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.

[Non-infectious respiratory emergencies in patients with cancer]. / Urgences respiratoires (non infectieuses) du patient d'onco-hématologie.

Patients with a solid tumor or hematologic malignancy are often addressed to emergency units for an acute respiratory complication associated with the underlying cancer or secondary to treatments. The current article is part of a thematic series: "Intensive care and emergencies in solid tumours and blood cancer patients" and will develop the following points: (1) malignant proximal airway obstruction and, more specifically, the role of therapeutic bronchoscopy; (2) superior vena cava syndrome by tumor compression and/or secondary to thrombosis (diagnosis, local and systemic treatments); (3) cancer-related pulmonary embolism (incidence, indications for low-molecular weight heparins and direct oral anticoagulants). Other respiratory emergencies will be dealt in the other articles of this series.

An updated narrative review on the management of the most common oncological and hematological emergencies.

Oncological emergencies are defined as an acute life-threatening event in a patient with a tumor occurring as part of their complex treatment regimen or secondarily to their underlying malignancy. These events can occur at any time from the initial diagnosis of their cancer to end-stage disease. These oncological emergencies are broadly classified into four major categories; metabolic, structural, hematological and treatment-related causes; and can be encountered in any clinical setting, ranging from primary care physician and emergency department visits to a variety of subspecialty environments. This study aims to cover an in-depth review of the underlying pathogenesis, clinical presentation, and updated management protocol of most common emergencies belonging to the above-mentioned categories. An all-language literature search was conducted on 15th October 2021 and 10th March 2022, limited to 5 years on PubMed database using the following search strings: oncological emergencies, malignant spinal cord compression, febrile neutropenia, hyperviscosity syndrome, superior vena cava syndrome, immune related adverse events, tumor lysis syndrome, hypercalcemia of malignancy, corrected calcium, malignant pericardial effusion and chemotherapy extravasation.

[Analysis of 42 cases of childhood superior vena cava syndrome associated with mediastinal malignancy].

Objective: To summarize the clinical features, management and outcome of superior vena cava syndrome (SVCS) associated with mediastinal malignancy in children. Methods: Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children's Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively. The clinical manifestations, pathological diagnosis, disease diagnosis process, and prognosis were summarized. Results: Among 42 children of SVCS associated with mediastinal malignancy, there were 31 males and 11 females. The age at diagnosis was 8.5 (1.9, 14.9) years. Cough and wheezing (33 cases, 79%), orthopnea (19 cases, 45%) and facial edema (18 cases, 43%) occurred most commonly. T-cell lymphoblastic lymphoma (T-LBL) was the most frequent pathological diagnosis (25 cases, 60%), followed by T-cell acute lymphoblastic leukemia (T-ALL) (7 cases, 17%), anaplastic large cell lymphoma (4 cases, 10%) and diffuse large B-cell lymphoma (2 cases, 5%), peripheral T-lymphoma, Hodgkin lymphoma, Ewing's sarcoma and germ cell tumor (1 case each). Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases, peripheral lymph node biopsy in 6 cases, and mediastinal biopsy in 22 cases. Twenty-seven cases (64%) had local anesthesia. Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia. Of the 42 cases, 27 cases had sustained remission, 1 case survived with second-line therapy after recurrence, and 14 cases died (2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease). The follow-up time was 36.7 (1.2, 76.1) months for 27 cases in continuous complete remission. The 3-year overall survival (OS) and events free survival (EFS) rates of 42 children were 59% (95%CI 44%-79%) and 58% (95%CI 44%-77%) respectively. Conclusions: SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality. The most common primary disease is T-LBL. The most common clinical symptoms and signs are cough, wheezing, orthopnea and facial edema. Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.

Not Feeling Swell: Superior Vena Cava (SVC) Syndrome Falsely Attributed to COVID-19 Vaccine Reaction.

BACKGROUND: The mass immunization campaign against Coronavirus disease 2019 (COVID-19) has resulted in more patients presenting to the emergency department (ED) with concern for a vaccine reaction. CASE REPORT: A 68-year-old man presented to the ED reporting an allergic reaction to the COVID-19 vaccine. He initially noted swelling of his face, neck, and right arm after receiving the first dose of the vaccine. After his second dose of the vaccine, the swelling became more pronounced and prompted him to seek care. On examination, he had fullness of the neck and engorgement of the left external jugular vein, which were exacerbated when the patient raised his arms above his head, consistent with Pemberton's sign. Apart from the swelling of the head and neck, there were no other findings consistent with an allergic reaction. The presence of Pemberton's sign prompted a computed tomography scan of the chest with contrast, which revealed a paratracheal mass measuring 4.5 × 2.0 cm with marked narrowing of the superior vena cava (SVC). The patient was admitted to the hospital for SVC syndrome, and further workup revealed a non-small cell lung cancer. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Patients may misattribute their symptoms to a COVID vaccine reaction when they are, in fact, experiencing a more serious underlying disease. This case highlights the importance of a thorough physical examination and maintaining a broad differential diagnosis. In this case, the presence of Pemberton's sign raised suspicion for SVC syndrome, and prompted further workup.

