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 Table of Contents  
REVIEW ARTICLE
Year : 2016  |  Volume : 12  |  Issue : 2  |  Page : 515-519

Superior vena cava syndrome: A radiation oncologist's perspective


1 Department of Radiation Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
2 Department of Medical Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
3 Department of Surgical Oncology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

Date of Web Publication25-Jul-2016

Correspondence Address:
Kaustav Talapatra
Department of Radiation Oncology, Kokilaben Dhirubhai Ambani Hospital, Four Bunglows, Andheri (W), Mumbai - 400 053, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.177503

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 > Abstract 


Superior vena cava syndrome is referred to as a constellation of symptoms and signs caused by obstruction of superior vena cava. It can occur due to both benign and malignant causes with the latter being the predominant. There is a paradigm shift in the approach to manage this condition. It is no longer considered a medical emergency and histological diagnosis is necessary before treatment. This article reviews the causes, symptoms, pathophysiology, and overall management policy which have changed over decades.

Keywords: Chemotherapy, medical emergency, radiation therapy, stent, superior vena cava syndrome


How to cite this article:
Talapatra K, Panda S, Goyle S, Bhadra K, Mistry R. Superior vena cava syndrome: A radiation oncologist's perspective. J Can Res Ther 2016;12:515-9

How to cite this URL:
Talapatra K, Panda S, Goyle S, Bhadra K, Mistry R. Superior vena cava syndrome: A radiation oncologist's perspective. J Can Res Ther [serial online] 2016 [cited 2019 Sep 21];12:515-9. Available from: http://www.cancerjournal.net/text.asp?2016/12/2/515/177503




 > Introduction Top


Superior vena cava syndrome (SVCS) is a known clinical situation in an oncology setting which occurs due to intrinsic and extrinsic compression of superior vena cava. SVCS is a distressing syndrome to the patient and health care providers.

Background

SVCS was initially described by Hunter in 1757 in a patient with large syphilitic aortic aneurysm compressing the SVC.[1] In the 1950s, Schechter studied series of cases with SVCS and attributed syphilitic aneurysm or tubercular mediastinitis to be the cause in almost half of them.[2] Although these infectious conditions were the major etiological factors, a few decades ago, current progression in antibacterial therapies and improvement in socioeconomic condition leads to their downfall. In the recent years, there has been an upsurge in malignancy and intravascular device-related thrombotic events as the cause of SVCS.[3]

Causes

A wide variety of etiological factors is responsible for SVCS. Malignancy contributes to Superior vena cava obstruction (SVCO) in more than 90% of the cases with lung cancer being the most common cause followed by lymphoma.[3],[4],[5],[6]

Approximately, 75% of all cases of SVCS are lung cancer.[4],[7],[8] Right-sided lung cancer is more prone to cause SVCS than left-sided counterpart.[4] Small cell lung cancer (SCLC) is the most common histologic type of lung cancer related to SVCO.[9] In a study by Sculier et al. among 643 patients with SCLC, SVCS was present in 8.6% before the start of treatment.[10] In a Cochrane review by Rowell and Gleeson, SVCS was present in 10% cases of SCLC and 1.7% cases of non-SCLC (NSCLC) at diagnosis.[11]

Lymphoma contributes to around 15% of cases of SVCS.[12] In the study by Perez-Soler et al., 36 of 915 patients with non-Hodgkin's lymphoma presented with superior vena cava syndrome (SVCS), and the histologic types associated were a diffuse large cell in 23 patients, lymphoblastic in 12, and follicular large cell in one patient.[12] Hodgkin's lymphoma rarely causes SVCS.[13] Metastatic carcinomas account for around 5% of cases of SVCS.[13]

The other important causes are thymoma, thyroid carcinoma, esophageal cancer, germ cell tumor, and breast cancer.[4]

In recent years, important benign emerging cause is various intravascular devices (e.g. central venous lines, parenteral feeding lines, and pacemaker, etc.) related thrombotic events.[3],[4]


 > Pathophysiology Top


The pathogenic basis of SVCS is the impaired venous drainage from the head, neck, upper extremities, and upper thorax due to obstruction of SVC.

