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Differential Diagnoses of Malignant Superior Vena Cava Syndrome

Differential Diagnoses of Malignant Superior Vena Cava Syndrome

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The superior vena cava syndrome (SVCS) comprises various symptoms due to occlusion of the SVC, which can be easily obstructed by pathological conditions (eg, lung cancer, due to the low internal venous pressure within rigid structures of the thorax [trachea, right bronchus, aorta]). The resulting increased venous pressure in the upper body may caus...

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... Additionally, vasculitic disorders (eg, Behçet disease) can also cause relevant venous occlusion. Table 1 summarizes non-malignant conditions that may lead to SVCS. ...

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Background: Superior Vena Cava Syndrome (SVCS) is an increase in the upper body venous pressure caused by obstruction or stenosis of the Superior Vena Cava (SVC), which causes congestion of the head, face, upper limbs, neck and upper body. The main etiology has been reported to be tumors of the lungs or mediastinum, and aortic aneurysm.
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Superior vena cava syndrome (SVCS) is usually caused by a malignancy or the presence of an intravascular device in a central vein. A 74-year-old male with a history of a superior vena cava (SVC) stent underwent embolisation of a brain arterio-venous malformation through the right meningeal artery with liquid Onyx. Two weeks later he presented with...
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We present 3 cases of superior vena cava (SVC) syndrome following percutaneous right ventricular assist device (RVAD) placement. Each case underscores the importance of early recognition of SVC syndrome in patients with percutaneous RVAD insertion via the internal jugular vein and calls for heightened awareness of device-associated complications. (...

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... Furthermore, reported complications often arising in patients with SVCS include altered respiration, which leads to breathing difficulties and pleural effusion. 5 Persistent SVCS may also impede the efficiency of central venous access for post-transplant care and monitoring, potentially leading to further complications or delays in management. 6 These complications highlight the importance of a thorough assessment and a multidisciplinary approach involving cardiologists, radiologists, and surgeons to ensure timely diagnosis and appropriate management of this rare but potentially severe complication. ...
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Superior vena cava syndrome (SVCS) results from the obstruction or narrowing of the superior vena cava, causing venous congestion and various symptoms such as facial and upper limb swelling, shortness of breath, chest pain, coughing, and, in severe cases, dizziness and headache. The primary treatment for SVCS is balloon angioplasty with endovascular stenting. Post-procedural complications are influenced by factors such as SVCS aetiology, comorbidities, and the presence of arteriovenous fistulas. This review examined eight clinical studies to assess the effectiveness of percutaneous endovascular stenting and associated complications, focusing on improving patient prognosis. The research, conducted through internet search engines and reputable databases, revealed that percutaneous endovascular stenting demonstrated efficacy ranging from 95–100% in addressing SVCS. Common complications post-procedure included SVC narrowing recurrence, airway constriction, and mortality, often linked to malignancy. The findings emphasise the need to refine therapeutic approaches, especially in addressing the root cause of SVCS, which is frequently malignancy. Consequently, implementing additional protocols to reduce the risk of SVCS development is crucial. This comprehensive review provides insights into the effectiveness of endovascular stenting in treating SVCS, highlighting the importance of tailored approaches and ongoing efforts to enhance patient outcomes.
... Sindrom vena kava superior (SVKS) merupakan kumpulan berbagai tanda dan gejala klinis akibat kompresi eksternal atau obstruksi intrinsik vena kava superior (VKS) itu sendiri atau sambungan cavoatrium superior yang mengakibatkan aliran darah berkurang. 1 Sindrom vena kava superior pertama kali dijelaskan oleh seorang dokter Skotlandia bernama William Hunter tahun 1957 pada seorang pasien dengan aneurisma aorta karena penyakit sifilis. [2][3][4][5] Schechter mempelajari serangkaian kasus dengan SVKS pada tahun 1950-an kemudian mengaitkan aneurisma sifilis dan mediastinitis tuberkular sebagai penyebab di hampir setengah kasus SVKS tersebut. ...
... Sebuah tinjauan literatur retrospektif pada 1986 pasien dengan SVKS antara tahun 1934 hingga 1984 melaporkan hanya 1 kematian yang dapat dikaitkan langsung dengan obstruksi VKS. 8 Tinjauan pustaka ini akan menjelaskan mengenai patofisiologi, etiologi, diagnosis dan tatalaksana SVKS dengan tujuan agar dapat dilakukan intervensi tepat waktu dan efektif untuk mengobati penyebab dari sindrom ini sehingga dapat meringankan gejala secara signifikan dan meningkatkan kualitas hidup pasien dengan SVKS. [1][2][3]12 Obstruksi akut VKS dapat menyebabkan peningkatan tekanan vena intrakranial yang signifikan menjadi 40 mmHg dari kisaran normal 2-8 mmHg. Peningkatan tekanan vena selain dapat menyebabkan edema pada tubuh bagian atas juga dapat menyebabkan penyempitan saluran pernapasan bagian atas akibat edema hidung dan laring. ...
