Primary Sarcoma Research Articles

ATRX and Its Prognostic Significance in Soft Tissue Sarcoma.

Recently, the association between ATRX and a more aggressive sarcoma phenotype has been shown. We performed a retrospective study of sarcomas from an individual institution to evaluate ATRX as a prognosticator in soft tissue sarcoma. Experimental Design. 128 sarcomas were collected from a single institution and stained for ATRX. The prognostic significance of these markers was evaluated in a smaller cohort of primary soft tissue sarcomas (n = 68). Kaplan-Meier curves were created for univariate analysis, and Cox regression was utilized for multivariate analysis. High expression of ATRX was found to be a positive prognostic indicator for overall survival and metastasis-free survival in our group of soft tissue sarcomas both in univariate analysis and multivariate analysis (HR: 0.38 (0.17-0.85), P=0.02 and HR: 0.49 (0.24-0.99), P=0.05, respectively). High expression of ATRX is a positive prognostic indicator of overall survival and metastasis-free survival in patients with STS. This is consistent with studies in osteosarcoma, which indicate possible mechanisms through which loss of ATRX leads to more aggressive phenotypes. Future prospective clinical studies are required to validate the prognostic significance of these findings.

Primary undiferentiated hepatic embryonal sarcoma: resect or transplant? Literature review and case report

Primary undiferentiated hepatic embryonal sarcoma: resect or transplant? Literature review and case report

Primary thoracic synovial sarcomas: clinical profile and treatment outcomes of a rare entity managed at a tertiary care centre.

Primary thoracic synovial sarcoma (PTSS) is a rare malignancy presenting with varying clinical manifestations. There is a paucity of data with few studies dedicated to this unique subset of neoplasms. We present our findings from one of the largest real-world studies among patients with PTSS. This is a single-centre, real-world study in patients with PTSS included between 2017 and 2023. Survival estimates were obtained by the Kaplan-Meier method and Cox regression analysis. 24 patients with a median age of 34.5 years (range 16-54) presented with chest pain (n = 11, 45.8%) and dyspnea (n = 10, 41.6%). Predominant primary sites of disease were the lung (n = 12, 50%) and mediastinum (n = 6, 25%). The stage at presentation was unresectable locally advanced (n = 10, 41.6%), localised (n = 8, 33.3%) and metastatic (n = 6, 25%) with pulmonary metastases (n = 10, 62.5%) and pleural effusion (n = 4, 25%). 16 (66.6%) patients underwent surgical resection including 7 (43.8%) who received neoadjuvant chemotherapy (NACT). NACT was given in ten patients producing stable disease in 5 (50%) and partial response in 3 (30%) patients, respectively, with surgery performed in 7 (70%). 11 (62.5%) operated patients had a microscopically complete resection and 10 (41.6%) received postoperative radiotherapy. Anthracyclines were given in 23 (95.8%) patients in the first line, while pazopanib was the most common therapy in the second and third lines, respectively. At a median follow-up of 32 months (range 16.7-47.2), the median overall survival (OS) was 41 months (95% CI: 23.7-58.2) and 8 months (95% CI: 1-25.6) overall and in metastatic disease, respectively. Presentation with metastases (p = 0.01) and treatment with surgical resection (p = 0.005) were significantly associated with OS on univariate analysis. The locally advanced nature of the disease at presentation signifies the need for early diagnosis and technically superior definitive therapies. The survival outcomes for metastatic disease remain poor and the need for novel therapies for advanced disease remains unmet so far. Not applicable.

Primary Pulmonary Leiomyosarcoma Managed With Pazopanib: A Case Report and Literature Review

