Primary Sarcoma Research Articles

Factors Influencing the Outcome of Patients with Primary Ewing Sarcoma of the Sacrum.

Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the sacrum as a primary site is very rare, and only few studies focusing on this location are published. Due to the anatomical condition, local treatment is challenging in sacral malignancies. We analyzed factors that might influence the outcome of patients suffering from sacral EwS. We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model. The presence of metastases (3y-EFS: 0.33 vs. 0.68; P < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; P < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; P=0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; P=0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; P=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; P=0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors. Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. This trial is registered with NCT00020566 and NCT00987636.

A case of poorly differentiated type primary pulmonary synovial sarcoma

A case of poorly differentiated type primary pulmonary synovial sarcoma

Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature

BackgroundSpinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management.Case presentationWe report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient’s sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3–L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted.ConclusionsIntradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.

Use of Vacuum-Assisted Closure to Reduce the Likelihood of Wound Complications After Limb-Sparing Resection of Pediatric Primary Bone Sarcomas of the Femur

BackgroundLimb-sparing surgery is the standard of care for primary bone tumors. However, such procedures are associated with high rates of wound complications, specifically in lower-extremity surgeries. Therefore, identifying and implementing interventions to minimize the likelihood of wound complications after limb-sparing resection of the lower extremity is crucial. MethodsPatients who underwent limb-sparing osteosarcoma or Ewing sarcoma resection during a 7-year period at a single institution were retrospectively reviewed. Data were collected on 39 patients who underwent limb-sparing resection of the femur. Patient demographics, tumor characteristics, and perioperative and postoperative data were extracted and analyzed. Patients who underwent resection before April 2017 received conventional postoperative incision dressings. Starting in April 2017, patients received vacuum-assisted closure (VAC) with the 3 M™ Prevena VAC system after surgical closure. Eighteen patients received conventional postoperative incision dressing, and 21 received incisional wound VAC. A wound complication was defined as any Clavien-Dindo classification greater than 0 within a 28-day postoperative period. ResultsPatients who received postoperative incisional wound VAC had lower rates of wound complications than those who received conventional incision dressings (14% vs. 50%; p = 0.035). Additionally, patients in whom wound complications developed had a longer average hospital stay than those without wound complications (5 days vs. 4 days; p = 0.029). ConclusionsWound complications prolong the hospital stay and can delay adjuvant chemotherapy for bone tumors. The use of postoperative incisional wound VAC is associated with less likelihood of wound complications and should be considered in any high-risk surgical closure. Level of EvidenceLevel III Treatment Study.

Exploring the Diagnostic Dilemma of Indeterminate Pulmonary Nodules in Patients with Primary Sarcoma of Bone.

Bone sarcomas are known to have a predilection for pulmonary metastasis. Surveillance protocols are thus focused on periodic chest imaging, typically with CT scan. Pulmonary nodules can be easily identified with this modality, but smaller nodules are not readily biopsied and may not represent metastatic disease. These are called indeterminate. The natural history of indeterminate nodules in a bone sarcoma population and factors associated with progression to true metastatic disease are not clearly defined. All bone sarcoma patients treated at a single institution from 2010 to 2020 were eligible for inclusion. We treated 327 patients over this period; 119 were excluded for age less than 16 years, 31 were excluded for evident metastatic disease at presentation, and 60 were excluded for incomplete clinical follow-up or CT chest imaging either at staging or in surveillance. We assessed chest CT images for presence of pulmonary nodules and selected variables both at the staging and on surveillance images. Nodules were considered metastatic if proven histologically with a biopsy or by clinical interpretation by the multidisciplinary sarcoma team. Clinical and imaging factors were assessed for the association of indeterminate nodule progression to true metastatic disease. Seventy three of the 117 patients had indeterminate nodules on their staging CT scan; 41.1% of those patients progressed to metastatic disease compared to 43.2% of the patients that did not have indeterminate nodules on staging CT. Fifty eight of the 117 patients developed indeterminate nodules on surveillance chest CT, and 55.2% of those patients progressed to metastatic disease. There were no clinical or imaging factors that predicted the development of metastatic disease in the group that had indeterminate nodules at presentation; however, the number and size of nodules did correlate with progression to metastasis in those that developed indeterminate nodules on surveillance. Indeterminate pulmonary nodules are common on staging CT scans in patients with a bone sarcoma. The presence or absence of these indeterminate nodules was not predictive of progression to true metastatic disease in this cohort. However, the development of indeterminate nodules on surveillance imaging was associated with progression to metastatic disease with the size and number of nodules being important factors.

