Radiol Oncol 2000; 34(4): 337-47.
Algorithm for percutaneous stenting in patients suffering from superior vena cava syndrome
Radiotherapy Institute and Internal Medicine Institute, Faculty Hospital ofHealth and Social Faculty ofOstrava University, Czech Republic
Background. Superior vena cava syndrome (SVCS) has been considered an emergent, life-threatening condition far a long time. The rate of causes of the syndrome has changed substantially since its first description by W. Hunter in a patient suffering from saccular aneurysm ofsyphilitic aorta in 1757. Since the beginning ofthe era ofradiotherapy, this was the main treatment modality far patients with SVCS. The emergent feature ofthe syndrome required immediate initiation ofradiotherapy, often without the proper knowledge ofthe histopathological diagnosis of the SVCS underlying cause. The development ofradiotherapy and chemotherapy in various types ofcancer; and the development of supportive care in oncology and an understanding that SVCS is not an emergent life-threatening oncological condition evoked a need far treatment differentiation in SVCS patients.
Conclusions. Percutaneous stenting became a very efficient method in the treatment of SVCS patients since 1986. The purpose ofstenting is supportive and/ar palliative. The algorithm far stenting use has been developed. Algorithm is based on 4 questions emerging from daily clinical practice. To get valid responses, certain diagnostic procedures and tools are recommended and required.
1.	Does the patient really suffer from SVCS?
2.	What is the patient's general condition?
3.	Is the stenting of SVCS contraindicated? (What is the origin of SVCS, what is the severity of SVCS?)
4.	Is the histopathology of the process causing SVCS known? What is the histopathology of the process causing SVCS?
Responses to the questions above give reasons far the selection of a freatment modality. Rational usage of percutaneous implantable stents in properly chosen patients suffering from SVCS of malignant ethiology ensures efficient differentiation offreatment modalities in supportive and/or palliative care ofSVCS patients.
Key words: superior vena cava syndrome-therapy; stents; palliative care, algorithm for stenting
Pave! Vodvafka, Petr Stverak
Received 13 September 2000 Accepted 25 September 2000
Corespondence to: Pavel Vodvarka MD Ph.D., Radiotherapy Institute and Internal Institute of Teaching Hospital of Health and Social Faculty, 17. listopadu 1790, 70852 Ostrava - Poruba, Czech Republic. Phone: +420 69 698 2264; Fax: +420 69 691 9010; E-mail: pavel.vodvarka@fnspo.cz
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Vodvtii'ka P and Stvertik P / Superior vena cava syndrome
Introduction
Superior vena cava syndrome (SVCS) is a critical condition diagnosed frequently by the symptoms and signs at the present time. SVCS may be caused by obstruction of SVC by tumorous invasion of venous walls and lumen or by extrinsic pressure of tumor mass both can be accompanied by intravascular trombosis.1'2 SVCS was firstly reported and described by William Hunter in 1757.3 In the 17th century however, the majority of SVCS was caused by inflammatory conditions such as saccular aneurysm of syphilitic aorta as in Hunter's first case. Bronchogenic carcinomas and malignant lymphomas are responsible for a vast majority of SVCS today.4-11 Moreover, there is a relatively new group of causes of SVCS that can be described as iatrogenic.u Proportions of malignant and benign causes of SVCS in the long run are given in Table l. Recently, a comprehensive overview of SVCS published causes has been given elsewhere.11 For a rather long period of time, SVCS has been suggested as a life threatening condition requiring an immediate treatment (usually radiotherapy) even without previous knowledge of tissue diagnosis.8'18-24 The development of radiotherapy tactics used in the treatment of SVCS was described in previous papers.8'18-20'24 Thus, radiotherapy became the main treatment modality for all (presumably) malignant cases of SVCS.1'25'26