Primary pulmonary leiomyosarcoma (PPL) arises from pulmonary smooth muscle tissue, with less than 0.5% incidence among pulmonary malignancies and 30% among primary pulmonary sarcomas. Here, we present a case of PPL managed with pazopanib. Moreover, a brief review of relevant literature was conducted. A 65-year-old female presented with chronic cough, weight loss, and recurrent pneumonia, with a medical history including diabetes mellitus, hypertension, and hyperlipidemia, as well as past surgical colectomy and hysterectomy (due to abnormal uterine bleeding with normal pathology and no evidence of uterine leiomyosarcoma). Upon admission, she exhibited fever, cough, dyspnea, and decreased breath sounds over the upper lung lobe. Diagnostic workups revealed a large pulmonary mass, diagnosed as leiomyosarcoma via core needle biopsy and subsequent immunohistochemical staining. Neoadjuvant treatment with ifosfamide and adriamycin followed by chemoradiotherapy was attempted, but surgery for tumor resection was not feasible. Then, gemcitabine and docetaxel were chosen as the new treatment after ifosfamide and adriamycin were not effective. Imaging revealed tumor not reacting to latest treatment, either. Given the disease's persistence and the patient's diminished capacity for chemotherapy, the patient is presently undergoing pazopanib treatment, with ongoing monitoring of its effects. After 3 months of treatment with pazopanib (administered orally at 200 mg twice daily), the patient experienced a significant reduction in tumor size, with a notable decrease from 135 mm to 80 mm, approximating one-third of the initial size, indicating a positive therapeutic effect. This case report provides preliminary evidence suggesting that pazopanib, an oral multi-tyrosine kinase inhibitor, may be a promising therapeutic option for the management of PPL. However, further in-depth and long-term studies are warranted to evaluate the clinical efficacy and superiority of this treatment.

Primary Extraskeletal Ewing Sarcoma, Initially Misdiagnosed as a Neurogenic Tumor

Primary Extraskeletal Ewing Sarcoma, Initially Misdiagnosed as a Neurogenic Tumor

Philosophies And Surgical Techniques for Ewing's Sarcoma of Spine with Review of Literature.

Ewing sarcoma (ES) is a malignant and aggressive bony tumor affecting the most common age group of 5-20 years. It constitutes 10%-15% of all bone sarcomas and is the second most common primary malignant bone tumor after osteosarcoma. It usually presents with pain, which is typically constant and progressive in nature. The primary source of pain is due to the instability of the spine to support the weight of the body, the vertebral body's expanding cortices due to the growing mass, compression of nerve roots due to tumour mass, pathologic fractures, spinal cord compression, and invasion of tissue by the tumour mass. We reviewed the literature on Ewing's Sarcoma of the spine to evaluate its etiology, clinical presentations, differential diagnosis, imaging modalities and management with chemotherapy, radiotherapy, and surgical management. PubMed, EMBASE, Google Scholar and Cochrane key articles were searched. Keywords like 'Ewing's Sarcoma,' 'Spine,' 'etiology,' 'treatment,' 'surgical management,' and 'en bloc resection' were used. The current management of Ewing's sarcoma of the spine usually involves three primary modalities: combination chemotherapy, surgery and/or radiotherapy. Recent improvements in combination chemotherapy (vincristine, doxorubicin, cyclophosphamide +/- Ifosfamide and etoposide) are among the most significant factors for improving survival. Also, recent advancements in radiotherapy, instrumentation, and fusion techniques in surgical management have been demonstrated to improve local disease control and overall survival. Primary Ewing sarcoma of the spine is a rare condition affecting the most common age group of 5-20 years, accounting for 1-3 cases/million/year. About 5 % of cases have spine involvement. Recent improvements in combination chemotherapy have improved the overall survival rates. Enbloc resection and/or radiotherapy have improved local control of the disease.

Kiosk 3Q-TA-02 - A Case of Cardiac Tamponade Caused by a Rare Primary Cardiac Ewing Sarcoma: The Crucial Role of Cardiovascular Magnetic Resonance in the Context of a Multimodality Imaging Approach

Kiosk 3Q-TA-02 - A Case of Cardiac Tamponade Caused by a Rare Primary Cardiac Ewing Sarcoma: The Crucial Role of Cardiovascular Magnetic Resonance in the Context of a Multimodality Imaging Approach

A Bibliometric Analysis of the 500 Most Cited Papers in Orthopaedic Oncology.

Despite notable progress over time, broad insight into the scientific landscape of orthopaedic oncology is lacking. We conducted a bibliometric analysis of the 500 most cited papers in the field. We searched the Science Citation Index Expanded database of the Web of Science Core Collection to find the 500 most cited articles in the field. Citation count ranged from 81 to 1,808. Articles were published from 1965 to 2018. Over half of all articles were published in the United States (53.6%). The 2000s was the most productive decade with 170 (34%) articles. All articles were written in English and were published across 29 journals. Female participation as first authors significantly increased from the 1960s to the 2010s (0% vs 14.6%, P = 0.0434). Similarly, female involvement as senior authors grew from the 1960s to the 2010s (0% vs 12.2%, P = 0.0607). Primary bone sarcomas were the most cited topic among articles from the 1970s to the 1980s. From studies produced in the 1990s up until the 2010s, reconstruction procedures were the most cited topic. Trends over the years have resulted in an emphasis on a surgical technique. Notable progress has been made regarding gender diversity, yet disparities still exist.

