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*

Superior vena cava (SVC) syndrome refers to a medical emergency resulting from compression of the SVC. It requires early diagnosis and treatment, and is usually caused by malignant tumors; rarely, mediastinal tuberculous lymphadenitis can cause SVC syndrome. Here, we present a case study of an immunocompetent 61-year-old woman who presented with acute onset SVC syndrome and was diagnosed with tuberculous lymphadenitis on thoracotomy; the symptoms resolved with anti-tuberculosis therapy. This unusual case highlights the importance of the differential diagnosis in patients presenting with acute onset SVC syndrome; a timely diagnosis and appropriate treatment lead to complete recovery.
Diagnosis ; Diagnosis, Differential ; Early Diagnosis ; Emergencies ; Female ; Humans ; Lymphadenitis ; Middle Aged ; Superior Vena Cava Syndrome* ; Thoracotomy ; Tuberculosis ; Tuberculosis, Lymph Node* ; 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

We present a case of a patient exhibiting isolated elevation of the central venous pressure with minimal hemodynamic deterioration in an immediate postoperative period after Bentall operation requiring re-exploration. Isolated elevation of the central venous pressure usually alerts physicians of a volume overload or right ventricular dysfunction. However, even in the absence of significant hemodynamic deterioration, the development of loculated hematoma that compresses the superior vena cava should be ruled out, as it can be life-threatening through the formation of cerebral and laryngeal edema, similar to superior vena cava syndrome. This case emphasizes the importance of a prompt differential diagnosis of the isolated central venous pressure elevation after cardiac surgery with transesophageal echocardiography for the administration of appropriate treatment.
Cardiac Surgical Procedures ; Central Venous Pressure* ; Diagnosis, Differential ; Echocardiography, Transesophageal ; Hematoma* ; Hemodynamics ; Humans ; Laryngeal Edema ; Postoperative Period ; Superior Vena Cava Syndrome ; Thoracic Surgery ; Vena Cava, Superior* ; Ventricular Dysfunction, Right

OBJECTIVE: To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. METHODS: A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. RESULTS: Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. CONCLUSION: Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.
Atrioventricular Block ; Bradycardia ; Double Outlet Right Ventricle ; Echocardiography ; Fetus ; Heart Block ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Heterotaxy Syndrome ; Humans ; Infant ; Isomerism* ; Live Birth ; Lost to Follow-Up ; Prenatal Diagnosis* ; Prevalence ; Pulmonary Atresia ; Retrospective Studies ; Survival Rate ; Vena Cava, Inferior ; Vena Cava, Superior

BACKGROUNDThe pathological diagnosis is of critical importance to the subsequent treatment for the pathients with superior vena cava syndrome (SVCS). The aim of this study is to report our experience in the diagnosis of SVCS by endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA).

METHODSThe data of 520 patients who underwent EBUS-TBNA from September 2009 to May 2012 at our institution were reviewed. Of these, there were 14 males and 6 females (mean age of 59.1 years) with SVCS who received EBUS-TBNA that were included in the analysis.

RESULTSThe mean short axis diameter of the paratracheal lesions was (3.32 ± 1.79) cm (range, 1.69 to 9.50 cm) and 6 cases also had subcarinal lymph node enlargement with a mean short axis diameter of (2.14 ± 0.49) cm (range, 1.73 to 3.01 cm). An average of 4.3 punctures was performed per lesion. Malignancy was confirmed in 16 cases (10 small cell carcinomas, 4 adenocarcinomas, 1 squamous cell carcinoma and 1 Hodgkin lymphoma). In two patients, pathological examination of tissue revealed no evidence of malignancy and for 13 to 24 months of follow-up. One patient from whom adequate tissue was not obtained refused further surgical biopsy since he had undergone endovascular stenting of the SVC. One patient in whom a diagnosis was not obtained by EBUS-TBNA underwent thoracoscopic biopsy and the final diagnosis was B cell non-Hodgkin's lymphoma. The diagnosis accuracy of EBUS-TBNA in SVCS was 18/20 patients.

CONCLUSIONEBUS-TBNA is a highly effective and safe procedure for the diagnosis of SVCS.

Adult ; Aged ; Biopsy, Fine-Needle ; Bronchoscopy ; Female ; Humans ; Image-Guided Biopsy ; Male ; Middle Aged ; Superior Vena Cava Syndrome ; diagnosis

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

OBJECTIVE: To determine the clinical significance of persistent left superior vena cava (PLSVC) in a fetus with and without cardiac and extra-cardiac anomalies. METHODS: This was a retrospective review of all cases of PLSVC detected prenatally at our institution between May 2001 and May 2008. This retrospective study included 85 fetuses with PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Patient charts and recorded images were reviewed in order to identify associated conditions and outcomes. Telephone interviews were conducted to check patients' conditions in cases of isolated PLSVC. RESULTS: Eighty-five cases of PLSVC were detected prenatally during this study period. Of these 85 fetuses, 11 were aborted due to associated, prenatally proven, severe congenital heart anomalies or chromosomal anomalies, and 52 fetuses were delivered. The cases for other 22 fetuses were lost to follow-up. Postnatal echocardiography was performed in the 33, surviving patients, and PLSVC was confirmed in 32 of these patients. The most common associated congenital cardiac anomalies were seen included VSD, AVSD, and DORV (54.1%, 17.6% and 17.6%, respectively) (Table 3). PLSVC was also seen in seven cases (8.2%) of right isomerism and in four cases (4.7%) of left isomerism. In only two cases was the coexistence of PLSVC and extra-cardiac anomalies noted in this study. Fifteen cases were prenatally diagnosed as isolated PLSVC and all of them had live births. The follow-up period in our isolated PLSVC patients ranged from 0.5 to 84 months (Mean 24.5 months). Thirteen of these infants were doing well at the time of preparing this document and one case was diagnosed as TAPVR on postnatal echocardiography and one case was lost to follow-up. CONCLUSION: We strongly suggest that PLSVC is a benign vascular malformation and does not affect to the patient after birth. However, PLSVC is frequently associated with heterotaxy syndromes as well as other cardiac malformations and can be misdiagnosed as TAPVR. So if we find PLSVC in prenatal ultrasonography, meticulous inspection of the fetal anatomy must be performed.
Echocardiography ; Female ; Fetus ; Follow-Up Studies ; Glycosaminoglycans ; Heart ; Heterotaxy Syndrome ; Humans ; Infant ; Interviews as Topic ; Isomerism ; Live Birth ; Lost to Follow-Up ; Parturition ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Pulmonary Artery ; Retrospective Studies ; Scimitar Syndrome ; Ultrasonography, Prenatal ; Vascular Malformations ; Vena Cava, Superior

Superior vena cava syndrome can occur from benign conditions that might not alter life expectancy. Here we present a case of a superior vena cava (SVC) obstruction caused by soft tissue encircling the SVC, which was strongly suspected of being an unusual focal type of fibrosing mediastinitis. A 39-year-old man with no prior medical history presented with a four-week history of facial plethora, headache and dilated veins of the neck with a dark purple color change on the anterior chest wall. Radiology examinations, including venography, and computed tomography with a 3-dimensional volume-rendering image of the chest, had revealed severe narrowing of the SVC due to tiny encircling soft tissue and collateral vessels. A total occlusion of the SVC occurred as a result of a thrombus that developed within 1 day after the diagnostic SVC angiogram. The patient underwent stent deployment three days after the administration of thrombolytic therapy.
Adult ; Fibrinolytic Agents/therapeutic use ; Humans ; Male ; *Stents ; Superior Vena Cava Syndrome/*diagnosis/etiology/surgery