Carcinoma male breast with tracheal and endobronchial metastasis, masquerading as nonsmall-cell lung cancer, presenting with superior vena cava obstruction - A rare case report.

Cancer male breast is not very common. It comprises <1% of all breast cancers. Primary sites of metastasis are bone, brain, lung, and liver. Endobronchial and tracheal metastasis is very rare. To the best of our information, this is the first case of male breast carcinoma with upfront tracheal and endobronchial metastasis which presented as Superior vena cava obstruction (SVCO), initially evaluated on the lines of metastatic nonsmall-cell lung cancer. A 60-year-old gentleman presented with shortness of breath and features of SVCO. On primary evaluation, he was thought to be a case of nonsmall-cell lung cancer. Later on, it was confirmed to be carcinoma male breast with endobronchial and tracheal metastasis, which was reconfirmed with biopsy. The case we came across had symptoms associated with endobronchial metastases from primary extrapulmonary tumor and which was later found out to be breast. Treatment options are very different from lung primary and thus, we should be aware of the unusual disease presentation.

A huge Ewing's sarcoma of the rib presenting with superior vena cava syndrome and dysphagia.

A 24-year-old male patient was admitted to our center complaining of dizziness (superior vena cava syndrome [SVCS]), dysphagia and pain in the right chest wall. At the initial diagnosis, the patient had been found to have an irregular shaped 35 × 30 × 27 cm mass in the right side of his chest. On November 12, 2019, this patient received surgery in our center. The right sixth rib and the tumor were completely removed (R0), while preserving all the lung tissue and other organs in the chest. The patient recovered well after surgery, and his right lung was fully expanded.

Isolated Myeloid Sarcoma and Intracardiac Thrombus Resulting in Superior Mediastinal Syndrome.

Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis.

Urgencias en cuidado paliativo oncológico / Emergencies in palliative oncological care

El paciente oncológico de cuidados paliativos puede presentar una variedad importante de condiciones clínicas que producen sufrimiento y disminución en la calidad de vida; esto se presenta como un reto para el clínico en la identificación y correcto abordaje de los pacientes. El dolor se ha considerado por años como el síntoma cardinal a tratar en el paciente oncológico, donde se deben considerar sus condicionantes fisiopatológicos, la farmacología de las intervenciones, los posibles efectos secundarios y los condicionantes familiares, sociales y personales del dolor, pero a pesar de su relevancia, no es el único síntoma, estando acompañado de un abanico de patologías, como las afectaciones gastrointestinales, pulmonares, vasculares, hematológicas y neurológicas, que favorecen la pérdida de capacidad del paciente y, en muchas ocasiones, la muerte. Se realizó una revisión actualizada en bases de datos como EMBASE, PUBMED, SCIELO, además de la revisión de guías de asociaciones internacionales con el objetivo de acercar a todos los médicos, sin distinguir su especialidad o área de trabajo, al abordaje y manejo del paciente oncológico en cuidado paliativo, favoreciendo la sensibilización con estas patologías y la importancia en el curso de vida de los pacientes.

The palliative care cancer patient can present a significant variety of clinical conditions that produce suffering and a decrease in the quality of life. This is a challenge for the clinician in the identification and correct approach of patients. Pain has been considered for years as the cardinal symptom to be treated in cancer patients, where its pathophysiological factors, the pharmacology of the interventions, possible side effects and the family, social and personal conditions of pain must be considered, but despite its relevance is not the onset of symptoms and is accompanied by a range of pathologies such as gastrointestinal, pulmonary, vascular, hematological and neurological affectations that favor the loss of capacity of the patient and in many cases death. An updated review was carried out in databases such as EMBASE, PUBMED, SCIELO in addition to the revision of guides from international associations with the aim of bringing all doctors without distinguishing their specialty or area of work to the approach and management of cancer patients in palliative care favoring awareness of these pathologies and their importance in the life course of patients.

Hodgkin Lymphoma-Associated Superior Vena Cava Syndrome: A Case Report and Review of the Literature.

