[An unusual presentation of superior vena cava syndrome]. / Une présentation inhabituelle de syndrome de la veine cave supérieure.

Superior vena cava syndrome (SVCS) is a clinical picture resulting from the obstruction of superior vena cava. SVCS usually causes symptoms and signs related to the head, the neck, the upper extremities and the upper thorax. We report a case of unusual clinical presentation of SVCS in a 25-year-old male patient who suffered from lymphoblastic lymphoma in childhood. Clinicians should be aware of abdominal wall presentations of SVCS especially in patients having undergone central venous catheterization, even years before. SVCS could be an emergency situation, requiring specific treatment or contraindicating surgical procedures.

Le syndrome de la veine cave supérieure (SVCS) est une entité nosologique résultant de l'obstruction de la veine cave supérieure. Un SVCS comporte habituellement des symptômes et des signes cliniques localisés à la région cranio-cervicale, aux membres supérieurs et au niveau thoracique supérieur. Nous rapportons un cas de présentation clinique inhabituelle de SVCS chez un patient de 25 ans aux antécédents de lymphome lymphoblastique dans l'enfance. En pratique clinique, il est important de garder à l'esprit l'existence de présentations inhabituelles pariétales abdominales de SVCS, en particulier chez les patients ayant subi un cathétérisme veineux central, même plusieurs années auparavant. Un SVCS peut nécessiter un traitement spécifique, parfois en urgence et contre-indiquer certaines interventions chirurgicales.

Clinical Features and Therapeutic Outcomes Comparing Primary Mediastinal Large B-cell Lymphoma to Mediastinal Hodgkin Disease.

Currently, there is limited data available comparing Primary Mediastinal Large B-cell Lymphoma (PMBL) and mediastinal Hodgkin disease, nodular sclerosis type (HDNS). This is a retrospective cohort study that compares the clinical features, histology through immunohistochemistry (IHC) and treatment outcomes of 19 cases of PMBL and 39 cases of HDNS diagnosed over 13 years at a single institution in San Juan, PR. Superior Vena Cava syndrome (SVCS) and elevated Lactate Dehydrogenase (LDH) levels were more frequently seen in the PMBL cohort. At the median follow-up visit, of 74 months, no significant difference was seen in overall survival or progression free survival between PMBL and HDNS. Almost all of the relapses in the PMBL group occurred within 12 months of diagnosis. Our data suggests that PMBL and HDNS differ in their clinical presentation and have a favorable prognosis.

Challenge Accepted: Lead Extraction in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Occlusion.

Persistent left superior vena cava (PLSVC) is an anatomic variant that is relatively uncommon in the general population. Lead extraction through PLSVC is extremely rare. Due to unusual anatomy, the procedure carries challenges that require special considerations and careful planning. The authors report a case of lead extraction through a PLSVC with occluded right superior vena cava and highlight the challenges and outcomes of the procedure.

Incidentally diagnosed superior vena cava narrowing prompted by screening mammography.

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.

Lead Extraction and Baffle Stenting in a Patient with Transposition of the Great Arteries.

This case report discusses a 42-year-old male with dextro-transposition of the great arteries (D-TGA) status post Mustard repair and sick sinus syndrome status post dual-chamber pacemaker implant, who developed symptomatic superior vena cava (SVC) baffle stenosis. He was treated with a combined pacemaker extraction and subsequent SVC baffle stenting. The case highlights the complexities of treating SVC baffle stenosis in the presence of cardiac implantable devices and demonstrates the efficacy of this combined approach. Furthermore, the authors delve into the intricacies of D-TGA, its surgical history, and the long-term complications associated with atrial switch procedures.

Safety and efficacy of large-bore uncovered stents for treating malignant superior vena cava syndrome.

