Humans ; Mediastinal Neoplasms/therapy* ; Superior Vena Cava Syndrome/therapy* ; Vena Cava, Superior/surgery*

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.
Child ; China ; Cough ; Edema ; Female ; Humans ; Male ; Mediastinal Neoplasms/diagnosis* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Sounds ; Retrospective Studies ; Superior Vena Cava Syndrome/therapy*

Consensus ; Humans ; Mediastinal Neoplasms/therapy* ; Superior Vena Cava Syndrome/therapy* ; Vena Cava, Superior

Adenomyoepithelioma (AME) of the breast is an uncommon tumor characterized by its dual differentiation into luminal cells and myoepithelial cells. In most cases these tumors have a benign clinical course, but distant metastases have been reported. We present the case of a 51-year-old woman diagnosed with malignant AME. The patient underwent a right modified radical mastectomy, and pathological examination confirmed the diagnosis of malignant AME. Ten months after the operation, multiple hepatic, pleural, and abdominal wall metastases were detected. A number of palliative chemotherapeutic agents were tried, including anthracycline and taxanes. However, the disease continued to progress, and superior vena cava syndrome developed as a result of direct tumor invasion. The patient received salvage eribulin monotherapy. After two cycles of this treatment, her clinical symptoms were ameliorated, and a computed tomography scan showed a partial response. Eribulin chemotherapy was thus effective in treating malignant AME in this case.
Abdominal Wall ; Adenomyoepithelioma* ; Breast* ; Diagnosis ; Drug Therapy ; Female ; Humans ; Mastectomy, Modified Radical ; Middle Aged ; Neoplasm Metastasis ; Phenobarbital ; Superior Vena Cava Syndrome ; Taxoids

OBJECTIVE: To evaluate the safety and efficacy of unilateral covered stent placement in patients with malignant superior vena cava (SVC) syndrome. MATERIALS AND METHODS: Between October 2008 and November 2012, expanded polytetrafluoroethylene-covered stent placement for malignant SVC syndrome was performed in 40 consecutive patients (35 men and five women; mean age, 61.4 years; range, 35-81 years). All covered stents were unilaterally placed within the SVC or across the venous confluence when needed to relieve venous obstruction and prevent tumor overgrowth, regardless of patency of contralateral brachiocephalic veins. RESULTS: Stent placement was technically successful in all patients. There were no major complications. Of the 37 patients symptomatic prior to stent placement, 34 (92%) experienced complete symptomatic relief 1-8 days after stent placement. Of the 29 patients who underwent covered stent placement across the venous confluence, nine patients had patent contralateral brachiocephalic veins prior to stent placement. However, no sign of SVC obstruction or contralateral upper extremity venous thrombosis was observed during the follow-up period. Kaplan-Meier analysis revealed median patient survival of 163 days. Stent occlusion occurred in four (10%) of 40 patents. Cumulative stent patency rates at 1, 3, 6, and 12 months were 95%, 92%, 86%, and 86%, respectively. CONCLUSION: Unilateral covered stent placement appears to be a safe and effective method for treating malignant SVC syndrome, despite the location of SVC occlusion.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; *Polytetrafluoroethylene ; Retrospective Studies ; *Stents/adverse effects ; Superior Vena Cava Syndrome/etiology/mortality/*therapy ; Treatment Outcome

Central venous catheters are the most frequent causes of benign central vein stenosis. We report the case of a 79-year-old woman on hemodialysis through a twin catheter in the right internal jugular vein, presenting with superior vena cava (SVC) syndrome with patent SVC. The clinically driven endovascular therapy was conducted to treat the venous syndrome with a unilateral left brachiocephalic stent-graft without manipulation of the well-functioning catheter. The follow-up was uneventful until death 94 months later.
Aged ; Brachiocephalic Veins ; Central Venous Catheters/*adverse effects ; Constriction, Pathologic/etiology ; Female ; Humans ; Jugular Veins ; Renal Dialysis/instrumentation ; *Stents ; Superior Vena Cava Syndrome/*etiology/therapy ; Vena Cava, Superior

OBJECTIVETo investigate the method, therapeutic effect and safety of interventional therapy for lung cancer patients with superior vena cava syndrome (SVCS).

