Mediastinal Sarcoma Research Articles

A case report of late detection of primary mediastinum herm cell tumor

Germ cell tumors are rare malignant neoplasms. Extragondal localization accounts for about 10% of all germ cell tumors, with up to 3% being mediastinal. The low incidence, along with a nonspecific clinical presentation, complicates initial diagnosis and leads to delays in diagnosis and the initiation of specific treatment. Primary care plays a crucial role in detecting such tumors and in patient routing. At the outpatient stage, the patient should be examined and referred to a specialized facility for specific treatment as quickly as possible. For differential diagnosis at the initial stages between mediastinal germ cell tumors and lymphoproliferative diseases, mediastinal sarcomas, thymomas, and others, monitoring tumor markers is necessary: alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (beta-HCG), and lactate dehydrogenase (LDH). When these markers are elevated and time is critical, it is permissible to start specialized antitumor treatment without histological confirmation.Currently, it is also important to consider the role of the new coronavirus infection, which complicates differential diagnosis at the initial stages. This article presents a clinical case of late detection of a germ cell tumor and attempts to conduct systemic therapy in the context of the disease’s complicated progression.

Emergent Total Endovascular Arch Repair for Contained Aortic Arch Rupture: Another Tool in the Box.

To date, emergent total endovascular aortic arch repair has not been described in the literature. We present a 67-year-old female with a poorly differentiated posterior mediastinal sarcoma. Imaging obtained was concerning for intravascular extension of the tumor into the thoracic aorta. While awaiting radiation therapy, the patient complained of worsening chest and arm pain, vital signs demonstrating tachypnea and hypoxia. Subsequent imaging revealed an increase in vascular erosion, concerning for a contained rupture, with complete obliteration of the left mainstem bronchus. The patient was emergently taken for percutaneous endovascular repair of her aortic arch. A three-vessel physician modified fenestrated graft was created and deployed with concurrent stenting of the innominate, left carotid, and left subclavian arteries. Interval computed tomography angiography revealed patency in all stented vessels, with no endoleak and no evidence of pseudoaneurysm. The patient was able to undergo chemotherapy with favorable decrease in tumor burden. Total endovascular aortic arch repair, when planned carefully, is an attractive option in high-risk patients who are otherwise not ideally suited for open total arch replacement.

Replacement of the descending aorta for mediastinal sarcoma: An unusual approach

We report the case of posterior mediastinal myxoid liposarcoma with right intra-pleural development operated through right thoracotomy. We observed a significant invasion of a segment of the descending aorta though there was no evidence of tumor infiltration in preoperative imaging

Pericardiotomy under clamshell approach for large posterior mediastinal sarcoma: a case report

BackgroundLarge mediastinal tumors are sometimes difficult to completely resect because they are surrounded by vital organs. We present the case of mediastinal liposarcoma successfully resected by the clamshell approach with a posterior pericardial wall incision.Case presentationAn 81-year-old man visited the hospital because of a prolonged cough. Imaging studies revealed a large tumor of the left posterior mediastinum. The tumor surrounded the left inferior pulmonary vein and was suspected of invading the left lower lobe and esophagus. Complete resection of a large mediastinal tumor invading the left inferior pulmonary vein and esophagus was achieved by adding a posterior pericardial wall incision to the clamshell approach under cardiopulmonary bypass. Histopathological diagnosis was well-differentiated liposarcoma. Postoperative course was good, and the patient is alive without any sign of recurrence for 53 months.ConclusionsThis approach could represent a novel method to safely and effectively remove large mediastinal tumors invading the lung and esophagus.

METASTATIC FIBROSARCOMA IN ORAL CAVITY: CASE REPORT

A 42-year-old female patient reported diffuse toothache associated with difficulty chewing and a lesion in the oral cavity for approximately 3 months. The patient's medical history showed a diagnosis of mediastinal sarcoma and she was undergoing chemotherapy. Extraoral examination showed no changes. In the oral cavity, a nodular lesion with an ulcerated surface in the right retromolar triangle associated with the occlusion trauma promoted by element 18 was observed. Panoramic radiography showed a radiolucent lesion of imprecise limits without promoting root resorption in adjacent teeth. Bone scintigraphy revealed a blastomatous lesion in the mandible and oral cavity, suggesting bone metastasis. Incisional biopsy was performed, and histological sections stained in hematoxylin and eosin showed low-grade ulcerated fusocellular sarcoma, consistent with fibrosarcoma. The patient followed chemotherapy with doxorubicin and dacarbazine but died from treatment complications. A 42-year-old female patient reported diffuse toothache associated with difficulty chewing and a lesion in the oral cavity for approximately 3 months. The patient's medical history showed a diagnosis of mediastinal sarcoma and she was undergoing chemotherapy. Extraoral examination showed no changes. In the oral cavity, a nodular lesion with an ulcerated surface in the right retromolar triangle associated with the occlusion trauma promoted by element 18 was observed. Panoramic radiography showed a radiolucent lesion of imprecise limits without promoting root resorption in adjacent teeth. Bone scintigraphy revealed a blastomatous lesion in the mandible and oral cavity, suggesting bone metastasis. Incisional biopsy was performed, and histological sections stained in hematoxylin and eosin showed low-grade ulcerated fusocellular sarcoma, consistent with fibrosarcoma. The patient followed chemotherapy with doxorubicin and dacarbazine but died from treatment complications.

