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Primary malignant extra gonadal germ cell tumours (GCT) of mediastinum are very rare tumours, comprising 1 - 4 % of all mediastinal tumours.1 Even though they are rare tumours of mediastinum, they should always be considered in the differential diagnosis of mediastinal tumours in young adults. Primary malignant GCT can be divided into 2 groups: Seminomatous and non-seminomatous tumours. Mature teratomas (non-seminomatous) are the most common primary malignant germ cell tumours of mediastinum.2 Non-seminomatous tumours tend to be more aggressive than seminomatous tumours present as heterogeneous masses with areas of necrosis and show local invasion. Seminomas usually present as lobulated homogeneous masses and rarely show signs of local invasion. These tumours usually present with asymptomatic lesions or with symptoms due to compression of adjacent structures and usually have a better prognosis than non-seminomatous tumours. Here, we present a case of primary malignant extra gonadal seminoma with sternal involvement.

Multilocular thymic cyst (MTC) and germ cell tumors are common diseases that impact the mediastinum. Correctly diagnosing these diseases can be difficult because several other conditions can mimic them. We report a male patient with MTC associated with mediastinal seminoma. A needle biopsy of the mediastinal tumor revealed numerous epithelioid cell granulomas that mimicked sarcoidosis or mycobacterial infection. However, large atypical cells positive for Oct3/4 and KIT were noted between the granulomas; thus, we diagnosed the patient with mediastinal seminoma. The resected tumor, after chemotherapy, consisted of multiple cystic lesions, and a residual germ cell tumor was first considered. However, thymic medulla-specific elements, namely, POU2F3-positive thymic tuft cells and rhabdomyomatous myoid cells accompanying the epithelium, led to the correct diagnosis of MTC. Our case underscores the importance of recognizing the histological features associated with mediastinal seminoma and provides novel findings for MTC pathogenesis, namely, the presence of thymic tuft cells.

Background This study was performed to clarify the treatment outcome of patients with primary mediastinal germ cell tumors (PMGCTs), focusing on the clinical manifestations and management during definitive therapy and long‐term follow‐up. Methods In this study, we retrospectively reviewed the medical records of patients with PMGCTs treated at Shinshu University School of Medicine, and examined the clinical profiles and treatment outcomes of 22 patients (mean age of 29 years) with primary mediastinal GCTs treated at our hospital between 1983 and 2019. Results Five patients were diagnosed with pure seminoma and 17 had nonseminomatous GCT. A total of 21 patients were treated with cisplatin‐based chemotherapy and 15 patients (68.2%) underwent thoracic surgery after chemotherapy. Although all cases of nonseminomatous GCT were negative for tumor markers after cisplatin‐based chemotherapy, two cases showed variable GCT cells and two had somatic components (angiosarcoma and rhabdomyosarcoma) in resected specimens. Three relapsed soon after surgery. Growing teratoma syndrome developed during chemotherapy in four cases. Urgent thoracic surgery was performed in three patients, but one case was inoperable. The calculated 10‐year overall survival rates were 100% in mediastinal seminoma and 64.7% in NSGCT. During follow‐up, second non‐GCT malignancies developed in three patients (colon cancer, 190 months; thyroid cancer, 260 months; non‐small cell lung cancer, 250 months after the initial chemotherapy) and one patient with primary mediastinal seminoma was associated with multiple type I endocrine tumors. Conclusions Our experiences demonstrated that long‐term survival and/or cure can be achieved with adequate chemotherapy followed by local surgical treatment even in patients with mediastinal GCTs. However, the clinical manifestations and biological behaviors during and/or after chemotherapy were complex and varied. In addition, the development of secondary malignancies should be taken into consideration for long‐term follow‐up. Clinicians should be aware of the various clinical features and secondary malignancies in primary mediastinal GCTs., The clinical profiles and treatment outcomes of 22 patients (mean age of 29 years) with primary mediastinal germ cell tumors (GCT) treated at our hospital between 1983 and 2019 were examined in this retrospective study. The calculated 10‐year overall survival rates were 100% in mediastinal seminoma and 64.7% in NSGCT. The clinical manifestations and biological behaviors during and/or after chemotherapy were complex and varied.

The Primary Mediastinal Seminoma (PMS) is a rare extragonadal malignant germ-cell tumor. We report a clinical case of our practice, which is the reason for this literature review and try to find the optimal multimodal therapeutic option and the role of radiotherapy.