BACKGROUND Hodgkin lymphoma (HL) is a relatively rare etiology of superior vena cava (SVC) syndrome, with only 24 cases reported in the literature. The characteristics, management, and prognosis of HL-associated SVC syndrome remain unclear. This case report describes nodular sclerosis classical HL and the associated clinical manifestations presenting as SVC syndrome in a middle-aged patient, and it summarizes the characteristics of HL-associated SVC syndrome. CASE REPORT In this case report, we present a 53-year-old Hispanic man with progressively worsening dyspnea, dry cough, facial and neck edema, and dysphagia. SVC syndrome was diagnosed, and pathology revealed nodular sclerosis classical HL. The patient was treated with doxorubicin, bleomycin, vinblastine, and dacarbazine. SVC syndrome improved, and repeated imaging showed that the lymphoma had decreased in size and had become metabolically inactive. CONCLUSIONS We reviewed the characteristics, management, and prognosis of HL-associated SVC syndrome, which may indicate more advanced and recurrent progression in patients with HL. This possibility suggests that physicians should provide urgent diagnosis and closer follow-up, and more aggressive therapies may be needed because of the high risk of recurrence. Therapy may induce late-onset SVC syndrome in patients with HL.

Primary angiosarcoma of superior vena cava: an unexpected diagnosis after an oncological emergency.

Angiosarcoma (AS) is a rare malignant tumour representing 1%-2% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide. We report a 69-year-old woman with neck discomfort, headache and dyspnoea for 3 months. CT angiography showed thrombosis in SVC and brachiocephalic veins resulting in an SVC syndrome. The patient began anticoagulant therapy and underwent balloon angioplasty with clinical improvement. Additionally, a positron emission tomography scan confirmed the presence of a mediastinal mass involving the SVC locally. The tumour was excised and a prosthesis was placed on the SVC. Histology revealed a heterogeneous tumour matrix, either myxoid and composed by fusiform cells with vimentin, homogeneous CD31 and a 30% Ki67 immunoexpression, supporting the diagnosis of an AS. Due to multiple complications, the patient never started chemotherapy, and after tumour recurrence, she died within 5 months after diagnosis.

Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome.

Immunoglobulin G4 (IgG4)-related disease was first identified as a systemic condition in 2003 when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Its peak incidence occurs in the fifth or sixth decades of life. Isolated extraaortic mediastinal involvement is extremely rare. This report describes a case of isolated extraaortic mediastinal IgG4-related disease encasing the superior vena cava (SVC) and manifesting as SVC syndrome in a 25-year-old man with no personal or family history of autoimmune disease. Resection with SVC reconstruction was performed.

Changes of the hepatic subcapsular blood flow in a case of high-grade retroperitoneal liposarcoma: what to expect?

Changes of the hepatic subcapsular blood flow with the early appearance of hypervascularity near the falciform ligament are rare radiologic findings. They present most frequently in cases of superior vena cava (SVC) obstruction and are related to the recruitment of the cavo-mammary-phrenic-hepatic-capsule-portal and the cavo-superficial-umbilical-portal pathways. We present the case of a 52-year-old female patient with an highly aggressive retroperitoneal liposarcoma with SVC obstruction caused by external compression due to a mediastinal metastatic mass. The patient exhibited no symptoms of SVC obstruction due to the collateral cavo-portal pathways.

Lead-related superior vena cava syndrome: Management and outcomes.

BACKGROUND: Superior vena cava (SVC) syndrome includes the clinical sequalae of facial and bilateral upper extremity edema, dizziness, and occasional syncope. Historically, most cases have been associated with malignancy and treatment is palliative. However, cardiac device leads have been identified as important nonmalignant causes of this syndrome. There are little data on the effectiveness of venoplasty and lead extraction in the management of these patients. OBJECTIVE: The objective of this study was to report the findings associated with the use of balloon angioplasty and lead extraction in the management of 17 patients with lead induced SVC syndrome. METHODS: Data collected from January 2003 to July 2019 identified 17 cases of SVC syndrome at our tertiary center. Their outcomes were compared to a control group of patients without SVC syndrome. A P value of <.05 was considered statistically significant. RESULTS: Of the 17 patients, 13 (76%) underwent transvenous lead extraction and venoplasty. Three patients (18%) were treated with venoplasty alone, and 1 patient (6%) underwent surgical SVC reconstruction. In 10 patients (59%), transvenous reimplantation was necessary. Symptom resolution was achieved in all 17 patients and confirmed at both 6 and 12 months' follow-up. There was no significant difference in the rate of complications associated with transvenous lead extraction for SVC syndrome vs control. CONCLUSION: In patients with SVC syndrome, venoplasty and lead extraction are safe and effective for resolution of symptoms and maintaining SVC patency.