Primary Endobronchial and Mediastinal Synovial Sarcoma in a Young Female- Case Report

Primary Endobronchial and Mediastinal Synovial Sarcoma in a Young Female- Case Report

A Case of Primary Synovial Sarcoma of the Posterior Mediastinum with Aortic Wall Infiltration with Complete Imaging Pathway and Histological Diagnosis

Danilo Caudo, Alessandro Santalco, Carmelo Anfuso, Ylenia Zullo, Alessandra Pelle, Caterina Benedetto, Annalisa Militi, Viviane Di Dio, Fabio Italiano, Ugo Barbaro Primary synovial mediastinal sarcomas are aggressive tumors with a very rare incidence. The few cases reported in the literature presented poor and incomplete imaging.

Primary Malignant Synovial Tumor in the Mediastinum

Primary mediastinal synovial sarcomas constitute a rare subset of mediastinal tumors. The diagnosis is often delayed at the time of presentation impacting the five-year survival rate due to its highly aggressive natural history. We report a 22-year old female with a monophasic variant of the primary mediastinal synovial sarcoma.A 22-year-old female, with a two-month history of productive cough, fever, breathlessness, was referred to our center in view of persistent right-sided hydropneumothorax despite multiple thoracocenteses. Examination revealed reduced right-sided air entry with a succussion splash. Imaging suggested a well-defined cystic lesion with fluid, air foci, and multiple septations. A provisional diagnosis of a ruptured hydatid cyst was made and exploratory thoracotomy was planned. Intraoperatively, a well-defined cystic lesion with 350 mL of hemorrhagic fluid, densely adherent to the lung and diaphragm, was found. The biopsy revealed a monophasic spindle cell variant of the primary synovial sarcoma. Follow-up positron emission tomography (PET) on postoperative day 20 showed no residual disease and evidence of metastasis. However, the patient was lost to follow up following one cycle of chemotherapy with ifosfamide.Primary mediastinal synovial sarcomas are aggressive tumors that warrant an early diagnosis for prompt treatment. They usually present with non-specific respiratory symptoms. The gold standard of diagnostic modalities is a molecular panel looking for the translocation - t(X;18)(p11;q11). However, in low-middle income countries, a biopsy may be more practical, as they are cost-effective. The treatment is surgical resection, with combined chemotherapy and radiotherapy if metastases are present.Our case emphasizes the early detection of this lesion, its mimicry with other lesions, and the impact of early diagnosis.

Primary synovial sarcoma of the mediastinum: a rare tumor diagnosed by endoscopic ultrasound-fine needle biopsy (EUS-FNB)-Cytomorphologic, immunohistochemical, and molecular analysis.

Primary synovial sarcoma is exceedingly rare in the mediastinum. The differential diagnosis of this rare tumor is complex as a wide array of primary and metastatic tumors occur in this site.A definite diagnosis might be challenging even after tissue sampling. Immunohistochemistry can be very helpful and supportive for the diagnosis, but still inadequate in some cases as these tumors can mimic histopathologically other soft tissue tumors. Hence, in some case, an advanced pathological molecular analysis is needed.Endoscopic ultrasound (EUS) is an important diagnostic tool for mediastinal tumors. While EUS-fine needle aspiration (EUS-FNA) samples are usually inadequate for advanced pathological analysis, tissue acquisition by the newer generation of EUS-fine needle biopsy (EUS-FNB) needles might be sufficient.Here, we present the first report on primary mediastinal synovial sarcoma diagnosed by an immunohistochemical and FISH analysis performed on EUS-FNB tissue sample.

Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the\xa0literature

BackgroundLow-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS.Case presentationA 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery.ConclusionIn conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences.

The role of molecular pathology in mediastinal sarcomas.