Mediastinal biopsy is essential for the clinical diagnosis of mediastinal disease. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a well-established approach for obtaining diagnostic samples from mediastinal masses or enlarged lymph nodes which is proven to be minimally invasive and effective. However, the insufficiency of intact samples acquired might restrict the diagnostic efficacy of EBUS-TBNA for mediastinal lesions such as rare malignancy and granulomatous disorder. We here present an EBUS-guided approach for the cryobiopsy of mediastinal diseases that is capable of providing larger amounts of intact tissue with few observed complications.

The azygos system is the most important pathway for decompression of the superior vena cava (SVC) when a blood flow obstruction to the right atrium is present. Thoracic and mediastinal malignancies, mainly lung cancers, are responsible for 60%-85% of superior vena cava syndrome (SVCS) cases. An uncommon origin of SVCS is primary malignant mediastinal germ cell tumor (PMMGCT) which represent 1%-4% of all mediastinal tumors and can be divided into two broad groups: seminomas and nonseminomatous germ cell tumors (NSGCTs). Primary mediastinal seminomas clinical presentation is often nonspecific, even if the majority of patients present with superior vena cava involvement. Here, we present the radiologic features of asymptomatic azygos system overflow in a patient with primary mediastinal seminoma.

Background Chylothorax is a life-threatening pathological condition associated with significant morbidity and mortality. If chyle leakage does not close spontaneously with medical therapy, surgical treatment is inevitable. Herein, we reported a case of spontaneous persistent chylothorax from mediastinal seminoma that was successfully closed between the descending thoracic aorta, and the vertebral column through a left mini-thoracotomy. Case presentation A 24-year old man with mediastinal seminoma was referred to our attention for management of high output persistent chylothorax (> 800 ml/24 h for 30 days) that did not close with conservative treatment. As the leak was isolated within left upper chest cavity, we planned to close the thoracic duct via Poirier’s triangle by uniportal thoracoscopy. However, the long conservative treatment favoured the formation of multiple, tenacious, and bleeding adhesions that made unfeasible thoracoscopy. A conversion to mini-thoracotomy was performed; by the incision of the posterior parietal pleura, the thoracic duct was isolated and ligated behind the thoracic aorta, in an anatomical space delimited by the 4th and the 5th posterior intercostal arteries and the vertebral column. Conclusions Complete resolution of chylothorax was obtained the day after. Patient was discharged on post-operative day 5, and no recurrence was observed during the follow-up. Electronic supplementary material The online version of this article (10.1186/s13019-019-0917-8) contains supplementary material, which is available to authorized users.

Primary mediastinal germ cell tumors are a rare finding, and one third of them are seminomas. Seminomas are found in the anterior mediastinum, whereas they are extremely rare within the posterior compartment. Most clinicians would not consider a primary seminoma in the differential diagnosis of a posterior mediastinal mass, as only two cases have been reported in literature. Here we present the case of a 57-year-old male with a primary seminoma arising in the left posterior mediastinum. He was asymptomatic and the mass was an incidental finding. Positron emission tomography (PET) revealed a small area with an avid tracer uptake. Transthoracic needle aspiration led to a non-diagnostic result. Due to the strong suspect of malignancy, a surgical excision was chosen to obtain a diagnosis. He underwent complete excision, and pathology report demonstrated a mediastinal seminoma. Subsequent further staging did not reveal any other location of the disease. Given the complete excision of the primary tumor, active surveillance was the treatment of choice. The patient is free of disease 48 months after diagnosis.

BackgroundCancer-associated thrombosis is a worrisome complication for patients receiving chemotherapy. Management of anticoagulant therapy is complex in cancer patients because of comorbidities such as thrombocytopenia, renal impairment and a hypercoagulability due to the cancer itself. Therefore, tailoring anticoagulant treatment is challenging. Case presentationWe describe the case of a 35-year-old male patient with primitive mediastinal seminoma undergoing treatment with Bleomycin, Etoposide and Cisplatin (BEP protocol) who presented with marked upper-extremity vein thrombosis unresponsive to low molecular weight heparin. After unsuccessful therapy with heparin, therapy was switched to edoxaban, a direct oral anticoagulant, which was able to dissolve the huge thrombus, suggesting that direct oral anticoagulants (DOACs) may be a reliable option especially in long-term anticoagulant treatments like in this case.A short review of the literature on cancer-related thrombosis and the use of direct oral anticoagulants in malignancies is also presented. ConclusionsOur case suggests that in severe and extended cancer-associated thrombosis, instituting treatment with direct oral anticoagulants instead of low molecular weight heparin can be an effective, safe and practical option. This choice is preferred by most patients as shown by their proper adherence to treatment.

