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*

Aged, 80 and over ; Arterio-Arterial Fistula ; diagnosis ; Coronary Aneurysm ; diagnosis ; Coronary Disease ; diagnosis ; Diagnostic Errors ; Female ; Humans ; Mediastinal Neoplasms ; diagnosis

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.
Child ; Diagnosis ; DiGeorge Syndrome* ; Humans ; Hypocalcemia ; Lymphoma ; Lymphoproliferative Disorders ; Magnetic Resonance Imaging ; Mediastinal Neoplasms ; Phenotype ; Pneumonia ; Thoracic Surgery, Video-Assisted ; Thorax ; Thymus Gland ; Ultrasonography

We aimed to evaluate the histologic components of primary mediastinal mixed germ cell tumors. A total of 221 patients diagnosed with a mediastinal germ cell tumor (GCT) were retrospectively reviewed. Among them, 14 patients underwent surgical resection after chemotherapy and 8 patients were diagnosed with mixed GCT, who were then selected for further evaluation. Clinical chart review and histologic review of biopsy and surgical specimens of 8 patients were performed. All 8 patients were young males and showed a mature teratoma or a mature teratoma with a focal immature teratoma in the resected specimens. Serum alpha-feto protein was variably elevated. Seven patients experienced an increase in tumor size after the chemotherapy. In 5 patients, a variable amount of vasculoconnective tissue was found along with the mature teratoma occupying average 66.3% of resected mass, and 3 of them showed an identical vasculoconnective component on biopsy before chemotherapy. We suggest that vasculoconnective tissue might be the intrinsic component of primary mediastinal mixed GCT. When vasculoconnective tissue is obtained on small biopsy of an anterior mediastinal mass of a young male, the possibility of underlying mixed GCT should be considered and further clinical work up should be performed.
Adolescent ; Adult ; Blood Vessels/*pathology ; Connective Tissue/*pathology ; Diagnosis, Differential ; Humans ; Male ; Mediastinal Neoplasms/*pathology ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/*pathology ; Teratoma/*pathology ; Young Adult

Humans ; Infant, Newborn ; Male ; Mediastinal Neoplasms ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Tomography, X-Ray Computed

OBJECTIVETo investigate the clinical features and prognostic factors of primary mediastinal large B-cell lymphoma (PMLBCL).

METHODSThe clinical data of 82 patients with PMLBCL enrolled from January 2000 to January 2008 were retrospectively studied. All these patients were treated in four affiliated hospitals of Central South University,Hunan province. The prognostic factors were investigated.

RESULTSOf the 82 patients, 45 were men and 37 were women, the ratio was 1.22:1. The median age was 29.5 (ranged from 12 to 78) years old. There were 40 (48.78%) patients in stage I/II, 42 (51.22%) in stage III/IV. The complete response (CR) rate was 13.4% (11/82), and the overall response rate 76.83% (63/82). The 5-year overall survival was 58%. The univariate analysis indicated that the poor prognostic factors included stage III/IV(P=0.005), without rituximab (P=0.004), without radiotherapy (P=0.000), LDH ≥ ULN (upper limit of normal) (P=0.000), disease progression (P=0.000), international prognostic index (IPI)≥ 2 (P=0.000) and superior vena cava syndrome (P=0.015). Chemo-therapy alone (P=0.000) predicted poor outcome. Combination therapy (such as chemo-radiotherapy, chemotherapy combined with rituximab) had better prognosis. Compared to second-line treatment, rituximab as the first-line treatment can prolong PFS, but had no effect on the OS. In multivariate analysis, chemo-radiotherapy and IPI were independently related to prognosis.

CONCLUSIONPMLBCL mostly affects young adults, male patients were slightly more than female patients. It presents with a typical bulky mediastinal mass at diagnosis, which constricts surrounding organs. Patients treated with rituximab or radiation therapy had better prognosis. Rituximab is recommended to be used for the first-line treatment.