Influence of nutritional status and body composition on postoperative events and outcome in patients treated for primary localized retroperitoneal sarcoma.

Retroperitoneal sarcomas (RPS) are rare tumours of mesenchymal origin, commonly presented as a large tumour mass at time of diagnosis. We investigated the impact of body composition on outcome in patients operated on for primary localized RPS. We retrospectively analysed data for all patients operated on for primary RPS at our institution between 1999 and 2020. Preoperative skeletal muscle area (SMA), visceral and subcutaneous adipose tissue area (VAT and SAT) and muscle radiation attenuation (MRA) were calculated using computed tomography scans at the level of third lumbar vertebra. European Working Group on Sarcopenia in Older People (EWGSOP2) criteria were applied to define myopenia. Using maximum log-rank statistic method we determined the optimal cut-off values of body composition parameters. Myosteatosis was defined based on determined MRA cut-offs. In total 58 patient were eligible for the study. With a median follow-up of 116 months, the estimated 5-year overall survival (OS) and local-recurrence free survival (LRFS) were 66.8% and 77.6%, respectively. Patients with myopenia had significantly lower 5-year OS compared to non-myopenic (p = 0.009). Skeletal muscle index and subcutaneous adipose tissue index predicted LRFS on univariate analysis (p = 0.052 and p = 0.039, respectively). In multivariate analysis high visceral-to-subcutaneous adipose tissue area ratio (VSR) independently predicted higher postoperative complication rate (89.2% vs. 10.8%, p = 0.008). Myosteatosis was associated with higher postoperative morbidity. Myopenia affected survival, but not postoperative outcome in RPS. Visceral obesity, VSR (> 0.26) and myosteatosis were associated with higher postoperative morbidity. VSR was better prognostic factor than VAT in RPS.

Assessment of medical students' knowledge of primary limb sarcomas

IntroductionTypically, oncology is not a structured part of the curriculum in Brazilian medical schools. Furthermore, sarcomas, which are uncommon tumors, are seldom covered in depth. A lack of comprehensive education on sarcomas might result in medical professionals being ill-equipped to care for patients with this condition.ObjectivesTo assess medical students' understanding and awareness of sarcomas and the specific principles related to these tumors.Materials and methodsA quantitative, cross-sectional study was conducted using a questionnaire, applied to medical students, focusing on the epidemiology, pathophysiology, and treatments of bone and soft tissue sarcomas. In all tests, the significance level adopted was 5%. The SPSS version 25.0 software was used.ResultsOf the 825 questionnaires distributed, 325 were returned. Educational sessions on sarcomas did not appear to significantly improve the student's knowledge. Only 29.5% of students identified the lack of pain as an indicator of potential malignancy in soft tissue sarcomas, while 73.8% correctly recognized pain as a symptom of bone sarcomas. Limb amputation as the optimal surgical method for patient recovery was incorrectly reported by 39.1% of the sample.ConclusionA great part of the surveyed population does not have adequate knowledge about the basic concepts associated with limb sarcomas. The minority of them are satisfied with the knowledge gained during their medical education about these tumors. Inadequate medical academic training may initially lead to the wrong clinical management of patients with bone and soft tissue tumor lesions. An educational effort is needed to enhance oncology education for medical students, especially concerning sarcomas.

Central Nervous System Metastases in Pediatric Patients With Ewing Sarcoma.