Mediastinal seminomas are rare neoplasms that can be found incidentally in asymptomatic patients. However, a few cases may present in the emergency room with mild to severe respiratory and/or cardiovascular symptoms. This can occur when the tumor grows large enough to cause compression and obstruction of the various structures present in the thorax. Here we present a case of a large medium mediastinal seminoma that grew to the extent of causing pulmonary artery compression which led to chronic passive backflow through the hepatic veins and hepatic congestion. This case was remarkable as well for the presence of testicular microlithiasis, a rare feature with unknown significance to date.

Background First described in 1955 Primary mediastinal seminomas are rare. Only 1–4% of mediastinal tumours are germ cell tumors; majority of which are teratomas. They typically present in men aged between 20 and 40 years. Very few cases are reported in the literature. Florid follicular lymphoid hyperplasia can obscure the malignant cells and is a rarer finding still. We present a rare case of a 48 year old man with a primary mediastinal seminoma with florid follicular lymphoid hyperplasia; found following excision of a clinically presumed thymoma. Case presentation A 48 year old man was referred for excision of a thymic mass. The presumed diagnosis was a thymoma; following preoperative investigations. The mass was incidentally found on a radiological imaging. However, the patient did report mid-sternal discomfort on lying flat and breathlessness. The patient underwent a thymectomy via a partial median sternotomy with good recovery. Histological assessment was that the mass was in fact a primary mediastinal seminoma with florid follicular lymphoid hyperplasia. A primary testicular malignancy was excluded and the patient required no further oncological treatment. Conclusions Only 11 cases have previously been reported of primary mediastinal seminoma with florid follicular lymphoid hyperplasia. Although rare, a primary mediastinal seminoma should be considered as a differential diagnosis for presentations with a thymic mass. Tumour markers can be helpful, however are only positive in third of cases. Ultrasound imaging of the gonads is essential to exclude a primary gonadal lesion. Pure seminomas are radiotherapy and chemotherapy sensitive however the mainstay treatment of primary mediastinal seminomas remains surgical excision. Radiotherapy is reserved postoperatively for incomplete surgical margins.

Purpose Primary mediastinal germ cell tumours (PMGCTs) are rare; with limited data available about their outcomes and optimal treatment in the low middle income countries setting. We studied the clinical profile of patients with PMGCT treated at our centre in order to estimate their survival outcomes and to identify prognostic factors affecting the same. Patients and methods Fifty-seven patients with PMGCTs treated between April 2001 and June 2019 were included. Baseline characteristics, details of first line chemotherapy, response rates, toxicity and surgical outcomes were noted. Progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Results Among 57 male patients (seminoma = 20 and nonseminomatous = 37), the median follow-up was 10 months (range: 1-120 months). For mediastinal seminoma, 9 (45%) and 11 (55%) patients had good and intermediate risk disease, respectively. Nineteen patients (95%) received BEP (Bleomycin, etoposide and cisplatin) chemotherapy. 94.7% had partial responses and median event-free survival was not reached. All patients were alive and disease free at 2 years. For primary mediastinal nonseminomatous germ cell tumours (PMNSGCTs), all patients were poor risk. Thirty-four (91.8%) received BEP/EP chemotherapy as first line. Responses were PRM+ (partial response with elevated markers) in 7 (20.5%) and PRM- in 12 (35.2%). The incidence of febrile neutropenia was 50% and 55.8% in seminole and PMNSGCT, respectively. The median OS was 9.06 months and median PFS was 4.63 months for PMNSGCT. The proportion of patients alive at 1 year and 2 years were 35% and 24.3%, respectively. Conclusion Primary mediastinal seminomas are rarer and have better survival outcomes. Treatment of PMNSGCT is still a challenge and is associated with poorer survival outcomes.