Primary hepatic undiferentiated pleomorphic sarcoma with recurrence and widespread metastasis.

A 59-year-old man was referred to us for evaluation of asymptomatic mass in the liver that had been detected on ultrasonography performed during a physical screening. He had no history of hepatitis, and was otherwise well. Tumor markers were normal, including alpha fetoprotein, carcinoembryonic antigen, neuron-specific enolase and cancer antigen 199. Abdominal CT showed a 5.0 cm diameter low density mass in the S6 segment of his liver, with mild peripheral enhancement. Abdominal MR demonstrated the mass with hypointensity on T1WI, inhomogeneous hyperintensity on fat-suppressed T2WI, and mild peripheral enhancement. Partial hepatectomy was performed and pathological examination indicated undiferentiated pleomorphic sarcoma, with immunohistochemical results as follows: Vimentin (+), CK (-), CD68 (+), INI-1 (+), Hepa (-), Gly-3 (-), Arg-1 (-), HMB45 (-), CAM5,2 (-), CD31 (-), CD34 (-), CK7 (-), CK19 (-),Desmin (-), SMA (+), EMA (-), S-100 (-), Ki67 (50%+). The patient underwent postoperative chemotherapy with karelizumab and gemcitabine. However, repeat MR 3 months later showed multiple nodules with rim-like enhancement around the surgical margin. Subsequent repeat CT 6 months later reveled marked progression of the lesions.

Outcome and response prediction for primary and oligometastatic spinal sarcoma patients who received stereotactic body radiation therapy (SBRT)

Outcome and response prediction for primary and oligometastatic spinal sarcoma patients who received stereotactic body radiation therapy (SBRT)

Pathology of Spinal Tumours

Abstract Spinal tumours are uncommon lesions, but they may cause significant morbidity and mortality. Majority of the spinal tumours are metastatic, that is, spread from tumours in other organs, which are mostly seen in older patients. Primary sarcomas involving the spinal column are rare. Benign tumours and tumour-like lesions of the musculoskeletal system are mostly seen in young patients. Spinal tumours can be subdivided into primary tumours, which are rare, and secondary tumours or metastases, which are much common. Primary vertebral tumours may be benign or malignant. Common benign spinal tumours include osteoid osteoma, osteoblastoma, osteochondroma, aneurysmal bone cyst and giant cell tumour. There are many malignant primary spinal tumours such as- osteosarcoma, chondrosarcoma, and chordoma, Ewing’s sarcoma, plasmacytoma and multiple myeloma. However, all of them, except multiple myeloma, are rare. Intraspinal tumours may be extradural or intradural. Common intradural extramedullary tumours include meningioma, schwannoma and intradural neurofibroma, whereas astrocytoma, ependymoma and haemangioblastoma are a few examples of intradural intramedullary tumours.

Extraosseous Ewing sarcoma of the pancreas: a case report.

Extraosseous Ewing sarcoma is a rare and aggressive malignancy belonging to the Ewing sarcoma family of tumors, primarily affecting soft tissues such as the pelvis, retroperitoneum, and chest wall. Although it predominantly involves these soft tissues, extraosseous Ewing sarcoma can also occur in solid organs, including the pancreas. Here, we present a rare case of a 4-year-old girl diagnosed with primary extraosseous Ewing sarcoma of the pancreas.

