Abstract
Case series
Patients
Male, 68-year-old • Male, 75-year-old
Final Diagnosis
Superior vena cava syndrome
Symptoms
Diffuse facial and bilateral upper edema
Clinical Procedure
Superior vena cava stenting
Specialty
Pulmonology
Objective
Unusual clinical course
Background
Superior vena cava syndrome (SVCS) is a clinical condition most commonly caused by malignancy. Its management becomes challenging when complicated by interstitial pneumonia because radiotherapy carries a high risk of fatal adverse events. This report presents 2 cases of malignant SVCS complicated by interstitial pneumonia that were successfully managed with endovascular superior vena cava (SVC) stenting.
Case Reports
Case 1: A 68-year-old man with advanced small-cell lung cancer was admitted due to facial and bilateral upper-limb edema. He was diagnosed with SVCS caused by tumor compression based on contrast-enhanced computed tomography (CT). With a prognosis of surviving only a few months, and radiation therapy deemed difficult due to interstitial pneumonia, SVC stent placement was performed. After the procedure, the edema improved and he was discharged after 7 days.
Case 2: A 75-year-old man with pulmonary squamous cell carcinoma was admitted due to facial and bilateral upper-limb edema, and dyspnea. A diagnosis of SVCS caused by tumor compression was established using contrast-enhanced CT. Based on a prognosis of surviving several months and interstitial pneumonia as a contraindication for radiation therapy, SVC stent placement was performed. The edema improved after the procedure and he was discharged 7 days later.
Conclusions
Stenting can be a useful indication for malignant SVCS, even in cases complicated by interstitial pneumonia. SVC stenting offers a fast and safe treatment option, quickly improving symptoms and quality of life, and allowing for early patient discharge.
Keywords: Edema; Lung Diseases, Interstitial; Stents; Vena Cava, Superior
Introduction
Superior vena cava syndrome (SVCS) is a clinical condition caused by venous flow obstruction due to external compression, or internal stenosis or occlusion of the superior vena cava (SVC) [1]. Malignancy is the most common cause of SVC obstruction, accounting for approximately 70% of cases [2]. Lung cancer is the leading cause of malignant SVCS, responsible for 75% of cases [3,4]. Within lung cancer types, non-small-cell lung cancer makes up 50% of SVCS cases, while small-cell lung cancer accounts for 25% [3,4]. Non-Hodgkin’s lymphoma and metastatic lesions from non-lung cancers each account for approximately 10% of cases [3,4]. The remaining 5% of cases are due to Hodgkin’s lymphoma; esophageal, thyroid, and germ-cell tumors; leukemia; and other causes [3,4]. SVCS has a wide range of signs and symptoms [1]. Facial, neck, and upper-limb swelling are the most common symptoms [5]. Rarely, patients present with cough, dyspnea, hoarseness, or even stridor when an acute obstruction arises from a rapidly enlarging tumor [5].
While there are no universally accepted treatment guidelines for SVCS, conventional management options include radiation therapy, with or without chemotherapy, surgical bypass, and endovascular therapy [6]. When the initiation or continuation of chemotherapy for lung cancer is difficult, or when tumor shrinkage is not expected from chemotherapy, radiation therapy alone is often performed [7]. However, given the risk of radiation pneumonitis or acute exacerbation of interstitial pneumonia, radiation therapy is difficult in cases complicated by interstitial pneumonia [8]. Recently, endovascular intervention with stenting has become the standard of care for SVCS [2]. Several reports have described stenting in tumors with low radiosensitivity or when urgent therapeutic intervention is required [9–11]. However, we were unable to find any cases of SVCS complicated by interstitial pneumonia in the literature.
This report presents 2 cases of malignant SVCS complicated by interstitial pneumonia in which radiation therapy carried a high risk of fatal adverse events and endovascular stenting was selected as a palliative treatment option.
Case Reports
Case 1
The patient had a medical history of interstitial pneumonia and chronic obstructive pulmonary disease, with a smoking index of 900. Regarding interstitial pneumonia, thoracic computed tomography (CT) revealed honeycombing at the lung bases, subpleural reticular opacities, and ground-glass opacities, as well as traction bronchiectasis, presenting usual interstitial pneumonia pattern (Figure 1A–1C). Based on these findings, he was clinically diagnosed with idiopathic pulmonary fibrosis. He was receiving apixaban for deep vein thrombosis, and was originally diagnosed with extensive small-cell lung cancer at age 66. Carboplatin and etoposide were administered as first-line treatment and amrubicin as second-line treatment. Subsequently, when carboplatin and etoposide were re-administered as third-line treatments, drug-induced pneumonitis developed (Figure 1D). Subsequently, chemotherapy for small-cell lung cancer was discontinued, and the best supportive care was provided. The tumors gradually grew over time.