Objectives. Primary mediastinal seminoma (PMS) was an uncommon carcinoma, and the appropriate treatment remained controversial due to the low incidence. We aimed to investigate the demographics and tumor biological characteristics to determine the potential effective treatment and predict the prognosis. Methods. Patients diagnosed with PMS were selected between 1975 and 2016 from Surveillance, Epidemiology, and End Results (SEER) database. Kaplan–Meier analysis and Cox proportional hazard model were conducted to determine the prognostic factors, and nomograms were employed to visually predict the prognosis. Concordance index (C-index), calibration curve, and receiver operating characteristic (ROC) curve were conducted to validate the prediction model. Results. A total of 476 patients were included with a median age of 31 years (range, 2–76 years), and a median size of the tumor was 11.6 cm (range, 0.2–24.0 cm). The 5- and 10-year overall survival (OS) rates were 70.4% and 68.4%, respectively. Age, the extent of the primary site, metastatic status, and surgery performance were independent prognostic factors. Not received surgery was considered a poor prognostic factor for OS (HR, 1.86; 95% CI, 1.13–3.03; P = 0.013 ). The C-index was 0.733 (95% CI, 0.685–0.781) and 0.819 (95% CI, 0.737–0.901) for internal and external validation for predicting OS, respectively. The area under the ROC curve (AUC) was 0.743 (95% CI, 0.681–0.804) for predicting OS (sensitivity, 0.532; specificity, 0.887) in the training cohort. Conclusions. The nomogram could efficiently predict the survival of patients with PMS. Surgery was the potential effective treatment, and chemotherapy was strongly recommended for patients over 40 years.

Germ cell tumors are neoplasms that typically occur in the gonads where they originate from multipotential primitive germ cells. Occasionally, these tumors are found to arise in extragonadal sites, usually along midline structures, such as the pineal gland, retroperitoneum, mediastinum, and sacral area. Extragonadal germ cell tumors are thought to develop from germ cells that are misplaced during their migration from the yolk endoderm to the gonads during early embryogenesis and can display the same spectrum of neoplasms native to the testis and ovary. Primary mediastinal germ cell tumors are rare, accounting for approximately 1–3% of all germ cell tumors, 15% of all anterior mediastinal tumors in adults and 24% in children. Germ cell tumors with benign behavior are more common in females and children, while the vast majority of malignant mediastinal germ cell tumors occur in older adolescent and adult male patients. In the mediastinum, these tumors are characteristically located in the anterior compartment, within or close to the thymic gland, although lesions arising in the posterior mediastinum, pericardium, and aortic adventitia have been reported.

Primary mediastinal seminoma is a rare extragonadal germ cell tumour that mainly occurs in males. The present study reports the case of a 27-year-old woman that presented with superior vena cava syndrome and a large mass in the mediastinum, which was diagnosed as primary female mediastinal seminoma. The patient received 6 cycles of cisplatin-based chemotherapy [4 cycles BEP chemotherapy (120 mg cisplatin, 0.45 g etoposide and 60 mg bleomycin, once every 3 weeks); 2 cycles IEP chemotherapy (120 mg cisplatin, 100 mg epirubicin and 6 g ifosfamide, once every 3 weeks)] and the patient showed an increase in the size of the mediastinal mass and hydropericardium, indicating a resistance to chemotherapy. Radiotherapy to the mediastinum (50 Gy over 18 fractions) and pericardium (30 Gy over 18 fractions) was performed. Following radiotherapy, the patient was considered to have a complete response to the treatment, and subsequent to a 5-year follow-up, no recurrence or metastasis was identified. To the best of our knowledge, no similar cases have been reported in the literature at present.

It is well-known that cardiovascular disease is the most common cause of sudden unexpected death, especially in the young. However complete postmortem investigation performed by qualified forensic pathologists is crucial in establishing the exact cause and manner of death. Sudden death caused by a cardiac tamponade occurring secondarily to a primary mediastinal tumor is extremely unusual. An autopsy on a 25-year-old man who died unexpectedly discovered a massive hemopericardium in association with an anterosuperior mediastinal mass. The tumor had invaded the pericardium and an ulcerative surface with hemorrhagic necrosis on the cut surfaces of the intrapericardial tumor was revealed. All cardiac chambers and intrathoracic great vessels were intact and both testes were normal. Histologic examination confirmed the diagnosis of primary mediastinal pure cell seminoma. Diffuse pulmonary edema, as well as generalized congestion of internal organs, was observed as evidence of acute decompensated heart failure. To our knowledge, this is the first reported case of unexpected death due to massive hemopericardium as a result of primary mediastinal seminoma.