Patency and disease-free status after oncovascular resection of truncal malignancies with major vascular invasion

BackgroundWith advances in current therapies, more patients are currently surgical candidates for oncologic resections that were previously deemed unresectable owing to vascular invasion. We analyzed our institution's outcomes of patients undergoing oncovascular resection of truncal malignancies with vascular invasion to identify factors associated with vascular reconstruction complications and oncologic recurrence. MethodsA retrospective chart review of all adult patients between January 1, 2002, and June 1, 2022, who underwent a surgical resection of a neoplasm at Northwestern Memorial Hospital and required vascular surgery intraoperative assistance was performed. Data on patient demographics, tumor anatomy and pathology, operative details, and clinical outcomes were extracted. Primary end points included 30-day and 1-year mortality, and 1-year primary patency of the vascular reconstruction. Secondary end points included vascular reintervention and tumor recurrence, both local and metastatic disease. ResultsA total of 104 patients (43.3% females) with a mean age of 58 years were included in the study. The median follow-up was 22.1 months (range, 0-207 months). The most common tumor pathology included advanced renal cell carcinoma (n = 58) and soft tissue and primary vascular sarcomas (n = 24). Vascular procedures included 98 venous interventions and 6 arterial interventions. The overall 30-day mortality was 1% and 1-year mortality was 14.4%. Primary vascular patency at 1 year among patients with ≥12 months of follow-up was 98.5%. No patients underwent a vascular reintervention. In total, 34 patients (38%) developed recurrent malignant disease (23.5% local and 76.5% metastatic) during the study period. The median time to tumor recurrence was 11.4 months (range, 1.2-100.0 months). ConclusionsOncovascular resections of truncal tumors involving major vascular structures can be performed safely with a low incidence of vascular complications; tumor recurrence remains the major contributor to morbidity. Further investigation of the factors associated with tumor recurrence in these patients may help to refine surgical techniques.

Primary Pulmonary Synovial Sarcoma; Abnormal Presentation of a Rare Lung Tumor

Primary synovial sarcomas of the lung are a very rare entity in thoracic surgery. Unusual presentation make it hard to diagnose such disease and manage it probably even harder. We present two cases with same tumor but different appearance.

Primary synovial sarcomas of the bone: clinical perspectives and correlation between the application of SS18-SSX and decalcification status.

Primary synovial sarcomas of the bone: clinical perspectives and correlation between the application of SS18-SSX and decalcification status.

Establishment of a staging system for visceral sarcoma.

Visceral sarcoma is a rare malignancy with a poor prognosis. However, there is no recommended prognostic staging system for the malignant disease. We analyzed the data of patients diagnosed with primary soft tissue sarcoma (STS) of the abdomen and thoracic visceral organs between 2006 and 2017 at our hospital. Prognostic factors (size, tumor grade, and lymph node metastasis) were analyzed in our cohort (n = 203) and the SEER validation cohort (n = 5826). Tumor size, grade, and lymph node metastasis were important prognostic factors for visceral sarcoma in both our and the SEER cohorts. Based on these prognostic factors, we established a new staging system for visceral sarcoma, by which patients could be stratified into clinically meaningful and non-overlapping stages in both our cohort and the SEER validation series. Moreover, the area under the curve (AUC) value of the staging system for 5-year survival was 0.84 (95% CI: 0.78-0.89) in our series and 0.80 (95% CI: 0.79-0.81) in SEER series, respectively. In addition, compared with the widely used FIGO staging system for female genital sarcoma, the visceral sarcoma staging system could more effectively and reliably stratify patients into four different prognostic groups. The visceral sarcoma staging system is applicable for STS of the abdomen and thoracic visceral organs and is better than the current FIGO staging system for female genital sarcoma and should be incorporated into the AJCC Cancer Staging Manual.

The Multidisciplinary Approach to Vaginal Leiomyosarcoma, an Extremely Rare Diagnosis - Case Report

Primary vaginal sarcoma is an extremely rare malignant condition within the field of gynecological pathologies. We present the case of a 53-year old patient with no history of gynecological issues or associated comorbidities who presented to our clinic with recurrent vaginal bleeding during menopause. We want to emphasize the importance of diagnostic management, going through all the necessary steps from local gynecological examination to vaginal biopsy, optimizing results with advanced imaging techniques. Once the histopathological diagnosis of vaginal leiomyosarcoma was established, the therapeutic approach was discussed within a multidisciplinary committee consisting of a gynecologist, surgeon, and oncologist. As a result, the patient underwent tailored surgical intervention relatively quickly after presentation, followed by referral to the oncology department, where she is currently undergoing her third course of adjuvant chemotherapy. Our current objective is the long-term follow-up of the patient and the acquisition of data regarding her survival and quality of life. These facts may contribute in the future to the implementation of standardized therapeutic guidelines for such a rare condition

Primary Uterine Alveolar Soft Part Sarcoma in a Postmenopausal Woman: Histopathologic and Immunohistochemical Characteristics of a Rare Case.