Experimental animal SVCS models, research of human SVCS causes, supportive care implementation and its results, better understanding of symptoms and sings development, revision of overall survival results were keystones of a change in the opinion on SVCS.4'27 Now, SVCS is not considered as an emergent, really life-threatening condition as originally thought of.4'9'28 Except in rare cases of SVCS with brain edema and/or intrabronchial obstruction accompanied with significant dyspnoea, there is no reason to start with immediate treatment without proper tis-
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sue diagnosis of the SCVS cause. The use of supportive treatment methods can diminish and alleviate symptoms and signs of SVCS for a time long enough to allow safe tissue biopsy and to establish the histopathological diagnosis of the SVCS cause.4'10'11'29
The diagnosis is the most important for a more "specific" treatment of SVCS. Such treatment can ensure fast disappearance of symptoms and signs of SVCS and longer overall survival in certain cases in comparison with previously universally used radiotherapy.30'31
Methods of supportive treatment in patients suffering from SVCS changed substantially. Intravenous stents started to be used in supportive and/or palliative treatment of SVCS in 1986.32 At present, several kinds of metallic stents are used.33'47 If properly indicated, stent indwelling may faster alleviate and exterminate the significant SVCS symptoms and signs.1'2'29
The treatment with self-expandable or balloon-expandable stents is not suitable for all patients because there are contraindications in stent indwelling as well as in certain situations that can be debatable from the point of view of the achievable aim of treatment and cost-benefit ratio.42-44'48'49
For the rational use of stents in supportive and/or palliative treatment of SVCS, the algorithm for stents indwelling has been developed. It is based on 4 questions that can be answered after the following examinations.28
Question No. 1: Does the patient really suffer from SVCS?
Reason for the question No. 1
The question is necessary because several patients had been referred for SVCS treatment while they suffered from different diseases (like parotitis, dermatological diseases, allergic edema, status post tooth extraction).10'50
352	Vodvtii'ka P and Stvertik P / Superior vena cava syndrome

Response
Response can be given by personal medical history, basic physical exam and duplex sonography assessing the blood flow of big veins. If the flow curve from duplex sonography is not physiological, computerized tomography with contrast medium and/or phlebography are necessary to obtain a more correct diagnosis.
If the response is "YES", go to the Question No.2.
If the response is "NO", go to the Treatment No.l.
Treatment No l
Give different treatments for the specific (different) diseases.
Question No. 2: What is the patient's general condition?
Reason for the question No. 2
The question is needed because patients in very poor general condition are incapable to undergo diagnostic procedures. Their death can be imminent. The stenting would be not ethical in this situation.
Response
Response can be given by an evaluation of the patient's condition by the examination of his or her subjective and objective status (basic physical examination) and according to certain validated models. E.g.: Escalante's model,51 Karnofsky's scale etc. (Table 1).
If the response is "POOR", go to the Treatment No. 2.
Table l. Escalante's validated model for predicting imminent death
Progressive disease Zubrod >/= 3 Triage Pulse >/= 110/min Respiration >/= 28/min
If the response is "GOOD", go to the Question No. 3.
Treatment No. 2
Palliative treatment at the hospice or at the palliative care unit for the symptomatic treatment (corticosteroids, diuretics, positioning, oxygen, antibiotics, etc.)
Question No. 3: Is the stenting of SVC
contraindicated? (What is the origin of SVCS, what is the severity of SVCS?)
Reason for the question No. 3
Stenting is contraindicated in patients with excessive obliteration of the large veins or their extensive invasion by of cancer (SVC and both brachiocephalic veins).