... Obstruksi VKS dapat disebabkan oleh kompresi eksternal atau obstruksi intrinsik VKS itu sendiri atau sambungan cavo-atrium superior. [1][2][3] Kompresi eksternal VKS dapat disebabkan oleh proses neoplastik seperti tumor yang timbul di bronkus lobus kanan utama atau oleh limfadenopati mediastinum dengan ukuran besar (subkarinal, perihilar dan paratrakeal) dan massa di mediastinum medial atau anterior, biasanya di sisi kanan garis tengah (midline). 1,2,13,14 Keganasan yang paling umum menjadi penyebab SVKS antara lain kanker paru kelompok bukan sel kecil (KPKBSK) (22-57%), kanker paru kelompok sel kecil (KPKSK) (10-39%) dan limfoma (1-27%). 2 Pada sebuah studi oleh Perez-Soler dkk, 36 dari 915 pasien dengan limfoma non-hodgkin menunjukkan SVKS, dan tipe histologis yang terkait adalah sel besar (23 pasien), limfoblastik (12 pasien) dan sel besar folikuler (1 pasien). ...
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Superior Vena Cava Syndrome (SVCS) includes various clinical signs and symptoms due to external compression and intrinsic obstruction of superior vena cava (SVC) itself or the superior cavo-atrial junction that cause reducing of blood flow. Infection becomes main etiology in previous decades but development in antibiotics treatment and improvement of socio-economyc condition made incidence of SVCS due to infection decreased. SVCS due to malignancy recently reach 60-90% of overall cases. 78-85% of SVC obstructions due to malignancy are caused by lung cancer and 80% of it is right lesion. Historically SVCS is considered as an oncology emergency condition and become one of a few indications for palliative radiotherapy. This literature review will explain the pathophysiology, etiology, diagnosis and management of SVCS with the aim of providing timely and effective intervention to treat the causes of this syndrome so that it can significantly relieve symptoms and improve the quality of life of patients with SVCS.
... 1 However, less common malignant causes include entities such as lymphoma or thymoma. 3 The development of malignant SVCS is considered a poor prognostic indicator. Additional reported causes of SVCS include infections such as tuberculosis and syphilis, iatrogenic causes including malpositioned central venous catheters, thrombosis within the SVC and postradiation treatment fibrosis. ...
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An adult woman with a prior history of treated non-Hodgkin’s lymphoma presented for screening mammography, which incidentally demonstrated dilated veins throughout the bilateral breasts. Concern for a superior vena cava stenosis or obstruction was raised despite the patient being asymptomatic; the patient underwent further imaging with chest CT, which revealed focal stenosis of the superior vena cava, attributed to fibrosis secondary to prior radiation therapy. Superior vena cava syndrome (SVCS), the spectrum of disease caused by superior vena cava narrowing or obstruction, requires prompt investigation given its association with intrathoracic malignancy, primary lung cancer and poor outcomes. This report explores the benign and malignant causes, signs and symptoms, preferred investigations, and treatment of SVCS. This case highlights the potential importance of screening mammography in revealing unexpected ancillary diagnoses, especially in high-risk patients.
... This is typically delivered to 5 to 10 fractions for patients with metastatic disease. For patients with non-metastatic disease, a limited number of these hypofractionated treatments can be delivered before proceeding with conventional 2 Gy fractions for the remainder of the course in order to reach a total dose with curative intent per current guidelines (27,28). Notably, however, no randomized trials comparing different dose fractionation regimens for SVC syndrome relief with radiation therapy have been conducted to date, and as a result no consensus currently exists on the ideal fractionation scheme (7,29). ...
... Notably, however, no randomized trials comparing different dose fractionation regimens for SVC syndrome relief with radiation therapy have been conducted to date, and as a result no consensus currently exists on the ideal fractionation scheme (7,29). Immunotherapy, either alone or in combination with chemotherapy, may also be part of a first-line or subsequent management option for SVC syndrome, but current data on the use of immunotherapy specifically for SVC syndrome are sparse (27). ...