Superior vena cava obstruction results most commonly from the extrinsic compression of the superior vena cava (SVC) by a neoplastic process such as tumor arising in the right main or upper lobe bronchus or by large volume mediastinal lymphadenopathy (subcarinal, perihilar and, paratracheal) and less commonly by neoplastic invasion and intravascular thrombus formation.[11],[15],[16]

Blood flow is redirected through multiple collateral blood vessels bypassing the obstruction into azygos vein or inferior vena cava depending on the level of obstruction. Over the course of time, the caliber of these collateral vessels increases leading to increased blood flow.[17],[18] Analysis of venacavograms by Stanford et al. in 27 patients has revealed four patterns of collateral return.[19]

Clinical presentation

SVCS is usually seen in the older age group with an average age of 50 years or higher. In a particular study by Armstrong et al., the mean age was found to be 55 years.[20]

The diagnosis of SVCS is mainly based on clinical findings.[14],[22] Facial puffiness and neck swelling are the most common presenting symptoms.[5],[22]

Other commonly noted symptoms are upper extremity swelling, shortness of breath, cough, dilated chest, and neck veins.[5] Raised venous pressure may lead to life-threatening consequences such as laryngeal or bronchial edema and cerebral edema.

The presentation may be acute (within few days), subacute (over 6 weeks), or chronic (more than 6 weeks) depending upon the rate and degree of narrowing of SVC. In acute cases, the collateral formation is less pronounced leading to more severe symptoms than subacute and chronic cases where there is sufficient time for proper formation and expansion of collaterals. For this reason in slow-growing diseases long-lasting, severe SVC obstruction can sometimes be found without significant related signs and symptoms.[22]

Is at an emergency?

Although in the past, SVCS was considered a potentially life-threatening medical emergency, at present, there are enough recent evidences suggesting that it is an urgency and appropriate care should instituted; however, it cannot be termed as a medical emergency.[23] In the study by Sculier et al., 55 patients with SCLC having SVCS, only one patient died even after delay in initiating treatment for 60–70 days.[10] Two pivotal reports about SVCS concluded that it does not constitute a radiotherapeutic emergency because there is little-shown evidence that venous obstruction has caused life-threatening situations (i.e., cerebral or laryngeal edema), and the patient's demise was the direct result of SVCO.[24],[25],[26]


 > Management Top


Traditionally, SVCS was treated with immediate radiation therapy without considering histological diagnosis pertaining to the belief that unresectable lung cancer would be the most probable cause, diagnostic procedures would carry a high-risk in this clinical context and also that SVCS was a medical emergency requiring immediate intervention.[9] Review of 1986, patients by Ahmann revealed that diagnostic procedures such as thoracotomies, mediastinoscopies, bronchoscopies, lymph node biopsies, and venograms can be performed safely and histological diagnosis before embarking upon treatment always should be done as diseases such as SCLC or lymphoproliferative disorders would be benefited from upfront combination chemotherapy and histological diagnosis after radiation therapy would be unsuccessful.[9]

A contrast-enhanced computed tomography scan of the chest is the most useful imaging study in detecting the underlying cause by guided biopsy and also the level of obstruction.[4] Judicious use of selected invasive diagnostic procedures such as cytology of sputum and/or bronchial washings, thoracentesis for pleural effusions, and needle biopsy of any palpable lymph nodes can be considered to establish the underlying cause.[11]

Treatment strategy includes purely symptomatic relief of SVCS and treatment of the underlying cause.[4] Common treatment modalities consist of steroid (prednisolone or dexamethasone) therapy, chemotherapy, external beam megavoltage radiotherapy, insertion of an expandable metal stent into the superior vena cava (with or without thrombolysis or anticoagulation), or any combination of these treatments.[11] The mainstay of symptomatic treatment includes supplementary oxygen, head elevation, use of diuretics, and often a course of parenteral steroids (dexamethasone, 4 mg every 6 h).[3],[4]


 > Treatment of Underlying Cancer Top


Radiotherapy

Radiotherapy is an effective treatment for SVCS. In a retrospective study by Armstrong et al. in 125 patients of SVCS due to various etiologies (79% bronchogenic carcinoma, 18% malignant lymphoma, and 6% other causes) revealed that approximately 80% of the patients obtained good to excellent symptomatic relief.[20] The optimal dose fractionation schedule of radiation therapy is yet to be established.[4] In the review study by Armstrong et al., both high fraction size (300–400 cGy daily for 3 fractions) and conventional fraction size (200 cGy daily, 5 weekly fractions) yielded similar good symptomatic relief within 2 weeks of treatment. A study by Davenport et al. advocated initial high dose per fraction followed by lesser dose per fraction until completion of the treatment.[27] In a study by Sculier et al., the radiation fractionations used were 6 Gy in a single fraction, 20 Gy in 5 fractions, 20 Gy in 10 fractions, and 30 Gy in ten fractions.[10] The treatment intent is mainly palliative and rarely curative.[4]