Mediastinal sarcomas represent rare neoplasms of mesenchymal origin. Most published data on mediastinal sarcomas is primarily derived from small series and case reports. Although rare, primary mediastinal sarcomas have a clinically aggressive course with worse 10-year survival rates than other types of mediastinal tumors, highlighting the importance of adequate diagnosis of these lesions. The diagnosis of mediastinal sarcomas is complicated by the varied histologic subtypes of tumors that can occur and which can sometimes display overlapping clinical, morphological, imaging, and immunohistochemical features. Cytogenetic analysis and more recently, molecular techniques, have provided new methods by which these tumors can be differentiated. Sarcomas occurring within the mediastinum are an extremely heterogenous group of tumors, although the specific incidence of the different subtypes of mediastinal sarcomas varies among studies, there is a subset of lesions that appear to occur more commonly across most published studies. These tumors include synovial sarcoma, liposarcoma, malignant peripheral nerve sheath tumor (MPNST), small round blue cell sarcomas (including Ewing sarcoma) and leiomyosarcoma (LMS). Other rare sarcoma subtypes may also less commonly occur within the mediastinum. Many of these sarcomas have specific, recurrent genetic abnormalities that can be identified through cytogenetic and molecular testing allowing for accurate diagnosis. This review aims to cover the role of molecular pathology, specifically with regards to diagnosis, as well as discuss the salient molecular genetic features of the various types of sarcoma that occur within the mediastinum. In addition, the various types of cytogenetic and molecular diagnostic tests available for the diagnosis of different types of sarcomas will be reviewed.

Undifferentiated pleomorphic sarcoma in the anterior mediastinum with a rapidly progressive course: A case report.

A 77-year-old woman with heart failure was admitted to our hospital. Computed tomography (CT) of the chest revealed an anterior mediastinal tumor. CT-guided biopsy revealed a malignant nonepithelial tumor of unknown origin. She was not treated with chemotherapy or radiotherapy because of her poor clinical condition. She died 33 days after admission. Following autopsy, we confirmed that the mediastinal tumor had infiltrated the large blood vessels. After final histological examination, undifferentiated pleomorphic sarcoma was diagnosed. Primary mediastinal sarcomas are very rare; clinicians should be aware of their possibility because some cases may progress rapidly as evidenced in this case.

Mediastinal sarcomas: experience using fine needle aspiration cytopathology.

Fine needle aspiration (FNA) cytology is a sparsely used diagnostic method in the evaluation of mediastinal sarcomas in most medical centers worldwide with most literature citations regarding this category of malignancies consisting of small series and individual case reports. Most of these published studies highlight vascular sarcomas such as epithelioid hemangioendothelioma, and angiosarcoma, various subtypes of liposarcoma including well-differentiated liposarcoma, myxoid liposarcoma, and pleomorphic liposarcoma, malignant peripheral nerve sheath tumor, and sarcomas of uncertain differentiation, primary synovial sarcoma and the Ewing sarcoma family of tumors. This paucity of cytopathology reports regarding mediastinal sarcomas is in marked contrast to the almost daily application of endobronchial ultrasound (EBUS)-guided FNA biopsy for sampling mediastinal lymph nodes and mediastinal masses for primary and metastatic carcinomas which, of course, are considerably more common that any type of sarcoma in this location. EBUS, endoscopic ultrasound-guided (EUS) needle biopsy, and percutaneous image-guided biopsy using either core needle, fine-needle, or both can serve a potentially useful role for diagnostic sampling of mediastinal sarcomas, be they primary or metastatic. This review catalogues much of the published data regarding FNA cytopathology and its application to mediastinal sarcomas. An attempt is made to primarily highlight case series rather than individual case reports; however, due to the paucity of these, case reports are cited and discussed where appropriate.

Isolated Mediastinal Myeloid Sarcoma After NPM1 Positive Acute Myeloid Leukemia

Isolated Mediastinal Myeloid Sarcoma After NPM1 Positive Acute Myeloid Leukemia

Immunotherapy for advanced lung cancer combined with surgery for mediastinal myxofibrosarcoma: a case report

BackgroundIt is unclear whether simultaneous primary neoplasm resection and immunotherapy for advanced lung cancer is safe. We report a case of an elderly man with advanced lung cancer and myxofibrosarcoma.Case presentationThe advanced lung cancer was treated with pembrolizumab, and partial response was achieved in 3 months. However, the mediastinal cyst enlarged rapidly. We resected the mediastinal tumor and diagnosed it as myxofibrosarcoma. The postoperative course was uneventful. Immunotherapy was resumed after the operation without any adverse effects. No recurrence of mediastinal sarcoma or progression of lung cancer was found until the patient died in an accident 8 months after surgery.ConclusionSurgery for mediastinal sarcoma could be performed safely in combination with immunotherapy for advanced lung cancer.