Metastatic central nervous system (CNS) involvement is rare in pediatric primary extracranial Ewing sarcoma (ES). We describe the incidence and course of 6 patients with extracranial ES who developed metastatic CNS lesions treated at a single institution. The median time to CNS disease detection was 16.3 months (10.0-28.3 months). Event-free and overall survival after CNS disease detection were 1.9 months (0.4 to 10.3 months) and 4.6 months (1.1 to 50.9 months), respectively. One patient was alive at the time of analysis. Clinical status and ability to obtain disease control should be considered when making decisions regarding aggressive interventions in these patients with poor prognosis.

Clinical Significance of Genetic and Molecular Changes in Primary Myeloid Sarcoma

To investigate the clinical significance of genetic and molecular changes in primary myeloid sarcoma (MS). Fourteen patients with primary MS were selected in Jiading District Central Hospital Affiliated Shanghai University of Medicine & Health Sciences, The First People's Hospital of Lianyungang from September 2010 to December 2021. AML1-ETO fusion, PML-RARα fusion and CBFβ breakage were detected by fluorescence in situ hybridization (FISH), and the mutations of NPM1, CEBPA, FLT3, RUNX1, ASXL1, KIT and TP53 genes were detected by new generation sequencing (NGS). Among 14 patients, the MS occurred in bone, breast, epididymis, lung, chest wall, cervix, small intestine, ovary, lymph nodes and central nervous system. The tumor cells expressed MPO (13 cases), CD34 (7 cases), CD43 (8 cases), CD68 (7 cases), CD99 (8 cases) and CD117 (6 cases). Cytogenetic abnormalities were observed in 4 cases, including 3 cases of AML1-ETO fusion and 1 case of CBFβ breakage, while no PML-RARα fusion was detected. There were no significant differences in overall survival (OS) and leukemia-free survival (LFS) between patients with and without AML1-ETO fusion/CBFβ breakage (both P >0.05). Among the 14 patients, the number of NPM1, CEBPA, FLT3-ITD, RUNX1, ASXL1, KIT and TP53 gene mutations was 5, 3, 5, 3, 2, 2, 1, respectively, of which 7 cases had at least one mutation in FLT3-ITD, RUNX1, ASXL1 and TP53 gene. The OS and LFS of patients with FLT3-ITD, RUNX1, ASXL1 or TP53 mutation were shorter than those without mutations (both P <0.01). The genetic and molecular abnormalities of primary MS can be detected by FISH and NGS techniques. FLT3-ITD, RUNX1, ASXL1 or TP53 mutation indicates a worse prognosis, but further clinical studies are needed to confirm it.

Primary sarcoma of prostate: A genomic landscape study.

181 Background: Primary sarcoma of prostate is exceedingly rare and not well-studied. We sought to describe the genomic landscape of this rare entity and identify potential therapy targets. Methods: From 19,057 cases of prostate cancers, only 11 (&lt;.01%) cases of primary sarcomas were identified and underwent comprehensive genomic profiling (CGP) using FDA-approved hybrid capture-based system to assess all classes of genomic alterations (GA). Genomic-based ancestry, genomic signature, gLOH, MSI and TMB status were determined by CGP. Germline status was predicted using a Somatic-Germline-Zygosity algorithm. PD-L1 expression was determined by IHC (Dako 22C3 TPC scoring). Results: The routine histology and IHC stains from all 11 cases were reviewed centrally. There were 9 stromal sarcomas, and 1 each leiomyosarcoma and rhabdomyosarcoma. All patients (median age 57) were clinically advanced stage at the time of CGP. The primary tumor site was used in 6 patients and metastatic sites (3 bone, 1 ureter and 1 lung metastasis) in 5 patients. The mean number of GA per case was 2.3 (1 to 4 GA/sample). The most frequent GA were in TP53 (36.4%), RB1 (27.3%), ATRX (18.2%). Potentially targetable GA were rare and included MTOR pathway inhibitors for GA in TSC2 and PTEN (1 case each) and PIK3CA inhibitors (GA in PIK3CA in 1 case). There were 3 cases with non “targetable” gene rearrangements including a STAT6- NAB2 fusion in a stromal sarcoma (possible solitary fibrous tumor of prostate), a BCOR- MAML3 fusion and a TMPRSS2- ERG fusion in a stromal sarcoma with an adjacent focus of Gleason 6 prostate adenocarcinoma. 1 case featured a predicted germline mutation of the FLCN gene. All cases were microsatellite stable. TMB ranged from 0 to 9.8 mutations/Mb with median of 3.4 mutations/Mb with no cases at ≥10 mutations/Mb. gLOH scores were low, ranging from 0% to 10.9% (median 2.6%). Genomic ancestry was European in 7 patients and Admixed American in 4 others. There were no specific genomic signatures identified. PD-L1 was tested in 4 cases (all negative). Conclusions: Prostate sarcoma is an exceedingly rare primary cancer of the prostate with limited opportunities for targeted therapy or immunotherapy strategies. These tumors do not appear to be driven by “targetable” gene fusions and individual “targetable” mutations are uncommon. [Table: see text]