Primary uterine alveolar soft part sarcoma (ASPS) is a rare, indolent mesenchymal malignancy with less than 40 patients documented in the literature. We report an example of ASPS in a 61-year-old postmenopausal woman. Macroscopically, the uterus showed multiple nodular masses. Microscopic examination revealed tumor arranged in nests and alveolar pattern. The tumor cells were moderately to markedly pleomorphic, epithelioid to polygonal, with eccentrically placed nuclei, vesicular chromatin, prominent macro-nucleoli, and moderate to abundant eosinophilic cytoplasm. PAS-positive and diastase-resistant intracytoplasmic crystals were also seen in some tumor cells. On immunohistochemistry, the tumor cells showed diffuse positivity for vimentin and nuclear positivity for TFE3, a surrogate marker for ASPS. These were negative for SMA, desmin, CD10, h-caldesmon, cyclin D1, EMA, Melan A, and CD34. SMARCB1 expression was retained. Based on the histopathology and IHC, a final diagnosis of uterine ASPS was rendered. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare tumors. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare sarcoma in an uncommon site with an unusual age.

Long-term results of oncological endoprosthetics of the ankle joint: multicenter study

Introduction. The distal tibia and fibula are rare sites of involvement by primary and metastatic tumors. For a long time, amputation remained the standard surgical intervention for this location. Oncological ankle replacement is associated with a number of difficulties, including a lack of soft tissue necessary to cover the implant, close proximity of anatomical structures, a high probability of vascular and infectious complications, and difficulties in restoring the biomechanics of the lost joint. Currently, due to the small number of analyzed groups and short-term observations, there is no generalized data on primary and revision oncological ankle replacement, and there is no developed approach to treatment and prevention of complications.Aim. To generalize the analysis of long-term results, the structure of complications, and functional results in a group of patients after primary and revision oncological ankle replacement in a statistically significant group of patients.Materials and methods. The study included 40 patients with benign bone tumors, primary localized sarcomas of bone and soft tissue, and metastatic lesions, which, since 2008, until 2023 56 primary and revision endoprosthetics of postresection defect of the distal tibia were performed. Over a period of 15 years, the study group included 40 (71.4 %) surgeries in the scope of primary endoprosthetics and 16 (28.6 %) surgeries in the scope of revision oncological endoprosthetics of the ankle joint.Results. Over a 15-year follow-up period, the overall incidence of complications, structured according to the International Society of limb Salvage (ISOLS) 2013 classification, after primary and revision arthroplasty was 50 %. The average time until detection of oncological and non-oncological complications was 16.1 months. The leading complication after endoprosthetics was aseptic instability (Type II; 21.4 %) and tumor recurrence (Type V) – 20 %. The average functional outcome after primary and revision ankle replacement was assessed using the MSTS scale and was 72 % after 6 months and ranged from 43 to 97 %. After 12 months, this figure was 78 % and ranged from 49 to 97 %.Conclusion. An objective assessment and increase in the statistical reliability of the results of oncological ankle replacement requires a larger number of surgeries, a longer observation period and joint consolidation of data from various clinics. Development of indications for such reconstructive operations, careful selection of patients taking into account the effect of conservative treatment allows to reduce the total number of complications, the number of local relapses and achieve a good functional result.

Intracranial Ewing sarcoma with obstructive hydrocephalus in pediatric patient: a rare case report

Link of Video Abstract: https://youtu.be/b4jB7Jos8tw Background: Ewing’s sarcoma is the second most common primary bone tumor presenting in childhood and adolescence. Ewing's sarcoma of the skull bones is an unusual occurrence (6 to 9% of all Ewing’s. sarcoma cases). Here, we present a rare case of Ewing's sarcoma involving the central nervous system (CNS) in a 13-month-old child and share our experience with its management. Case Presentation: We report a case of a 13-month-old girl with a chief complaint of gradually progressive head size. There is a history of seizures once a week before admission. The head CT scan with contrast administration revealed non-communicating hydrocephalus with bone defect in the occipital region and an intraaxial tumor of the posterior fossa due to Ewing’s sarcoma. Conclusion: Primary cranial Ewing's sarcoma of the cranium is a rare occurrence; its extension to intraparenchymal is even rare. This diagnosis can be considered in the differential diagnosis in children with a tumor involving the skull with destruction of the bone.