Treatment Approaches and Outcomes for Primary Mediastinal Sarcoma: Analysis of 976 Patients

Primary mediastinal sarcomas are rare and deadly. Our objective was to describe the clinicopathological features, treatment strategies, and overall survival outcomes for a contemporary cohort of patients diagnosed with primary mediastinal sarcoma in the United States. We queried the National Cancer Database for cases of mediastinal sarcoma diagnosed from 2004 to 2012. Five-year overall survival (OS) was examined using the Kaplan-Meier method. Differences in OS were assessed using log-rank analysis and Cox proportional hazards regression. The mean age of diagnosis was 53 years (range, 0 to 90) with a male predominance (59.2%). The most common histological subtype was hemangiosarcoma (27.1%). Fewer than half of patients underwent surgery (48.9%), and 19.7% of patients had no treatment. For all patients, OS was 14.8%. The best unadjusted OS was seen in patients treated with surgery and radiation (40.1%); untreated patients had the worst unadjusted OS (4.2%). Of those who underwent surgery (n= 477, 48.9%), OS was significantly better for those who achieved an R0 resection (30.1% versus 18.9%; p= 0.002). In multivariable analysis, surgery combined with radiation therapy was again associated with the best survival (HR, 0.24; 95% CI, 0.16 to 0.36). Other factors associated with improved OS included younger age, fewer comorbidities, and leiomyosarcoma histology. Worse OS was associated with poorly differentiated or undifferentiated grade, metastases, treatment in the New England region, and having Medicaid or no insurance. Sex and tumor size had no effect on OS. The 5-year OS for primary mediastinal sarcoma is poor. Surgical resection can be successful and should be considered whenever possible.

Primary mediastinal synovial sarcoma: clinical, radiologic, and pathologic features in five cases

Primary mediastinal synovial sarcoma: clinical, radiologic, and pathologic features in five cases

Near-infrared intraoperative imaging during resection of an anterior mediastinal soft tissue sarcoma.

Sarcomas are rare malignancies that are generally treated with multimodal therapy protocols incorporating complete local resection, chemotherapy and radiation. Unfortunately, even with this aggressive approach, local recurrences are common. Near-infrared intraoperative imaging is a novel technology that provides real-time visual feedback that can improve identification of disease during resection. The presented study describes utilization of a near-infrared agent (indocyanine green) during resection of an anterior mediastinal sarcoma. Real-time fluorescent feedback provided visual information that helped the surgeon during tumor localization, margin assessment and dissection from mediastinal structures. This rapidly evolving technology may prove useful in patients with primary sarcomas arising from other locations or with other mediastinal neoplasms.

An analysis of clinical characteristics and patient outcomes in primary mediastinal sarcomas

ABSTRACTBackground: Published data concerning primary mediastinal sarcomas are limited to small-sized retrospective series. This study reviewed the clinical outcomes of these cases from the SEER (surveillance, epidemiology and end results) database.Methods: Primary mediastinal sarcomas (1988 - 2013) were assembled from the SEER database. The incidence and 10-year cancer-specific survival rates were compared to other primary mediastinal malignancies (thymic carcinoma, germ cell tumors, neurogenic tumors, Hodgkin / non Hodgkin lymphomas) as well as to non mediastinal sarcomas.Results: A total of 204 patients were recruited into this cohort. Multivariate analysis showed better overall survival for patients with younger age at diagnosis, low grade [hazard ratio 0.427 (95% CI: 0.224-0.814; P= 0.010)], posterior mediastinum location [hazard ratio 0.458 (95% CI: 0.268-0.781; P= 0.004)], node negative disease [hazard ratio 0.463 (95% CI: 0.232-0.923; P= 0.029)] and surgical treatment [hazard ratio 0.488 (95% CI: 0.336-0.709; P <0.0001)]. Compared to other mediastinal malignancies, primary mediastinal sarcomas have the worst 10-year overall survival. Similarly compared to non mediastinal sarcomas, primary mediastinal sarcomas have worse 10-year overall survival (23% versus 55%).Conclusion: Primary mediastinal sarcoma is a rare entity with worse prognosis compared to non-mediastinal counterparts. Surgical resection plays a particularly important role in the management.

Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy

BackgroundClear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of the melanocyte master transcription factor and drives the same down-stream pathways in CCS and melanoma leading to significant clinical parallels between these malignancies. Striking success of immune checkpoint blockade in melanoma has promoted interest in immunotherapy of CCS.Case presentationWe report the first complete clinical response of a bulky chest wall recurrence of mediastinal CCS in a young woman to anti-PD1 checkpoint blockade with pembrolizumab combined with standard fractionation radiotherapy to enhance regional control and potentially boost the systemic immune response. The treatment was well tolerated with grade 2 skin toxicity within the range expected with radiation alone. Significant reduction in tumor bulk occurred after only 2 radiation fractions and complete response was achieved at 50 Gray.ConclusionThe complete clinical response observed in our patient suggests synergy between concurrent radiotherapy and PD1 blockade in CCS. This case and the striking parallels between CCS and melanoma indicate the need for prospective trials of immune checkpoint blockade combined with radiotherapy in this rare malignancy.