... Superior vena cava (SVC) syndrome is a collection of signs and symptoms arising from the hindrance of blood circulation in the SVC [1]. Various factors, both malignant and non-malignant, can contribute to SVC syndrome [2]. Notably, lung cancer and non-Hodgkin's lymphoma account for approximately 90% of cases associated with SVC syndrome [3]. ...
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Background Superior vena cava (SVC) syndrome is an urgent condition arising from restricted blood flow through the SVC, often linked to factors like malignancy, thrombosis, or infections. Typically, confirmation of the diagnosis involves computed tomography. However, many patients experience respiratory distress and cannot lie supine. Given the increasing integration of point-of-care ultrasound in emergency medicine, it is important to be familiar with findings that are suggestive of this important condition. Case report In this case report, we highlight a young patient presenting to the emergency department with superior vena cava syndrome symptoms, successfully diagnosed using point-of-care ultrasound. Conclusion This case highlights the utility of point-of-care ultrasound based diagnosis of SVC syndrome and upper arm deep venous thrombosis in a patient with underlying malignancy which ultimately led to early involvement of relevant speciality for initiation of treatment.
... Although lung cancer has been described as the main cause of SCVS due to solid tumors, other etiologies have also been described, such as thymoma, primary germ cell neoplasms, mesothelioma, and mediastinal lymph node metastases from other neoplasms such as breast cancer [4,14,15] Rice and cols, described that bronchogenic carcinoma accounted for 46 % of the etiology of SVCS [13]. In our series lung cancer accounted for 38.9 % of cases that underwent endovascular stenting. ...
Article
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Background Malignant Superior Vena Cava Syndrome (SVCS) corresponds to the clinical manifestations due to the restriction of venous return to the right atrium secondary to obstruction of the superior vena cava and/or its main tributaries for a tumor. Endovascular management has proven to be safe, effective and cause a fast symptomatic relief in patients with SVCS. There is limited evidence in factors associated with outcomes in malignant setting for this procedure. Materials and methods An analytical retrospective study was conducted and included patients that underwent endovascular management for malignant SVCS at the National Cancer Institute of Colombia between May 2016 and May 2021. Clinical and technical variables were analyzed to found associations with outcomes in these patients. Results 54 patients were analyzed. Successful procedure rate was 94.4 %. At 10 months, the OS of the entire cohort of patients was 25 %. Patients with breast or lung cancer (P = 0.031), unsuccessful procedure (P = 0.011), and also with short time of symptoms to the date of the endovascular procedure (P = 0.027) had worse OS. Multivariate analysis showed that lung cancer [HR = 2.55, 95%IC:(1.21–5.36)] and left internal jugular vein or left Innominate vein distal stent attachment [HR = 3.27, 95%IC:(1.31–8.15)] were independent factors for worst OS. Conclusions Based in the high success rate of the endovascular management and the better outcome in patients with early and successful procedure, this procedure should be considered as part of the multimodal treatment in patients with SVCS independent of the clinical scenario and the oncological diagnosis.
... Until the first development of endovascular stenting for SVCS around 35 years ago (8), radiotherapy (RT) was the most commonly used treatment for malignant SVCS, while chemotherapy has been an option for highly chemosensitive tumours (2). Concurrent chemoradiation may produce a higher response rate but is associated with greater toxicities (9). Surgical resection of the tumour followed by venous bypass or reconstruction is theoretically an option (10), but it is rarely performed in patients with stage IV malignancies, and it is not the most definitive oncologic therapy in light of the histologies most associated with SVCS. ...
Article
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Malignant superior vena cava syndrome (SVCS) is no longer considered a medical emergency in most cases because it rarely leads to life-threatening complications. However, it results in disturbing symptoms that can significantly affect patients' quality of life. Treating this condition effectively while minimising treatment-related morbidity is of increasing importance as cancer patients are living longer from advances in oncological treatments. This clinical practice review discusses the implications of these advances on the decision to consider stenting as the initial treatment for SVCS. Stenting is increasingly popular as it provides quick symptomatic relief with low rates of complications. Systemic treatments have evolved in the past two decades with the development of immunotherapy and targeted therapies that have different response patterns compared to conventional chemotherapy. Furthermore, major changes have also been seen in radiotherapy techniques that allow treatments to better conform to targets while sparing normal tissues. These advances have changed practice patterns for stent placement in SVCS patients in both the localised and metastatic settings. Prospective studies using standardised patient-reported outcome tools are needed to determine the optimal treatment sequence for SVCS patients, as current recommendations are mainly based on retrospective single-arm studies. An individualized approach with multidisciplinary input is therefore important to optimize patient outcomes before more robust evidence is available.