Transaortic removal of a large primary sarcoma from the left ventricle assisted by strategic partial resection and endoscopic guidance: a case report

BackgroundSurgical resection remains the mainstay of treatment for cardiac sarcoma, a rare but lethal disease. Achieving complete removal of a large-sized left ventricular sarcoma remains a challenge even with various surgical approaches that have been employed.Case presentationWe present a case of a 74-year-old woman with shortness of breath who underwent surgical removal of a primary cardiac sarcoma, measuring 6 × 3.5 × 3 cm, attached to the septum of the left ventricle and caused sub-aortic valve obstruction. Transaortic approach was chosen and the access to this entire huge mass was enabled by using interim partial resection which created a space for further dissection and subsequent deeper endoscopic views. The further dissection was finally able to be advanced on the apex, and the residual mass was completely resected with gross tumor-free margins.ConclusionInterim partial resection and endoscopic guidance can highly facilitate the transaortic removal of even large left ventricular sarcomas.

Primary follicular dendritic cell sarcoma of the kidney – a case report of a rare tumor with emphasis on diagnostic pitfalls

BackgroundFollicular dendritic cell sarcoma (FDCS) is a rare low-grade tumor of the lymph nodes, but roughly one-third of the cases emerge from extranodal sites, posing diagnostic challenges.Case presentationIn this report, we present the case of a 59-year-old lady who complained of renal colic. During investigation, a kidney tumor was discovered. A radical nephrectomy was performed, and histological examination identified the tumor as a sarcomatoid renal cell carcinoma. The case was then referred to a genitourinary pathologist for further evaluation. The tumor cells exhibited positive staining for CD21, CD23, somatostatin receptor 2 A, and MDM2 expression. Additionally, MDM2 gene amplification was confirmed by the FISH study. Ultimately, the tumor was diagnosed as a primary renal FDCS. The patient was placed under active oncological surveillance and did not receive any further therapy. Remarkably, after 91 months of follow-up, she remains tumor-free.ConclusionThis case represents a well-documented primary renal FDCS. Our aim in presenting this extremely rare tumor is to enhance awareness and highlight the importance of considering FDCS in the differential diagnosis.

Internal hemipelvectomy: A single institution's learning curve and longitudinal experience.