PURPOSE: To investigate the safety and efficacy of large-bore uncovered stents for treating malignant superior vena cava syndrome. METHODS: This retrospective study included 115 patients (89 men, 26 women; mean age 63.2 years; range 21-83 years) who underwent endovascular large-bore (≥18 mm in diameter) uncovered stent placement between August 2015 and July 2022. One patient was lost to follow-up. Therefore, 114 patients were available for follow-up. RESULTS: Stent placement was technically successful in all 115 patients. Minor procedure-related complications occurred in nine (7.8 %) patients. One hundred eight (93.9 %) patients experienced complete or marked symptomatic relief (Kishi score ≤ 2) at a mean of 3 days after procedure. The cumulative stent patency rates were 98.2 %, 95 %, 93.7 %, 91.5 %, 83.5 %, and 83.5 % at 1, 3, 6, 12, 18, and 24 months, respectively. Stent occlusion occurred in ten (8.8 %) of 114 patients at a mean of 215 days (range 1-732 days) due to thrombosis (n = 7) and tumor ingrowth (n = 3). Stent occlusion did not occur in 21 patients who underwent subsequent central venous catheter insertion. The median patient survival time was 159 days (95 % confidence interval 102-216 days). Univariate and multivariate Cox regression analysis revealed adjuvant anticancer treatment (p = 0.001) and tumor response (p < 0.001) as independent predictors of patient survival. CONCLUSIONS: Endovascular placement of large-bore uncovered stents was a safe and effective treatment for malignant superior vena cava syndrome. Large-bore stent placement can effectively prevent stent occlusion by tumor ingrowth in most cases, and it can provide a sufficient diameter for subsequent insertion of central venous catheters.

Safety and Effectiveness of Abre Self-Expanding Venous Stent for Treatment of Superior Vena Cava Syndrome.

PURPOSE: Superior vena cava (SVC) syndrome is a constellation of symptoms that results from partial or complete SVC obstruction. Endovascular SVC stenting is an effective treatment for SVC syndrome with rapid clinical efficacy and low risk of complications. In this study, we assess the technical and clinical outcomes of a cohort of patients with SVC syndrome treated with the AbreTM self-expanding venous stent (Medtronic, Inc, Minneapolis, MN, USA). METHODS: An institutional database was used to retrospectively identify patients with SVC syndrome treated with AbreTM venous self-expanding stent placement between 2021-2023. Patient demographic data, technical outcomes, treatment effectiveness, and adverse events were obtained from the electronic medical record. Nineteen patients (mean age 58.6) were included in the study. Thirteen interventions were performed for malignant compression of the SVC, 5 for central venous catheter-related SVC stenosis, and 1 for HD fistula-related SVC stenosis refractory to angioplasty. RESULTS: Primary patency was achieved in 93% of patients (17/19). Two patients (7%) required re-intervention with thrombolysis and angioplasty within 30 days post-stenting. Mean duration of clinical and imaging follow-up were 228.7 ± 52.7 and 258.7 ± 62.1 days, respectively. All patients with clinical follow-up experienced significant improvement in clinical symptoms post-intervention. No stent related complications were identified post-intervention. CONCLUSIONS: Treatment of SVC syndrome with the AbreTM self-expanding venous stent has high rates of technical and clinical success. No complications related to stent placement were identified in this study.

Management of malignant superior vena cava syndrome.

Superior vena cava (SVC) syndrome occurs due to obstructed blood flow through the SVC. It can present clinically on a spectrum, between asymptomatic and life-threatening emergency. Patients commonly report a feeling of fullness in the head, facial, neck and upper extremity edema, and dyspnea. On imaging, patients commonly have superior mediastinal widening and pleural effusion. The majority of cases are due to malignant causes, with non-small cell lung cancer, small cell lung cancer, and lymphoma the most commonly associated malignancies. When evaluating patients, a complete staging workup is recommended, as it will determine whether treatment should be definitive/curative or palliative in intent. If the patient requires urgent treatment of venous obstruction, such as in the cases of acute central airway obstruction, severe laryngeal edema and/or coma from cerebral edema, direct opening of the occlusion by endovascular stenting and angioplasty with thrombolysis should be considered. Such an approach can provide immediate relief of symptoms before cancer-specific therapies are initiated. The intent of treatment is to manage the underlying disease while palliating symptoms. Treatment approaches most commonly employ chemotherapy and/or radiation therapy depending on the primary histology. Mildly hypofractionated radiation regimens are most commonly employed and achieve high rates of symptomatic responses generally within 2 weeks of initiating therapy.