Figure 1.
Presentation of Case 1. (A) Thoracic computed tomography (CT) showing honeycombing at the lung bases (red arrows). (B) Thoracic CT revealing subpleural reticular opacities and ground-glass opacities (red arrows). (C) Thoracic CT showing traction bronchiectasis (red arrows). (D) Thoracic CT of drug-induced lung injury that developed during chemotherapy with carboplatin and etoposide.
At the age of 68, he was admitted for treatment of diffuse facial and bilateral upper-extremity edema. At the time of admission, his weight was 59.6 kg and his height was 163 cm. His vital signs were as follows: blood pressure, 141/104 mmHg; pulse rate, 105 beats/min; pulse oximetry, 94% with nasal cannula oxygenation at 4 L/min; respiratory rate, 19/min; and body temperature, 35.8°C. Physical examination revealed facial and bilateral upper-extremity edema (Figure 2A). His blood test results were unremarkable. A chest radiograph showed an enlarged mass shadow in the right pulmonary hilar region compared to the previous month. Contrast-enhanced CT revealed severe narrowing of the SVC due to compression by a mass in the right pulmonary hilum (Figure 3). Hypoalbuminemia, heart failure, and venous thrombosis – which can cause generalized edema – were considered unlikely based on the test results. He was therefore diagnosed with SVCS. Because there were no additional anticancer drug options, his prognosis for survival was only a few months. Due to the interstitial pneumonia, radiotherapy was difficult, and SVC stenting was therefore indicated.
Figure 2.
Facial edema in Case 1. (A) On admission, the face is markedly edematous. (B) After superior vena cava stenting, facial edema improved.
Figure 3.
Contrast-enhanced thoracic computed tomography (CT) in Case 1 showing a tumor of the right pulmonary hilum (red arrows). The tumor is compressing the superior vena cava (SVC) and narrowing its lumen. (A, B) Transverse section. (C) Coronal section. (D) Three-dimensional-constructed CT image showing SVC blood flow disruption (red arrow). RA – right atrium; PA – pulmonary artery; Ao – aorta.
After obtaining informed patient consent, an 8-Fr sheath was inserted into the right common femoral vein and another into the right internal jugular vein. SVC venography revealed stenosis due to the tumor (Figure 4A). A 0.035-inch guidewire was passed through the stenosis. The guidewire was captured by a 10-mm snare in the SVC; “pull-through” was achieved (Figure 4B). Subsequently, a stent delivery system was inserted beyond the site. A self-expandable bare metallic stent, 20 mm in diameter and 8 cm in length (Spiral Relief Stent; Cosmotec, Tokyo, Japan), was placed across the stenotic site (Figure 4C, 4D). Angioplasty using a balloon catheter was performed over the region of greatest stenosis. The stenosis was dilated using a balloon catheter while viewing the vessel morphology using sheath angiography (Figure 4E). Venography was then performed to confirm improvement in blood flow to the SVC (Figure 4F). The pressure in the SVC decreased after stenting, and his facial and bilateral upper-extremity edema improved (Figure 2B). Apixaban, which was originally administered internally, was continued after stent implantation to prevent stent occlusion due to thrombi. The patient was discharged from the hospital on day 7 of his stay. He spent time at home without any SVCS symptoms or complications from SVC stenting until he died of worsening lung cancer approximately 2 months after discharge.
Figure 4.
Superior vena cava (SVC) arteriography in Case 1 showing stenosis due to a tumor (red arrow) (A). Guidewire is fixed from the SVC to the inferior vena cava (B). Stent insertion into the SVC stenosis (C, D). Stent dilation using a balloon (E). After stenting, venography showed improvement in SVC blood flow (red arrow) (F).
Case 2
This patient had a medical history of interstitial pneumonia, diabetes mellitus, hypertension, angina pectoris, and an abdominal aortic aneurysm, with a smoking index of 250. Regarding interstitial pneumonia, thoracic CT showed probable usual interstitial pneumonia pattern, with reticular opacities and ground-glass opacities predominantly in the bilateral lower lobes beneath the pleura, along with traction bronchiectasis (Figure 5A–5C). Based on these findings, he was clinically diagnosed with idiopathic pulmonary fibrosis. At the age of 72, he was diagnosed with squamous cell lung carcinoma (stage IB) and underwent a right lower lobectomy. Lung cancer recurred at 75 years of age, but only palliative treatment was administered owing to progressive cachexia and a decline in performance status. Subsequently, the tumor grew gradually over time.