Although primary central nervous system (CNS) germ cell tumors (GCTs) are one of the most treatable types of malignant brain tumor, a subset of patients remain resistant to standard chemotherapy. Gain-of-function mutations of the c-Kit gene, and KIT protein expression, have been observed in a number of GCTs, including testicular seminoma, ovarian dysgerminoma and mediastinal seminoma in various ethnic groups. Although a small number of studies have reported the role of c-Kit in CNS GCTs, few have focused on Chinese patients exhibiting CNS GCTs. In the present study, the frequency and location of c-Kit mutations and KIT protein expression levels in CNS GCTs were investigated in 30 patients, between January 1994 and October 2014. Immunohistochemical assays suggested that KIT protein expression was present in 59.1% patients (66.7% in males and 42.9% in females); however, no statistically significant correlation was identified between KIT protein expression and patient clinicopathological features. By performing PCR amplification and direct sequencing, 4 mutational hot spots of the c-Kit gene (exons 9, 11, 13 and 17) were examined, and c-Kit gene mutation was identified in 1/17 (5.9%) CNS germinoma cases. This mutation was located in exon 11 at codon 557-558 WK (Tryptophan-Lysine). No c-Kit gene mutations were detected in non-germinomatous GCTs. Imatinib, a tyrosine kinase inhibitor, may be an effective treatment against standard chemotherapy-resistant CNS germinoma patients exhibiting c-Kit mutations.

Background: The objective of this study was to evaluate the long term treatment results of patients with primary mediastinal seminoma. Methods: Sixteen patients aged 21–46 diagnosed with primary mediastinal seminoma between 1983 and 2014. Mean size of the tumor was 65 cm 2 . In all patients gonadal involvement was excluded. In 6 patients, metastases to regional lymph nodes were found and none of them presented with distant metastases. HCG was elevated in 6 patients (38%). Eight patients underwent surgery as first-line of the treatment: 7 partial and 1 complete resection of the tumor. Chemotherapy (CTH) and radiotherapy (RTH) were methods of treatment in 14 cases. Tumor was irradiated to total dose of 36.0–50.4 (median 42.5) Gy. In statistical analysis overall survival (OS) was calculated using Kaplan-Meier method. Results: During median time of 11 years of the follow-up, complete or partial regression (PR) of the tumor was seen in all patients after primary treatment. Recurrence of the tumor was seen in 3 patients. The 5-, 10- and 15-year local control rates were 75%. One was treated with CTH, other two with RTH. All of them responded with complete regression (CR) of the tumor. Three patients died during the follow-up. All others are alive without disease. The 5-, 10- and 15-year OS was 100%, 91% and 91% respectively. Conclusions: Chemoradiotherapy of primary mediastinal seminoma gives satisfactory treatment results with good local control rate. The treatment outcome is comparable to primary testicular seminoma.

Mediastinal seminoma is an uncommon tumor that accounts for 25% of primary mediastinal germ cell tumors, which in turn comprise fewer than 5% of all germ cell tumors. Although CT normally shows a solid, lobulated tumor, mediastinal cystic seminoma has rarely been described. Here, we report a 24-year-old man who presented with a mediastinal cystic tumor that was resected after an 18-month delay via video-assisted thoracoscopic surgery while in the supine position; the procedure involved lifting the chest wall with a subcutaneous Kirschner wire. Pathological examination revealed a mediastinal cystic seminoma. No evidence of recurrence has been noted during 25 months of follow-up. Mediastinal cystic seminoma should be considered in the differential diagnosis of cystic lesions of the mediastinum. Moreover, video-assisted thoracoscopic resection may be an appropriate option for the diagnosis and treatment of such lesions.

Germ cell tumors can occur in the mediastinum. They account for about 20% of tumors at this location. The majority are located in the anterior mediastinum and usually occur in young adult males. Extension of mediastinal germ cell tumors into the neck with mass formation is a very rare and unusual event. Herein, we report a case of a 34 year old male who presented with a progressively enlarging neck mass. Fine Needle Aspiration (FNA) was performed as initial evaluation and showed cellular smears comprising atypical large cells with prominent irregular nucleoli and moderate amount of cytoplasm with lymphocytic infiltrate and some epithelioid granulomas in the background. The mass was misdiagnosed initially on the cytology smears as anaplastic thyroid carcinoma. The subsequent tissue core biopsy showed sheets and nests of atypical cells admixed with ill-defined granulomatous inflammation. By immunohistochemistry, the tumor cells were immunoreactive with SALL4, PLAP and OCT3/4, compatible with malignant germ cell tumor, seminomatous type. It is very rare for patients with primary mediastinal seminoma to present initially with a neck mass. Fine Needle Aspiration (FNA) of this "neck mass" can lead to misinterpretation of findings due to similarities in cytological features between malignant germ cell tumors and other undifferentiated malignant neoplasms and the diagnosis, therefore, can be very challenging.