Radiation therapy in small cell lung cancer [Table 1]
Table 1: Small cell lung cancer

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In a prospective randomized study by Spiro et al. in SCLC patients with SVCS treated with chemotherapy only and chemotherapy and radiotherapy (40 Gy in 20 fractions) showed no additional advantage from adding radiotherapy in terms of initial response, median survival, and SVCS recurrence.[28] However, a more recent study by Chan et al. showed that addition of mediastinal irradiation significantly prolonged the time of SVCS recurrence in limited stage SCLC mainly.[23] The Cochrane review by Rowell and Gleeson showed that 77.6% (142/183) patients with SCLC were relieved of symptoms the following radiotherapy.[11]

Radiation therapy in nonsmall cell lung cancer [Table 2]
Table 2: Nonsmall cell lung cancer

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Radiation therapy is administered as the primary treatment modality in patients with NSCLC having SVCS.[20],[27],[29] In the Cochrane review, the objective response rate was seen in 63% (104/165) patients with NSCLC and SVCS.[11]

Following results were seen in various series indicating the effectiveness of radiotherapy and chemoradiotherapy in SCLC and NSCLC:

Radiation therapy in lymphoma

A study by Perez-Soler et al. in 36 patients with lymphoma and SVCO revealed that radiotherapy only was equivalent to chemotherapy alone or chemotherapy plus radiotherapy in achieving symptomatic relief but inferior in prolonging relapse-free survival and overall survival.[12] However, including radiotherapy resulted in fewer local recurrences in large cell lymphoma.[12]

Chemotherapy

Chemotherapy is an effective treatment for SVCS.[28],[34] A study by Sculier et al. demonstrated that symptomatic relief was seen in 43% (3/7) of patients initially treated with radiation and in 73% (35/48) of patients treated with induction chemotherapy.[10] In the Cochrane review, the objective response rate (partial and complete response) to chemotherapy was 68.4% (188/275).[11]

Chemotherapy is also standard of care in patients with lymphoma having SVCS.[4]

Stent insertion [Table 3]
Table 3: Results in endovascular stent

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Endovascular metallic expandable stents can be considered to provide rapid symptomatic relief in patients with suspected malignant SVCS with investigations and histological diagnosis being actively pursued.[35],[36] It is also recommended in persistent or recurrent SVCS after failing chemotherapy or radiotherapy.[37],[38],[39] A study by Nicholson et al. comparing the relative therapeutic efficacy of metal stents versus radiotherapy in malignant SVCS revealed that stenting provided higher response rate and faster relief of SVCS.[40] In the Cochrane review also stenting was found to provide the most effective and rapid treatment response with 151 out of 159 patients from 23 studies getting relieved.[11] Many studies also used balloon angioplasty and thrombolytic therapy in conjunction with stent insertion.[11] The drawback in the literature supporting stenting is the nonavailability of good level I/II evidence.

Role of steroids

Steroids are used routinely in the management of SVCO although there is no evidence to support of refute the use of steroids.[11] Conventionally, steroids have also been used with radiation therapy to reduce the radiation-induced edema.[4]

Role of surgery

Surgical intervention in the form of vascular grafting has a limited role in the management of malignant SVCS owing to favorable response with chemotherapy or radiotherapy and increased morbidity and mortality from surgery.[44] However, in highly selected patient population like advanced intrathoracic disease after chemotherapy or radiotherapy failure surgical salvage may be considered.[45]


 > Grading of Small Cell Lung Cancer Top


A grading system has been proposed by Yu et al.[46] depending upon the severity of symptom ranging from 0 (asymptomatic) to 5 (fatal). In Grade 1 (mild), Grade 2 (moderate), and most Grade 3 patients diagnostic and staging procedures should be initiated first, and treatment should be directed toward tumor type and stage. In Grade 4, (life-threatening) urgent stent insertion should be considered.[46]


 > Conclusion Top


In recent years, SVCS is considered a medical urgency and not an emergency. Clinical alertness and promptness in initiating the appropriate diagnostic and interventional steps are the crux of handling this syndrome rather than getting panicked by its gory presentation. Radiation therapy is an effective treatment and should be used judiciously following the establishment of proper histopathological diagnosis and stage of the disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 > References Top

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