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Medicine Aug 2019Medical decisions should be well-planned to improve prognosis and reduce complications of mediastinal tumors. In this study, we analyzed the clinical presentations of... (Observational Study)
Observational Study
Medical decisions should be well-planned to improve prognosis and reduce complications of mediastinal tumors. In this study, we analyzed the clinical presentations of pediatric mediastinal tumors and their correlation with long-term clinical outcome.Forty patients under 18 years of age diagnosed with mediastinal tumors at China Medical University Children's Hospital between 2001 and 2016 were enrolled. The patients' sex, age of onset, initial clinical symptoms, and treatment outcomes were analyzed.75% of the patients with mediastinal tumors in this study were men, and the median age of onset was 13 years old (age range: 0-17 years). The overall mortality rate was 40%. The most common tumors were lymphoma (47.5%), followed by germ cell tumors (12.5%), neuroblastoma (12.5%), and thymoma (7.5%). Neuroblastoma was more prevalent in girls younger than 5 years old. The initial presentations of these patients included breathing difficulty (65%), productive cough (47.5%), pleural effusion (54.5%), superior vena cava (SVC) syndrome (35%), neck mass (35%), airway compression (32.5%), fever (30%), chest pain (27.5%), and pericardial effusion (25%). Lymphomas were more likely to be accompanied by neck mass (52.6% vs19.0%, P = .04) and SVC syndrome (52.6% vs 19.0%, P = .026), yet also had a better 1-year-survival rate (68.4% vs 52.4%, P = .02).Overall, lymphoma should be suspected when children present with neck mass and SVC syndrome. Neuroblastoma with a posterior mediastinal origin should be suspected among children younger than 5 years old. Tumor-related airway obstruction, pleural effusion, and pericardial effusion were leading cause of cardiopulmonary instability during sedation for invasive procedures, which should be managed cautiously.
Topics: Adolescent; Age of Onset; Child; Child, Preschool; Dyspnea; Female; Humans; Infant; Male; Mediastinal Neoplasms; Pleural Effusion; Sex Factors; Superior Vena Cava Syndrome
PubMed: 31393382
DOI: 10.1097/MD.0000000000016732 -
Thoracic Cancer Dec 2018Liposarcoma has been subclassified histologically into well-differentiated, myxoid, pleomorphic, and dedifferentiated types. The dedifferentiated type generally shows... (Review)
Review
BACKGROUND
Liposarcoma has been subclassified histologically into well-differentiated, myxoid, pleomorphic, and dedifferentiated types. The dedifferentiated type generally shows poorer prognosis than the well-differentiated type. Because of its rarity, the clinicopathological features and clinical outcomes of primary mediastinal dedifferentiated liposarcoma remain unclear.
METHODS
Five patients with primary mediastinal dedifferentiated liposarcoma were treated at Shinshu University Hospital between January 2012 and August 2017. We investigated the clinical characteristics, including age, gender, radiographic findings, pathological status, and clinical and treatment outcomes.
RESULTS
Four of the five patients initially underwent radical surgical resection. One patient was disease-free after surgery, but the remaining three patients developed local recurrence in the mediastinum after surgical resection. Two of these patients underwent repeat surgical resection, resulting in long survival (60 and 40 months, respectively), while the other underwent proton beam therapy and showed no evidence of recurrence as of 17 months after treatment. The remaining patient was treated with chemotherapy using doxorubicin because of advanced inoperable disease, but failed to show a response and died within a month of the initiation of chemotherapy. Although the maximum standardized uptake values on fluorodeoxyglucose-computed tomography were relatively low, there was a slight positive relation between these values and the Ki-67-positive ratio in the tumor.
CONCLUSION
Aggressive treatment by surgical resection should be considered for mediastinal dedifferentiated liposarcoma, even in cases with local recurrence.
