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

Locally advanced non-small cell lung cancer (LA-NSCLC) is composed of heterogeneous subgroups that require a multidisciplinary team approach in order to ensure optimal therapy for each patient. Since 2010, the National Comprehensive Cancer Network has recommended chemoradiation therapy (CRT) for bulky mediastinal disease and surgical combination for those patients with single-station N2 involvement who respond to neoadjuvant therapy. According to lung cancer tumor boards, thoracic surgeons make a decision on the resectability of the tumor, if it is determined to be unresectable, concurrent CRT (CCRT) is considered the next choice. However, the survival benefit of CCRT over sequential CRT or radiotherapy alone carries the risk of additional toxicity. Considering severe adverse events that may lead to death, fit patients who are able to tolerate CCRT must be identified by multidisciplinary tumor board. Decelerated approaches, such as sequential CRT or high-dose radiation alone may be a valuable alternative for patients who are not eligible for CCRT. As a new treatment strategy, investigators are interested in the application of the innovative radiation techniques, trimodality therapy combining surgery after high-dose definitive CCRT, and the combination of radiation with targeted or immunotherapy agents. The updated results and on-going studies are thoroughly reviewed in this article.
Carcinoma, Non-Small-Cell Lung* ; Chemoradiotherapy ; Combined Modality Therapy ; Humans ; Immunotherapy ; Lung Neoplasms ; Mediastinal Diseases ; Neoadjuvant Therapy ; Patient Care Team ; Radiotherapy ; Research Personnel ; Surgeons

PURPOSE: To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution. METHODS: Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group classification used for GCTs in adults to validate the classification guidelines for GCTs in children. RESULTS: The median patient age was 4.4 years. In 34 patients (51.5%), the primary tumor site was the gonad. Extragonadal GCTs were detected in 32 patients. The 5-year overall survival and event-free survival (EFS) were 92.0%+/-3.5% and 90.4%+/-3.7%, respectively. In univariate analysis, tumor histology, metastasis, and elevated alpha-fetoprotein were not prognostic factors in children with extracranial GCTs. However, EFS was poorer in patients with mediastinal disease (n=12, 66.7%+/-13.6 %) than in those with nonmediastinal disease (n=54, 96.0%+/-2.8%) (P=0.001). The 5-year EFS was lower in patients older than 10 years, (n=21, 80.0%+/-8.9%) compared with those younger than 10 years (n=45, 95.2%+/-3.3%) (P=0.04). Multivariate analysis identified the mediastinal tumor site as the only independent prognostic factor. CONCLUSION: The prognosis of children with extracranial GCTs was favorable. However, nongerminomatous mediastinal tumors were associated with poor survival in children. Further research is needed to improve the prognosis of children with malignant mediastinal GCTs.
Adult ; alpha-Fetoproteins ; Child* ; Classification ; Disease-Free Survival ; Drug Therapy* ; Germ Cells* ; Gonads ; Humans ; Mediastinal Diseases ; Mediastinum ; Multivariate Analysis ; Neoplasm Metastasis ; Neoplasms, Germ Cell and Embryonal* ; Prognosis ; Survival Rate

Aged, 80 and over ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Neoplasms ; diagnostic imaging ; drug therapy ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Fluorodeoxyglucose F18 ; Heart Neoplasms ; diagnostic imaging ; drug therapy ; Humans ; Lymph Nodes ; Lymphoma, Large B-Cell, Diffuse ; diagnostic imaging ; drug therapy ; Mediastinal Neoplasms ; diagnostic imaging ; drug therapy ; Peripheral Nervous System Neoplasms ; diagnostic imaging ; Positron Emission Tomography Computed Tomography ; Prednisone ; therapeutic use ; Radiopharmaceuticals ; Vincristine ; therapeutic use

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

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

We report an unusual case of 9.5-cm-sized embryonal rhabdomyosarcoma arose from a mediastinal mature teratoma in a 46-yr-old man. A man presented with chest trauma as a result of an accident at 10 September 2011. On chest X-ray, an anterior mediastinal mass was detected. To obtain further information, chest computed tomography (CT) with contrast enhancement was performed, revealing an anterior mediastinal mass. Complete surgical excision was performed and entire specimen was evaluated. Pathologic diagnosis was embryonal rhabdomyosarcoma arising in mature cystic teratoma. After surgical excision, two cycles of dactinomycin-based chemotherapy were performed. Lung metastasis was detected on follow up CT in September 2012, and wedge resection was performed. Pathological finding of the lung lesion showed same feature with that of primary rhabdomyosarcoma.
Antibiotics, Antineoplastic/therapeutic use ; Dactinomycin/therapeutic use ; Desmin/metabolism ; Humans ; Immunohistochemistry ; Lung Neoplasms/radiography/secondary/surgery ; Male ; Mediastinal Neoplasms/*diagnosis/pathology ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/drug therapy/*radiography/surgery ; Rhabdomyosarcoma, Embryonal/drug therapy/*radiography/surgery ; Teratoma/*diagnosis/pathology ; Tomography, X-Ray Computed

BACKGROUNDPrimary malignant germ cell tumors (GCTs) of mediastinum are rare neoplasms. We introduce our institutional experience in managing patients with primary malignant GCTs of the mediastinum, focusing on the analysis of therapeutic modalities.

METHODSA retrospective review was done in 39 consecutive patients with mediastinal malignant GCTs treated in our institution between 1991 and 2007.

RESULTSA total of 39 patients were enrolled in this study with a median age of 27 years. The 5-year overall survival (OS) and progression-free survival (PFS) rates of the whole population were 60.2% and 57.7%, respectively. Stratified by the histology, 18 patients (46.2%) had seminoma and 21 patients (53.8%) had nonseminomatous germ cell tumors (NSGCTs). The 5-year OS rate of patients with seminoma was 87.4% as compared with 36.7% in patients with NSGCTs (P = 0.0004). The 5-year PFS rate was also significantly higher in seminoma patients (87.4% vs. 31.6%, P = 0.003). For 19 patients with NSGCTs managed with multi-modality treatment, chemotherapy exposure appeared to impact the prognosis. The 5-year OS rate was 44.9% in patients with chemotherapy exposure as compared with 20.0% in patients without it (P = 0.43).

CONCLUSIONOur study confirmed the significance of systemic chemotherapy in the treatment of primary mediastinal GCTs.

Adolescent ; Adult ; Disease-Free Survival ; Female ; Humans ; Male ; Mediastinal Neoplasms ; drug therapy ; radiotherapy ; Middle Aged ; Neoplasms, Germ Cell and Embryonal ; drug therapy ; radiotherapy ; Retrospective Studies ; Seminoma ; drug therapy ; radiotherapy ; Testicular Neoplasms ; Young Adult