Wide margin resection for pelvic tumors via internal hemipelvectomy is among the most technically challenging procedures in orthopedic oncology. As such, surgeon experience and technique invariably affect patient outcomes. The aim of this clinical study was to assess how an individual surgeon's experiences and advancements in technology and techniques in the treatment of internal hemipelvectomy have impacted patient outcomes at our institution. This study retrospectively examined a single tertiary academic institution's consecutive longitudinal experience with internal hemipelvectomy for primary sarcoma or pelvic metastases over a 26-year period between the years 1994 and 2020. Outcomes were assessed using two separate techniques. The first stratified patients into cohorts based on the date of surgery with three distinct "eras" ("early," "middle," and "modern"), which reflect the implementation of new techniques, including three-dimensional (3D) computer navigation and cutting guide technology into our clinical practice. The second method of cohort selection grouped patients based on each surgeon's case experience with internal hemipelvectomy ("inexperienced," "developing," and "experienced"). Primary endpoints included margin status, complication profiles, and long-term oncologic outcomes. Whole group multivariate analysis was used to evaluate variables predicting blood loss, operative time, tumor-free survival, and mortality. A total of 72 patients who underwent internal hemipelvectomy were identified. Of these patients, 24 had surgery between 1994 and 2007 (early), 28 between 2007 and 2015 (middle), and 20 between 2016 and 2020 (modern). Twenty-eight patients had surgery while the surgeon was still inexperienced, 24 while developing, and 20 when experienced. Evaluation by era demonstrated that a greater proportion of patients were indicated for surgery for oligometastatic disease in the modern era (0% vs. 14.3% vs. 35%, p = 0.022). Fewer modern cases utilized freehand resection (100% vs. 75% vs. 55%, p = 0.012), while instead opting for more frequent utilization of computer navigation (0% vs. 25% vs. 20%, p = 0.012), and customized 3D-printed cutting guides (0% vs. 0% vs. 25%, p = 0.002). Similarly, there was a decline in the rate of massive blood loss observed (72.2% vs. 30.8% vs. 35%, p = 0.016), and interdisciplinary collaboration with a general surgeon for pelvic dissection became more common (4.2% vs. 32.1% vs. 85%, p < 0.001). Local recurrence was less prevalent in patients treated in middle and modern eras (50% vs. 15.4% vs. 25%, p = 0.045). When stratifying by case experience, surgeries performed by experienced surgeons were less frequently complicated by massive blood loss (66.7% vs. 40% vs. 20%, p = 0.007) and more often involved a general surgeon for pelvic dissection (17.9% vs. 37.5% vs. 65%, p = 0.004). Whole group multivariate analysis demonstrated that the use of patient-specific instrumentation (PSI) predicted lower intraoperative blood loss (p = 0.040). However, surgeon experience had no significant effect on operative time (p = 0.125), tumor-free survival (p = 0.501), or overall patient survival (p = 0.735). While our institution continues to utilize neoadjuvant and adjuvant therapies following current guideline-based care, we have noticed changing trends from early to modern periods. With the advent of new technologies, we have seen a decline in freehand resections for hemipelvectomy procedures, and a transition to utilizing more 3D navigation and customized 3D cutting guides. Furthermore, we have employed the use of an interdisciplinary team approach more regularly for these complicated cases. Although our results do not demonstrate a significant change in perioperative outcomes over the years, our institution's willingness to treat more complex cases likely obscures the benefits of surgeon experience and recent technological advances for patient outcomes.

Clinicopathological Features and Prognosis of Primary Breast Sarcoma: A Single Centre Retrospective Study

Objective: This study aims to describe clinicopathological characteristics and outcomes of primary breast sarcoma (PBS) and the relevance of clinical and pathological characteristics with the outcomes.&#x0D; Methodology: &#x0D; Study Design: Retrospective longitudinal study&#x0D; Place and duration of study: Department of Medical Oncology Shaukat Khanum Memorial Cancer Hospital and Research Centre Lahore from January 1995 to August 2021.&#x0D; Patients aged 18 years and older were included in the study. Individuals with primary or secondary angiosarcoma or cystosarcoma phyllodes were not included. The hospital information system was used for collecting data, and data was collected for 27 patients meeting inclusion study criteria.&#x0D; Results: Twenty-seven patients were treated with surgery, seven out of 27 patients (25.90%) received adjuvant chemotherapy and 2 patients (7.40%) received chemotherapy on a palliative intent. The remaining (66.70%, n=18) did not receive chemotherapy. Twenty-four out of 27 patients (88.90%) received radiation therapy; intent was adjuvant in the entire cohort. Median PFS was 18 months with 74.01% of patients having no progression at their last follow-up. The median OS was 23 months. Adjuvant radiotherapy was associated with a significant overall survival benefit (P-value: 0.05). Adjuvant chemotherapy was not associated with a significant survival benefit (P-value: 0.5). Age, tumor size, and disease status (localized disease) conferred a non-significant trend towards a better outcome.&#x0D; Conclusion: Adjuvant radiotherapy is the only factor that affects the outcomes of this disease. Further multicentred and preferably prospective studies with a larger number of patients are required to define disease characteristics and patient outcomes of primary breast sarcoma in Pakistan.&#x0D; Keywords: clinical characteristics, outcomes, prognosis, primary breast sarcoma (PBS)

Identification of CT Features to Differentiate Pulmonary Sarcoma from Carcinoma.