Topics: Aged; Aged, 80 and over; Biomarkers, Tumor; Biopsy; Female; Humans; Immunohistochemistry; Liposarcoma; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Grading; Positron Emission Tomography Computed Tomography; Tomography, X-Ray Computed; Tumor Burden
PubMed: 30329218
DOI: 10.1111/1759-7714.12888 -
Internal Medicine (Tokyo, Japan) 2011
Topics: Adolescent; Humans; Male; Mediastinal Neoplasms; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma; Superior Vena Cava Syndrome; Tomography, X-Ray Computed
PubMed: 22082907
DOI: 10.2169/internalmedicine.50.6353 -
Biology of Blood and Marrow... Oct 2018Primary mediastinal (thymic) large B cell lymphoma is a subtype of diffuse large B cell lymphoma with distinct clinical, molecular, and genetic features, many of which...
Primary mediastinal (thymic) large B cell lymphoma is a subtype of diffuse large B cell lymphoma with distinct clinical, molecular, and genetic features, many of which overlap with Hodgkin lymphoma. Increasingly, initial therapy for these patients has used dose-dense chemotherapy with or without radiation with excellent results. In patients with relapsed and primary refractory disease, outcomes of second-line therapy followed by consolidation with high-dose therapy and autologous stem cell transplantation remains largely undefined. We reviewed the outcomes of 60 transplant-eligible patients with relapsed or refractory primary mediastinal (thymic) large B cell lymphoma enrolled on sequential protocols with uniform second-line therapy with intent to consolidate with autologous stem cell transplant. The estimated 3-year overall and event-free survivals for all patients were 61% and 57%, respectively, and 68% and 65%, respectively, for patients proceeding to stem cell transplant. Multivariable analysis of risk factors before transplant revealed that an incomplete response to initial therapy, advanced Ann Arbor stage at disease progression, and failure to achieve a partial remission or better to second-line therapy to be independently associated with inferior event-free and overall survival. A risk score based on these variables was able to identify patients who are unlikely to respond to conventional second-line strategies. These results suggest that salvage chemoradiotherapy with intent of subsequent high-dose therapy and autologous stem cell transplant is successful in most patients with relapsed and refractory primary mediastinal (thymic) large B cell lymphoma. Alternative strategies are warranted for a significant subset of patients with high-risk disease who are unlikely to be cured with this strategy.
Topics: Adult; Aged; Autografts; Consolidation Chemotherapy; Disease-Free Survival; Female; Hematopoietic Stem Cell Transplantation; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Recurrence, Local; Retrospective Studies; Survival Rate; Thymoma; Transplantation, Autologous
PubMed: 29909154
DOI: 10.1016/j.bbmt.2018.06.009 -
Cirugia Y Cirujanos 2015Carcinoid of the small intestine, is a well-differentiated neuroendocrine tumor that rarely presents with clinical signs. This tumour can be associated with other... (Review)
Review
BACKGROUND
Carcinoid of the small intestine, is a well-differentiated neuroendocrine tumor that rarely presents with clinical signs. This tumour can be associated with other conditions, such as inflammatory bowel disease, presenting a wide range of symptoms. In some cases they have an aggressive and highly symptomatic behaviour; thus, clinical suspicion must be high to make an early diagnosis.
CLINICAL CASE
A 60 year-old male patient with Crohn's disease and gastrointestinal symptoms attributed to this disease within the last year. He presented with intestinal obstruction initially treated with conservative management with no improvement. Exploratory laparotomy was performed finding a mesenteric tumour that caused the bowel obstruction. Bowel resection with primary anastomosis was performed. The pathology report showed an intestinal carcinoid tumour with lymph node metastases. The patient recovered well, and was discharged without complications to continue medical treatment and follow-up by the Oncology department.
CONCLUSION
In almost 42% of the cases, the most common site of carcinoid tumours is the small intestine, and of these, 41% are presented as locoregional disease. Patients with Crohn's disease present a higher incidence. In these cases, the most common presentation is an acute intestinal obstruction (90%). Surgery is usually curative, and follow up is important as the symptoms of Crohn's disease can hide any recurrence.