Figure 5.
Presentation of Case 2. (A, B) Thoracic computed tomography (CT) showing subpleural reticular opacities and ground-glass opacities (red arrows). (C) Thoracic CT revealing traction bronchiectasis (red arrow).
He was admitted to our department for the treatment of diffuse facial edema, bilateral upper-extremity edema, and shortness of breath. At admission, his weight and height were 48.0 kg and 165.8 cm, respectively. His vital signs were blood pressure 153/80 mmHg; pulse rate 72 beats/min; pulse oximetry 92% on room air; respiratory rate 20/min; and body temperature 36.1°C. Physical examination revealed facial and bilateral upper-extremity edema. His blood examination results were unremarkable. Chest radiography revealed a massive pleural effusion and a tumor in the right lung. Contrast-enhanced CT revealed severe narrowing of the SVC due to compression by the tumor occupying the right thoracic cavity (Figure 6). Hypoalbuminemia, heart failure, and venous thrombosis – which can cause generalized edema – were considered unlikely based on the test results. He was therefore diagnosed with SVCS with a prognosis of only surviving a few months. Radiotherapy was impossible because of interstitial pneumonia; therefore, SVC stenting was indicated to improve his edema. After obtaining informed consent from the patient, an 8-Fr sheath was inserted into the right common femoral vein and another into the right internal jugular vein. SVC venography revealed tumor-induced stenosis (Figure 7A). A 0.035-inch guidewire was passed through the stenosis and was captured by a 10-mm snare in the SVC; “pull-through” was achieved. A stent delivery system was subsequently inserted beyond the site. A self-expandable bare metallic stent, 20 mm in diameter and 6 cm in length (Spiral Relief Stent), was placed across the stenotic site. A balloon catheter was used to perform angioplasty over the region of greatest stenosis. The stenosis was dilated using a balloon catheter while viewing the vessel morphology via sheath angiography. Venography was then performed to confirm improvement of blood flow to the SVC (Figure 7B). Following stenting, the pressure in the SVC decreased, and facial and bilateral upper-extremity edema improved. To mitigate the risk of stent occlusion due to thrombi, edoxaban administration was initiated following stent placement. The patient was discharged from the hospital on day 7 of his stay. He spent time at home without any symptoms of SVCS or complications from SVC stenting until he died of worsening lung cancer approximately 1 month after discharge.
Figure 6.
Presentation of Case 2. (A-C) Contrast-enhanced thoracic computed tomography (CT) showing a large tumor occupying the right thorax. The tumor compresses the superior vena cava (SVC) and narrows its lumen (red arrows) (A: coronal section, B: sagittal section) (C) Three-dimensional images by enhanced CT showing disruption of SVC blood flow (red arrow). RA – right atrium, PA – pulmonary artery, Ao – aorta.
Figure 7.
Superior vena cava (SVC) arteriography in Case 2 showing SVC stenosis due to the tumor (red arrow) (A). Dilation of SVC stenosis after stenting (B).
Discussion
This report highlights 2 key clinical insights from our experience of endovascular stenting for malignant SVCS complicated by interstitial pneumonia. First, stenting may be a useful indication for patients with SVCS who have a poor prognosis. Second, SVC stenting can be safely performed in patients with SVCS complicated with interstitial pneumonia.
Compared to other SVCS treatments, SVC stenting has several advantages. Although to the best of our knowledge there are no prospective randomized studies on SVCS treatment, a quantitative synthesis showed that the technical success rate of stenting was 98.8% [6]. The total complication rate of SVC stenting is approximately 9%, and the procedural mortality rate is approximately 1% to 2% [12]. In a retrospective cohort of patients with malignant SVCS, the average time required for stent placement was approximately 90 minutes, which was shorter than that required for other treatments [5]. Moreover, the rates of symptom relief from stenting, radiation therapy, chemotherapy, and surgery were 80–95%, 56–96%, 79–93%, and 59–77%, respectively [1,13]. Additionally, the times for symptom relief were 0–72 hours, 3–30 days, 1–2 weeks, and 0–72 hours, respectively [1,13]. The benefits of SVC stenting include rapid resolution of symptoms with a high technical success rate, minimal procedural complications, and a relatively short procedure duration. Utilization of intravascular ultrasound during SVC stent placement allows for confirmation of the stenotic area and tumor, as well as measurement of the vessel lumen diameter to prevent the use of improperly sized stents. Such advancements in the technique of SVC stent placement are expected to further reduce the risk of complications [14]. In our 2 cases, there were no additional anticancer drug options, and patients’ prognoses for survival were only a few months. Their facial and bilateral upper-extremity edema improved quickly after SVC stenting, allowing them to be discharged within a week. They were able to live the rest of their lives without recurrence of SVCS symptoms. For patients with a short life expectancy due to a primary disease, their remaining days are very valuable, and it is important to quickly improve their quality of life after a short hospital stay.