Table 2. Kishi's scoring system for signs and symptoms of SVCS obstruction
Signs and symptoms	grade
Neurological symptoms	
Stupar, coma, or blackout	4
Blurry vision, headache, dizziness,	
or amnesia	3
Changes in mentation	2
Uneasiness	1
Laryngopharyngeal or thoracic symptoms	
Orthopnea or laryngeal edema	3
Stridor, hoarseness, dysphagia,	
glossal edema, or shortness	
of breath	2
Cough or pleural effusions	1
Nasal and facial signs or symptoms	
Lip edema, nasal stiffness, epistaxis,	
or rhinorhea	2
Facial swelling	1
Venous dilatation	
Neck vein or arm vein distension,	
upper extremity swelling,	
or upper body plethora	1
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Vodvtii'ka P and Stvertik P / Superior vena cava syndrome
Response
Response can be given by phlebography with digital subtraction (DSA) and spiral computerized tomography with contrast medium. Clinically, a severity of SVCS can be evaluated by Kishi's48 or Nicholson's49 classifications (Table 2).
If the response is "YES", go to the Treatment No. 3.
If the response is "NO", go to the Questions No. 4.
Treatment No. 3
Supportive care with corticosteroids, diuretics, oxygen, positioning, anticoagulants, antibiotics; relevant histopathology (endoscopy and/or biopsy); anticancer palliative therapy may be applied (chemotherapy or radiotherapy, or consider bypass).
Question No. 4a: 1s the histopathology of the process causing SCVS known?
Reason far the questions No. 4
73 - 97% of SVCSs are caused by cancer. (Table 3)
The question is raised by different treatments of different cancers.
Table 3. The rate of benign and malignant causes of SVCS during the time since its first observation
Year	Author	Causes	
		Benign	malignant
		(%)	(%)
1757	Hunter3	97	3
1934	Ehrlich12	54	46
1949	Mclntire13	67	23
1954	Schechter14	40	60
1975	Lokich8	3	97
1987	Fincher15	13	87
1992	Baker1	10	90
1993	Escalante2	3	97
1994	Tayade16	13	87
1998	Kee17	27	73
If the response is "NO", go to Treatment No. 4A.
If the response is "YES", go to Question No. 4B.
Treatment No 4A
Supportive care: Percutaneous stenting and endoscopy and/or bioptic methods to obtain tissue sample.
Question No. 4B: What is the histopathology of the process causing SVCS?
SVCS can appear in patients suffering from malignant tumors in three situations:10'44
Group A: SVCS is the first sign of cancer (its
histopathology is not known). Group B: SVCS appears during a diagnostic process due to suspect for cancer (its histopathology can or cannot be known).
Group C: SVCS appears during the follow-up period (histopathology is usually known if cancer progression is revealed), in some cases, an oncological treatment is exhausted and cannot be repeated (group Cl), in other cases, progressive disease is not praven - a cause of SVCS may be late sequels of the previous treatment (group C2).
According to our assessment of 151 patients with SVCS, the rates of patients in the groups are as follows: A - 25%, B - 65%, C -10%.1°-50
Chemosensitive cancers (groups A, B, C)
-	Lymphomas
-	Small cell lung cancer
-	Germ cell tumors
-	Etc
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Rather chemoresistant cancers (groups A, B, C)
-	Non small cell lung cancer
-	Malignant melanoma
Progressive cancer - all oncological treatment is already exhausted (group CI)
-	Further oncological treatment is not possible any more
Tie cause ofSVCS is late sequel of previous oncological treatment (group C2)
-	Radiation fibrosis of mediastinum Treatment No. 4B
-	Differentiated chemotherapy according to the diagnosis (as curative or palliative treatment of patients of groups A, B, and the rest of group C - see the group definitions)
-	Percutaneous stenting or surgical methods (bypass) (both as a palliative treatment in patients of the groups Cl and C2 and as supportive care in patients of groups A and B and rest of group C), and
-	Chemotherapy and/or radiotherapy (as curative or palliative treatment in patients of groups A, B, and the rest of group C)
In summary, endovascular treatment is a simple and safe procedure to restore the patency of SVC in patients with malignancies. In most cases, it should be indicated as the first-line treatment and performed as early as possible. With proper knowledge of the SVCS cases, the best results could be obtained, if stenting follows the correct treatment schedule.
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