Topics: Carcinoid Tumor; Crohn Disease; Delayed Diagnosis; Disease Susceptibility; Humans; Ileal Neoplasms; Intestinal Obstruction; Lymphatic Metastasis; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Invasiveness
PubMed: 26319938
DOI: 10.1016/j.circir.2015.05.047 -
Annals of Oncology : Official Journal... 2002
Review
Topics: Adolescent; Adult; Age Distribution; Aged; Biopsy, Needle; Combined Modality Therapy; Female; Germany; Germinoma; Humans; Incidence; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Staging; Prognosis; Retroperitoneal Neoplasms; Risk Assessment; Sex Distribution; Survival Analysis; Survival Rate; Testicular Neoplasms
PubMed: 12401700
DOI: 10.1093/annonc/mdf669 -
World Journal of Surgical Oncology Jan 2017Optimal extent of surgery remains controversial in types 2 and 3 adenocarcinoma of esophagogastric junction (AEG). We aimed to determine whether the extended procedure...
BACKGROUND
Optimal extent of surgery remains controversial in types 2 and 3 adenocarcinoma of esophagogastric junction (AEG). We aimed to determine whether the extended procedure including mediastinal lymphadenectomy is essential in all patients with AEG by comparing prognosis and recurrence of proximal gastric adenocarcinoma based on total gastrectomy with intra-abdominal lymphadenectomy.
METHODS
The clinicopathologic characteristics of 672 patients (type 2: 90, type 3: 211, upper third of the stomach: 371 cases) who underwent curative total gastrectomy with lymphadenectomy between 2003 and 2009 were reviewed.
RESULTS
Recurrence was observed in 36.7, 16.1, and 16.1% of cases of type 2 AEG, type 3 AEG, and cancer of the upper third of the stomach, respectively. The 5-year disease-free survival rates were 62.6, 82.5, and 84.6%, respectively. Subgroup analysis revealed that in early cancers, there was no difference in survival between the groups (93.2 vs. 96.7 vs. 98.7%) but in advanced cancers, there was a difference (47.9 vs. 75.4 vs. 71.8%, P < 0.001). There was no survival difference in stage 1 (97.5 vs. 98.7 vs. 98.3%), but, in stage 2, type 2 AEG had a worse prognosis (41.9 vs. 92.1 vs. 83.0%). Types 2 and 3 advanced AEG had higher rates of locoregional recurrence, especially in the vicinity of the esophagojejunostomy and mediastinal lymph nodes compared to proximal gastric cancer.
CONCLUSIONS
Total gastrectomy without mediastinal lymphadenectomy might produce favorable outcomes in early AEG and acquisition of a greater length of proximal margin, and removal of mediastinal lymph nodes might be helpful in advanced cancers.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Esophageal Neoplasms; Esophagectomy; Esophagogastric Junction; Female; Follow-Up Studies; Humans; Lymph Node Excision; Lymph Nodes; Male; Mediastinal Neoplasms; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 28100248
DOI: 10.1186/s12957-016-1088-x -
Journal of Nuclear Medicine : Official... Aug 1993
Topics: Adolescent; Child; Female; Gallium Radioisotopes; Hodgkin Disease; Humans; Male; Mediastinal Neoplasms; Neoplasm Recurrence, Local; Radiography; Radionuclide Imaging; Thallium Radioisotopes; Thymus Hyperplasia
PubMed: 8326394
DOI: No ID Found -
Frontiers in Endocrinology 2020Mediastinal parathyroid carcinoma (PC) is a rare entity in primary hyperparathyroidism. The aim of this report is to demonstrate a case of mediastinal PC, and to provide... (Review)
Review
Persistent Hypercalcemia Crisis and Recurrent Acute Pancreatitis Due to Multiple Ectopic Parathyroid Carcinomas: Case Report and Literature Review of Mediastinal Parathyroid Carcinoma.