Background Primary lung sarcoma (PLS) differs in management protocols and prognosis from the more common primary lung carcinoma (PLC). It becomes imperative to raise a high index of suspicion on radiological and pathological features. Purpose The aim of this study is to highlight the variable imaging appearances of PLS compared with PLC, which impacts radiologic - pathologic correlation. Materials and Methods A retrospective observational study of 68 patients with biopsy-proven lung tumors who underwent baseline imaging at our tertiary care cancer hospital was conducted between January 2018 and March 2022. The patient details and imaging parameters of the mass on contrast-enhanced computed tomography (CECT) were recorded and analyzed for patients with PLS and compared with PLC. Follow-up imaging was available in 9/12 PLS and 52/56 PLC patients. Results Among 12 patients with PLS, 5 patients had synovial sarcoma on histopathology. PLS was seen in patients with a mean age of 40.8 years; the mass showed a mean size of 13.2 cm, lower lobe (75%), parahilar (75%), hilar involvement (41.7%), oval shape (41.7%), circumscribed (25%) or lobulated (75%) margins, lower mean postcontrast attenuation of 57.3 HU, fissural extension (50%), calcification (50%), and no organ metastasis other than to the lung. PLC (56 patients) was seen in the elderly with a mean age of 54.8 years; the mass showed a mean size of 5.7 cm, irregular shape (83.9%), spiculated margins (73.2%), higher mean postcontrast attenuation (77.3 HU), chest wall infiltration (30.4%), and distant metastasis (58.9%) at baseline imaging. A statistically significant difference ( p < 0.05) was seen between sarcoma and carcinoma in the mean age, size, site, shape, margins, postcontrast attenuation, presence of calcifications, fissural extension, and distant metastasis. Conclusion The distinct imaging features of sarcoma help in differentiating it from carcinoma. This can also be used to corroborate with histopathology to achieve concordance and guide clinicians on further approach.

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study

BackgroundPrimary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. ObjectivesThe aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). MethodsA retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. ResultsAmong 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. ConclusionsAlthough ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

Radiotherapy versus limb-sparing surgery alone in low-grade soft-tissue sarcoma of the extremity and trunk wall: a systematic review and meta-analysis.

Current guidelines recommend the use of radiotherapy in the management of intermediate and high-grade soft-tissue sarcoma of the extremity and trunk wall. Its use in low-grade sarcoma is less clear. To date there have been no pooled data analyses regarding its role in this context. Its use is not without complications and therefore must be justified. We aim to assess the oncological impact of radiotherapy versus limb-sparing surgery alone in this subset of sarcoma. Medline, EMBASE and Cochrane's databases were searched from 1982 to present. Studies on or having a subgroup analysis of low-grade soft tissue sarcoma, with a radiotherapy and a surgery only arm were included. Outcomes included local recurrence and overall survival. Patients were at least 16 years of age with primary de-novo sarcoma who had not undergone prior resection or treatment. Those undergoing concomitant therapy were excluded. Data extraction was performed independently by two reviewers. Results were pooled using a random-effects model and presented as a forest plot. Primary outcome measures included local recurrence and overall survival. Eleven unique studies were included, consisting of two RCTs and nine non-randomized studies. Overall, there were 12 799 patients. Four studies were included in meta-analysis and the overall pooled effect showed a limited role of radiotherapy in overall survival outcomes when compared to limb-sparing surgery alone HR 1.00 [0.83-1.20] P = 0.41. Descriptive analysis suggests there is limited role of radiotherapy in improving local recurrence outcomes. This study suggests there is limited role for radiotherapy versus limb-sparing surgery alone in low-grade soft-tissue sarcoma. These findings strongly suggest there is lack of high-quality data and that further research must be undertaken prior to forming any strong conclusions regarding the management of low-grade soft-tissue sarcoma. Demonstrating a role for radiotherapy may help improve the quality of excisional margins and thus potentiate limb-sparing surgery.

