor and the
average life expectancy is 6 months [30].
To sum up, it is important to prevent
SVCS development by early diagnosis and
efficient treatment of cancer. Nevertheless, if it does arise, it should be promptly
diagnosed and treated. That is why all the
physicians have to take it into account while
making the differential diagnosis, especially
among the patients with cancer.
Acknowledgements
Authors would like to thank Mrs Joanna
Gołąb for editing the English version of the
article.
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