Primary Mediastinal Sarcomas Research Articles

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.

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.

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.

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.

Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature.

Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH) demonstrated the t(X:18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

EComment. Primary mediastinal sarcomas

We have read with great interest the article by Luo et al. concerning the surgical outcomes for primary mediastinal sarcomas [1]. Sarcomas of the mediastinum and great vessels remain a rare subgroup of soft tissue sarcomas and prognosis remains poor with patients often presenting with advanced disease. Surgical resection represents the mainstay of therapy. The aim of this brief comment is to highlight the role of chemotherapy in a preoperative and postoperative setting. The key is to identify chemosensitive subtypes of mediastinal sarcomas (i.e. synovial sarcomas) and there is evidence of satisfactory results in the literature [2,3]. Conflict of interest: none declared.

Primary mediastinal tumors in children

Mediastinal masses in children constitute a heterogeneous group of malignant and benign neoplasms. The majority of malignant tumors in our series of 188 children with a mediastinal mass were Hodgkin's and non-Hodgkin's lymphoma (87 patients) arising in the anterior and middle mediastinum. Ganglioneuroma (17) arising in the posterior mediastinum made up the bulk of benign tumors (52). Infants less than 2 yr old presented with symptoms of tracheal compression, whereas older children had fewer symptoms but a far greater likelihood of having a malignancy. In the assessment of patients with mediastinal tumors, the posteroanterior and lateral chest roentgenograms were most often diagnostic, and computed tomography of the chest provided the most information concerning preoperative resectability. The excellent survival of patients with stage I and II Hodgkin's disease reflects the radiosensitivity of the tumor; in our series of 33 patients, survival was not increased by radical resection. Unlike lymphomatous tumors, neurogenic masses should be completely excised. When initial exploration reveals unresectability, biopsy followed by radiation therapy and second-look excision of the tumor can be accomplished. In patients with primary mediastinal sarcomas, total resection should be carried out if possible. In our series, adjuvant radiation therapy or chemotherapy was effective in only 1 of 15 children with sarcoma. Surgical treatment of all mediastinal masses except lymphoma should be complete excision. In cases of suspected lymphoma, cervical or supraclavicular node biopsy can yield adequate tissue for diagnosis.