Adolescent ; Adult ; Aged ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Child ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; therapy ; Male ; Mediastinal Neoplasms ; diagnosis ; pathology ; therapy ; Middle Aged ; Prognosis ; Radiotherapy ; Retrospective Studies ; Rituximab ; Young Adult

Myelolipoma in the mediastinum is an extremely rare entity. In this report, we present the case of a 79-year-old asymptomatic man who had three bilateral paravertebral mediastinal tumors. The three tumors were resected simultaneously using bilateral three-port video-assisted thoracoscopic surgery (VATS). There has been no evidence of recurrence within four years after the operation. Multiple bilateral mediastinal myelolipomas are extremely rare. There are no reports in the English literature of multiple bilateral thoracic myelolipomas that were resected simultaneously using bilateral VATS. We also present characteristic features of myelolipomas, which are helpful for diagnosis.
Aged ; Diagnosis ; Mediastinal Neoplasms ; Mediastinum ; Myelolipoma* ; Recurrence ; Surgical Procedures, Minimally Invasive ; Thoracic Surgery, Video-Assisted* ; Thoracoscopy

Magnetic resonance imaging (MRI) of the breast is used for various indications. Contrary to computed tomography as a staging tool, breast MRI focuses on the breast parenchyma and axilla. In spite of narrow field of view, many structures such as the anterior portion of the lungs, mediastinum, bony structures and the liver are included which should not be neglected because the abnormalities detected on the above structures may influence the staging and provide a clue to systemic metastasis, which results in the change of treatment strategy. The purpose of this pictorial essay was to review the unexpected extra-mammary findings seen on the preoperative breast MRI.
Adult ; Aged ; Axilla ; Bone Neoplasms/*diagnosis/secondary ; Breast Neoplasms/*diagnosis ; Female ; Humans ; *Incidental Findings ; Liver Neoplasms/*diagnosis ; Lung Neoplasms/*diagnosis ; Magnetic Resonance Imaging/*methods ; Mediastinal Neoplasms/*diagnosis ; Middle Aged ; Tomography, X-Ray Computed

Mediastinal teratomas have been reported to mimic pleural effusions on chest radiography. Further evaluation of such tumours using computed tomography usually yields diagnostic characteristics that distinguish them from pleural collections. We report a patient with a mediastinal teratoma that mimicked a massive left pleural effusion on chest radiography and computed tomography.
Adult ; Contrast Media ; Female ; Humans ; Mediastinal Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Pleural Effusion ; Radiography, Thoracic ; Teratoma ; diagnosis ; diagnostic imaging ; surgery ; Tomography, X-Ray Computed

This study was aimed to investigate the clinical manifestation, pathological features, treatment and related prognosis factors of primary mediastinal large B cell lymphoma (PMLBCL). The clinical data of 29 PMLBCL patients admitted in Peking University First Hospital were summarized and the related factors were analyzed retrospectively from January 2000 to November 2013. The results showed that 29 patients with the median age 32 were all pathologically diagnosed as PMLBCL. The main clinical features included mediastinal bulk mass (72.4%), superior vena caval syndrome (51.7%), dyspnea (62.1%), serous membrane fluid (48.3%), with 62.1% extranodal invasion and 62.1% extra-thoracic involvement. According to Ann-Arbor stage, 16 patients (55.1%) were classified to stage I/II and 13 patients (44.9%) to stage III/IV, 12 patients (41.4%) had B symptoms. Among the 29 patients, 2 patients failed to be followed and the others were followed for the median time of 29 months, 17 patients achieved CR, 5 patients achieved PR, 1 patient replaced and 4 patients died of disease progression. The 5-year overall survival rate (OS) was 85.2%, in which RCHOEP regimen group patients had OS 94.4% and CHOEP group patients had OS 75%; 8 patients underwent auto-HSCT and 1 patients underwent allo-HSCT who kept in CR state. Univariate analysis by log-rank test showed albumin level and LDH ≥ 2ULN, the initial therapy response and IPI score were prognostic factors , but neither were independent prognostic factors by Cox Regression Model. It is concluded that PMLBCL has distinct clinical features. RCHOEP chemotherapy regimen can achieve satisfactory results, but needs to be explored by further clinical trials.
Follow-Up Studies ; Humans ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; therapy ; Mediastinal Neoplasms ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Survival Rate