Hemorrhagic pericardial tamponade in a hemodialysis patient with catheter-related superior vena cava syndrome: a case report.

BACKGROUND: Iatrogenic complications of endovascular treatment for central venous stenosis have not yet been reported. Here we present a case of a patient on maintenance hemodialysis who developed catheter-related superior vena cava syndrome and subsequently suffered from hemorrhagic pericardial tamponade after undergoing percutaneous transluminal angioplasty and stenting. CASE PRESENTATION: A 72-year-old male patient presented with uremia, and had been receiving maintenance hemodialysis for the past five years. The patient initially presented with dysfunction of the dialysis catheter (a cuffed tunneled double-lumen catheter in the right internal jugular vein). Imaging examination revealed a segmental occlusion of the superior vena cava stretching from the distal end of the dialysis catheter up to right atrium entrance, apparent compensatory dilatation of the azygos vein, and abundant subcutaneous collaterals. The patient underwent percutaneous transluminal balloon dilatation and stenting (covered stent) of the superior vena cava in the Cath Lab. During the procedure, with forceful advancement of the guidewire, it was observed to progress for a distance before a "smoke" appeared, and an outward spillage of contrast agent was visible, which suggested a possible vessel puncture leading into the mediastinum. Unfortunately, postoperative hemorrhagic pericardial tamponade occurred and the patient developed cardiogenic shock. He experienced symptoms included chest tightness and breath shortness with a recorded blood pressure of 84/60mmHg. After draining 600 ml of bloody fluid through pericardiocentesis, the patient's symptoms alleviated and his condition improved. CONCLUSIONS: The case emphasizes the need for increased attention to iatrogenic endovascular injuries during catheter placement and endovascular treatment, such as causing pericardial hemorrhage leading to cardiac tamponade.

Surgical resection of an intraluminal tumor in the azygos vein with an unknown primary site causing superior vena cava syndrome.

Intraluminal tumor in the azygos vein is a rare disease that can cause superior vena cava (SVC) syndrome. Radiotherapy and endovascular stenting with or without chemotherapy are reported to have a high clinical success rate for the management of SVC syndrome with malignancy, but a poor survival rate. Here, we report a 69-year-old man who presented with swelling of the face and upper extremities, who was diagnosed with SVC syndrome caused by an intraluminal tumor in the azygos vein. Enhanced chest computed tomography revealed an intraluminal mass with a filling defect from the azygos vein to the SVC, with no extravascular extension or dissemination of the primary tumor. Surgical resection of the mass en bloc with the azygos vein and SVC reconstruction was performed. A poorly differentiated carcinoma was diagnosed on postoperative pathological evaluation. Twelve months after resection, the patient was well with no signs of recurrent disease. This case highlights that surgical resection should be considered as a treatment of choice for the management of SVC syndrome caused by an intraluminal malignancy in the azygos vein.

Successful resection of a primary angiosarcoma of the azygos vein: A case report.

A 63-year-old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video-assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor-free 24 months after surgery. In addition, contrast-enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.

Emergency radiation therapy in modern-day practice: Now or never, or never again ?

Radiation therapy plays a fundamental role in oncological emergencies such as superior vena cava syndrome (SVCS) and metastatic epidural spinal cord compression (MESCC). These are two examples of critical complications of metastatic cancer in terms of pain and functional impact (respiratory and/or neurological). The aim of this review is to explore the current indications, treatment options and outcomes for emergency radiotherapy regarding to these complications.Regarding SVCS, studies are mostly retrospective and unanimously demonstrated a beneficial effect of radiotherapy on symptom relief. Spinal cord compression remains an indication for urgent radiotherapy, and should be combined with surgery when possible. The innovative stereotactic body radiotherapy (SBRT) showed promising results, however this technique requires small volumes and more time preparation and therefore is often unsuitable for SVCS and MESCC emergencies.This review concluded that radiotherapy has a central role to play within a multimodal approach for SVCS and MESCC treatment. Further prospective studies are needed to confirm the effectiveness of radiation and establish the criteria for selecting patients to benefit from this treatment option.