SVCS scoring systems are based on severity and a treatment strategy can be selected according to the score. Yu et al described the severity of SVCS based on clinical presentation and proposed a grading scale ranging from 0 (asymptomatic) to 5 (death) [15]. This scale is useful for making diagnostic and treatment decisions [2,10,15]. The Kishi score is another rating system that was developed to assist decision-making regarding stent therapy [16]. It evaluates neurological, laryngeal, facial, and cardiovascular signs and symptoms to assess SVCS severity. A Kishi score >4 indicates severe symptoms and serves as an indication for SVC stenting to lower the venous pressure and prevent cerebral edema [2,17,18]. Using the scale of Yu et al, both Cases 1 and 2 were Grade 2, while the Kishi score was 2 and 4 for Case 1 and 2, respectively. Stenting was not indicated for Case 1 based on the Kishi score. Therefore, indications for SVC stenting should be determined not only by score, as in Case 1, but also by considering the patient’s symptoms, prognosis, and expectations.
Stenting is an effective treatment option for SVCS, particularly in patients with interstitial pneumonia. Radiation therapy often leads to radiation pneumonitis, a frequent adverse effect [19]. Patients with interstitial pneumonia are typically excluded from clinical trials of radiation therapy because the presence of interstitial pneumonia is a risk factor for severe radiation pneumonitis and increases the risk of treatment-related mortality [19]. In addition, interstitial pneumonia can lead to acute exacerbations due to radiation pneumonitis [20]. When radiation therapy is performed for lung cancer combined with interstitial pneumonia, acute exacerbation of interstitial pneumonia or radiation pneumonitis occurs in 20% to 30% of cases [8,21]. Furthermore, patients with interstitial pneumonia have a 165.7 times higher risk of death during radiation therapy compared to those without interstitial pneumonia [8,21]. Our patients had interstitial pneumonia, which made it difficult to perform radiotherapy for SVCS. In both cases, SVC stenting was performed without exacerbation of interstitial pneumonia. There are no guidelines for the appropriate treatment of SVCS complicated by interstitial pneumonia. Therefore, further case studies are needed to establish the optimal treatment strategy.
The phase II trial reported by Takeuchi et al involved stent placement in 28 patients with SVCS secondary to malignant tumors, demonstrating significant symptom-score improvement and confirming its utility [22]. Wong et al further recommended considering stenting as first-line therapy in malignant SVCS based on response rates, durability of response, radiation technique, and overall prognosis [5]. We suggest that SVC stent placement can also benefit patients with poor prognoses or comorbid interstitial pneumonia. However, this case report is limited by its small sample size (2 cases) and unknown long-term outcomes. Although further case accumulation is needed, the rarity of the procedure may delay consensus development. While fatal complications are rare, stenting carries risks such as cardiac tamponade, acute pulmonary embolism, and respiratory failure [6]; thus, thorough individual assessment is essential. In patients with interstitial pneumonia, where radiotherapy may be high-risk, SVC stenting is a viable alternative treatment.
Compared with previously reported cases of malignant SVCS managed by endovascular stenting, our patients presented with typical symptoms of facial and upper-extremity edema, but had the additional complication of interstitial pneumonia, which made it difficult to perform radiotherapy [9–11,14]. In contrast to some reports using intravascular ultrasound to optimize stent sizing [14], our procedures were performed under conventional venography guidance and achieved rapid symptom improvement without complications. These findings suggest that SVC stenting can be an effective and safe palliative option, even in patients with comorbid interstitial pneumonia.
Conclusions
SVC stenting can be a good indication for patients with SVCS who have a poor prognosis. Moreover, it can offer a safe and effective palliative treatment for malignant SVCS complicated by interstitial pneumonia, providing rapid symptom relief and early discharge.
Acknowledgments
We would like to thank Editage (www.editage.com) for their assistance with English language editing.
Footnotes
Financial support: None declared
Conflict of interest: None declared
Publisher’s note: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher
Institution Where Work Was Done: Chiba University Hospital, Chiba, Japan
Patient Consent: As the patients had already died, written consent was obtained from their families.
Declaration of Figures’ Authenticity: All figures submitted have been created by the authors who confirm that the images are original with no duplication and have not been previously published in whole or in part.
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