Mediastinal parathyroid carcinoma (PC) is a rare entity in primary hyperparathyroidism. The aim of this report is to demonstrate a case of mediastinal PC, and to provide a systemic literature review of this rare condition. A 34-year-old woman who had already undergone two cervical operations for hyperparathyroidism suffered from another recurrence, presenting with recurrent acute pancreatitis and persistent hypercalcemic crisis. Technetium-99 methoxyisobutylisonitrile imaging (MIBI) and computed tomography scanning (CT) identified three possible parathyroid tumors, one of which was the recurrence of residual tumor locating in the thyroid region, while the other two were ectopic tumors locating in the suprasternal fossa and thymus region, respectively. Pathological examination confirmed the diagnosis of PC. We conducted a systemic literature review by searching the PubMed MEDLINE from 1951 to 2019 for studies of all types in the English language only, using terms "mediastinal, mediastinum, parathyroid, carcinoma." Including our reported case, a total of 21 cases with ectopic mediastinal PCs were assessed for demographic data, tumor location and size, biochemical findings, and symptomatology, etc. Two thirds of the patients were men, with a mean age of 44 years old, a mean serum calcium of 14.2 mg/dl, and a mean serum intact parathyroid hormone of 1,216 pg/ml. We identified 89.5% of carcinomas in the anterosuperior mediastinum, and 10.5% in the middle mediastinum, with a mean diameter of 54 mm, and a mean weight of 216 g. MIBI and CT were the most commonly used methods to localize these mediastinal tumors, with 69.2 and 100% sensitivity, respectively. Half of the patients underwent more than one operation. Diagnosis and treatment of mediastinal PCs represent a challenge. Early suspicion, appropriate preoperative localization studies, and the cooperation of endocrinologists and surgeons are crucial in the effective management.
Topics: Adult; Female; Humans; Hypercalcemia; Mediastinal Neoplasms; Neoplasm Recurrence, Local; Pancreatitis; Parathyroid Neoplasms
PubMed: 33013712
DOI: 10.3389/fendo.2020.00647 -
BMC Pulmonary Medicine Dec 2016Staging of mediastinal lymph nodes in non-small cell lung cancer (NSCLC) is mandatory. The maximum Standard Uptake Value (SUVmax) obtained using F-18 fluorodeoxyglucose...
BACKGROUND
Staging of mediastinal lymph nodes in non-small cell lung cancer (NSCLC) is mandatory. The maximum Standard Uptake Value (SUVmax) obtained using F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) is the best non-invasive technique available for this evaluation, but its performance varies from center to center. The aim of the present study was to identify FDG-PET predictors of mediastinal malignancy that are able to minimize intercenter variability and improve the selection of subsequent staging procedures.
METHOD
A multicenter study of NSCLC patients staged through FDG-PET and endobronchial ultrasonography with needle aspiration (EBUS-NA) was performed using therapeutic surgery with systematic nodal dissection as gold standard. Intercenter variability and predictive power for mediastinal malignancy of different FDG-PET measures were assessed, as well as the role of these measures for selecting additional staging procedures.
RESULTS
One hundred and twenty-one NSCLC patients, of whom 94 (72%) had ≥1 hypermetabolic spots in the mediastinum, were included in the study. Mean SUVmax of the primary tumor was 12.3 (SD 6.3), and median SUVmax of the highest hypermetabolic spots in the mediastinum was 3.9 (IQR 2.4-7). Variability of FDG-PET measures between hospitals was statistically significant (p = 0.016 and p < 0.001 respectively), but lost significance when SUVmax in the mediastinum was expressed as a ratio or a subtraction from the primary tumor (SUVmax mediastinum/tumor, p = 0.083; and SUVmax mediastinum - tumor, p = 0.428 respectively). SUVmax mediastinum/tumor showed higher accuracy in the ROC analysis (AUC 0.77 CI 0.68-0.85, p < 0.001), and showed predictive power for mediastinal malignancy when using a 0.4 cutoff (OR 6.62, 95%CI 2.98-14.69). Sensitivities and negative predictive values of clinical staging through EBUS-NA attained values ranging between 57% and 92% after FDG-PET, which improved with additional techniques when the tumor had a diameter >3 cm and/or a SUVmax mediastinum/tumor ratio >0.4.
CONCLUSION
The SUVmax mediastinum/tumor ratio is a good predictor of regional tumor extension in NSCLC. This measure is not influenced by intercenter variability and has an accuracy of over 70% for the identification of malignancy when using a 0.4 cutoff.
Topics: Aged; Carcinoma, Non-Small-Cell Lung; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Female; Fluorodeoxyglucose F18; Humans; Logistic Models; Lung Neoplasms; Lymph Nodes; Male; Mediastinal Neoplasms; Mediastinum; Middle Aged; Neoplasm Staging; Positron-Emission Tomography; Prospective Studies; ROC Curve; Spain
PubMed: 27931198
DOI: 10.1186/s12890-016-0338-6