Primary intradural extraosseous Ewing's sarcoma of the L3 nerve root: illustrative case.

Ewing's sarcoma is an uncommon, aggressive malignancy that typically presents as an osseous lesion, most commonly in children and adolescents. Very rarely Ewing's sarcoma can present as an intradural extramedullary mass mimicking more common tumors. A 32-year-old female had a left L3 nerve root-associated lesion identified in the setting of recent-onset radiculopathy. Contrast-enhanced magnetic resonance imaging of the lumbar spine was favored to demonstrate a schwannoma or neurofibroma. Hemilaminectomy, facetectomy, and resection of the mass led to improved radiculopathy and a tissue diagnosis of Ewing's sarcoma. Immediate referral to medical oncology facilitated expeditious initiation of adjuvant chemotherapy and radiation. The differential diagnosis for newly identified nerve root-associated tumors should remain broad, including common benign pathologies and rare malignant entities. Tissue remains the gold standard for diagnosis, as preoperative imaging suggested a nerve sheath tumor. Malignant pathologies such as Ewing's sarcoma must be considered, especially in the setting of rapidly progressive symptoms or interval growth on serial imaging. Early diagnosis allows for the timely initiation of comprehensive oncological care. Long-term multidisciplinary follow-up is necessary for the surveillance of disease progression.

A primary pulmonary artery sarcoma masquerading pulmonary embolism: a case report and literature review

BackgroundPrimary pulmonary artery sarcoma (PAS) is an extremely rare malignant tumor with a poor prognosis. The clinical manifestations of PAS are diverse, including dyspnea, chest pain, cough, and hemoptysis. The poor prognosis is often due to delayed diagnosis caused by similarity in imaging findings with pulmonary thromboembolism (PTE). These cues of diagnosis include the “wall eclipsing sign”, lobulated bulging margins, gadolinium enhancement during MRI imaging, and FDG uptake during PET/CT imaging. However, there are still many misdiagnoses.Case presentationThis article reports a woman of reproductive age presenting with a pulmonary artery mass. The computed tomographic pulmonary angiography and positron emission tomography/computed tomography did not show obvious signs of pulmonary artery sarcoma, however, contrast-enhanced echocardiography showed moderate perfusion, which helped differentiate between pulmonary artery sarcoma and pulmonary artery thrombosis, leading to timely surgical treatment.ConclusionsPAS is a rare form of cancer that can occasionally be visually similar to PTE on radiographic images. Early diagnosis of PAS is of vital importance to the prognosis of the patients. There are several visual cues that can help differentiate between the two conditions. Additionally, contrast-enhanced echocardiography provides additional information on tumor perfusion, offering another effective approach for a prompt and accurate diagnosis.

Primary pulmonary Ewing sarcoma: a rare case report

Ewing sarcoma (ES) is a malignant soft tissue tumor that consists of undifferentiated neuroectodermal cells. The anatomical sites of ES are commonly the pelvis and long bones. Metastasis is the cause of the most prevalent pulmonary ES. The primary lung origin of ES is extremely uncommon. Here, we report a rare case of primary pulmonary ES diagnosed from cytology and biopsy material. A chest X-ray revealed a lesion with a 9 × 7.5 cm diameter in the pericardiac area of the right lung. Clinical and radiological examinations (computed tomography and positron emission tomography) demonstrated that the lesion was a primary lesion. No distant metastasis was detected. Bronchoscopy-guided fine-needle aspiration and cytological analysis of the lesion revealed uniformly shaped small round cell morphology. Immunohistochemistry performed on the cell block produced positive results for CD99 and FLI-1. These immunohistochemical findings support the ES diagnosis.