METHODSFifty-two cases of lung cancer with SVCS who received interventional therapy in our hospital between Jan to Dec 2011 were included in this study. Of the 52 cases, 50 cases had successfully carried out superior vena cava stent implantation. The distal venous pressure was measured before and after angioplasty, and the results were assessed by Wilcoxon matched-pairs test. In addition, the 50 patients were followed up and the therapeutic effect and postoperative survival rate were evaluated.

RESULTSThe mean distal venous pressure in the 50 patients was significantly decreased from preoperative (28.2 ± 1.9)cm H2O to postoperative (8.7 ± 0.5)cm H2O (P = 0.0085). The efficacy of the treatment was as follows: complete remission (20/52, 38.5%), partial remission (28/52, 53.8%), ineffective 4 (4/52, 7.7%), and total effective rate 92.3%. The complications after angioplasty and stent implantation included chest pain (12 cases, 23.1%), hematoma at the puncture site (5 cases, 9.6%), and fever (2 cases, 3.8%). No serious complications such as massive hemorrhage, pulmonary embolism and stent migration into the cardiac atrium were observed. The rate of postoperative restenosis was low (2/52, 3.8%). For the SCLC group, the objective effective rate was 74.1% and 1-year survival rate was 21.0%. For the NSCLC group, the objective effective rate was 21.7% and 1-year survival rate was 35.0%.

CONCLUSIONSFor lung cancer patients with SVCS, interventional therapy may relief obstruction effectively, promote blood flow recovery, and relieve clinical symptoms. Interventional therapy with endovascular angioplasty and stenting may be highly recommended as the first choice for palliative treatment of SVCS. It is an effective initial palliative treatment. However, subsequent comprehensive anti-tumor treatment is necessary.

Adult ; Aged ; Angioplasty ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Blood Pressure ; Carcinoma, Non-Small-Cell Lung ; complications ; drug therapy ; radiotherapy ; Chest Pain ; etiology ; Female ; Follow-Up Studies ; Hematoma ; etiology ; Humans ; Lung Neoplasms ; complications ; drug therapy ; radiotherapy ; Male ; Middle Aged ; Radiotherapy, High-Energy ; Remission Induction ; Small Cell Lung Carcinoma ; complications ; drug therapy ; radiotherapy ; Stents ; Superior Vena Cava Syndrome ; complications ; therapy ; Survival Rate

We report successful outcomes after endovascular placement of a stent graft in a 74- and a 77-year-old men, both of whom had malignant superior vena cava syndrome caused by squamous cell carcinoma. In each patient, successful palliation of the malignant superior vena cava syndrome was achieved by placement of a stent graft. No procedure-related complications were observed. The patients were asymptomatic until their deaths, seven and 14 months after stent graft placement, respectively.
Aged ; Biopsy ; Bronchoscopy ; Carcinoma, Squamous Cell/*therapy ; *Endovascular Procedures ; Humans ; Male ; Palliative Care ; *Stents ; Superior Vena Cava Syndrome/diagnosis/pathology/*therapy ; Tomography, X-Ray Computed

Adult ; Aged ; Female ; Humans ; Male ; Mediastinoscopy ; methods ; Middle Aged ; Stents ; Superior Vena Cava Syndrome ; diagnosis ; therapy

AIDS related malignancies have gradually decreased after the introduction of highly active antiretroviral therapy (HAART). Nevertheless, non-Hodgkin's lymphoma is still a major malignancy in resource limited countries that have difficulty supplying HAART, and same holds true for Korea. We report a case of non-Hodgkin's lymphoma involving the heart in a Korean AIDS patient, who presented with sudden arrhythmia and superior vena cava syndrome. He was diagnosed with AIDS in 2008, but refused to receive HAART. Immunohistologic findings were compatible diffuse large B cell lymphoma, and futher studies demonstrated involvement of the liver, adrenal gland, heart, and pericardiac space. HAART was initiated and non Hodgkin's lymphoma was successfully managed with intensive chemotherapy. This is the first cardiac involvement non Hodgkin's lymphoma in AIDS patients in Korea.
Adrenal Glands ; Antiretroviral Therapy, Highly Active ; Arrhythmias, Cardiac ; Heart ; Humans ; Korea ; Liver ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Superior Vena Cava Syndrome