... Superior vena cava (SVC) obstruction is generally a late sign as the tumor will have taken up a large portion of the mediastinum. Superior vena cava syndrome is highly suggestive of thoracic malignancy and is generally associated with poor prognosis [2]. Management of SVC syndrome is determined largely by the underlying pathology and can include chemo/radiotherapy as well as stenting or surgical bypass in selected cases [2]. ...
... Superior vena cava syndrome is highly suggestive of thoracic malignancy and is generally associated with poor prognosis [2]. Management of SVC syndrome is determined largely by the underlying pathology and can include chemo/radiotherapy as well as stenting or surgical bypass in selected cases [2]. In the above case a mediastinal liposarcoma resulted in SVC syndrome and after surgical resection, the symptoms rapidly subsided. ...
... En cuestión de horas la presión venosa aumenta por medio de venas colaterales a la vena ácigos, paravertebrales, mamarias internas e intercostales, por nombrar algunas, las cuales se dilatan, mecanismo que les lleva algunas semanas. Este mecanismo compensatorio se puede ver limitado si la compresión de la VCS se encuentra por debajo de la vena ácigos, empeorando las manifestaciones clínicas asociadas a este síndrome (8). ...
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El síndrome de la vena cava superior es un conjunto de signos y síntomas que resultan de la compresión u oclusión de la vena cava superior, más comúnmente secundario a procesos neoplásicos. Las imágenes juegan un papel importante en el diagnóstico e identificación de este síndrome. Se presentan el caso y las imágenes de un paciente con síndrome de vena cava superior secundario a adenopatía mediastinal, relacionado con posible metástasis de carcinoma uroepitelial de vejiga.
... Some manifestations are less common, such as signs and symptoms of distant metastases or paraneoplastic syndromes. The presence of any of these manifestations in a patient with suspected lung cancer should prompt further testing (Chute et al., 1985;Hirshberg et al., 1997;Lepper et al., 2011). ...
Chapter
Lung cancer is the most common cause of cancer death around the world with over 1.80 million deaths per year. About 60% of cases have been diagnosed at an advanced stage, and the 5-year survival rate falls between 10% and 20%. Since lung cancer survival is largely determined by the stage of the disease at diagnosis, developing a reliable and effective screening method for early diagnosis has been a long-term goal of lung cancer care. Through effective screening, lung cancer can be diagnosed and treated earlier, resulting in reduced morbidity and mortality. Lung cancer screening would be more accurate and efficient by incorporating an automated intelligent system that matches or surpasses the diagnostic capabilities of human experts. The use of Artificial Intelligence (AI) for lung cancer screening can yield significant benefits. Application of AI in various healthcare settings is currently proving to be a success due to its ability to emulate human cognition in different areas such as analysis, interpretation, and comprehension of complex data sets using complex algorithms and software. By applying AI to imaging diagnostics, radiologists will be less burdened, and screening for lung cancer will be more sensitive, which, in turn, will reduce the morbidity and mortality associated with lung cancer. There has been a significant amount of research using AI to develop tools for detecting and classifying lung cancer using patient data with the goal of improving outcomes. In this article, we provide an overview of the application of AI in lung cancer for improving nodule detection and classification, as well as the challenges that remain to be overcome.A comprehensive search was performed in PubMed, Google Scholar, Web of Science, IEEEXplore, and the Cochrane Database for studies published between 2011 and 2021. The search terms were “lung cancer,” “Artificial Intelligence,” “machine learning,” “deep learning,” “screening,” “detection,” and “classification.” After excluding animal studies, relevant articles reporting AI models for the analysis of chest CT scan images in the case of lung cancer detection were discussed.Based on the results of this study, there is no doubt that AI-implemented neural networks will be the future of lung cancer screening and diagnosis. In CT images, these neural networks offer great promise in detecting lung nodules with a diameter of just 3 mm. Thus, neural networks can detect lung nodules in their earliest stages, thereby improving the survival rate of the patients.In this chapter, we discuss the role of AI, especially neural networks, in lung cancer as it relates to improving screening, diagnosis, and patient’s outcomes. There is a need for future research that will investigate deep neural networks in a clinical and biological context, as well as validate these networks in prospective data.KeywordsArtificial Intelligence (AI)Computer-aided detection (CAD)Convolutional neural networks (CNNs)DiagnosisLung cancerLung cancer detectionMachine learning (ML)Pulmonary nodule