Malignant Soft Tissue Sarcoma Research Articles

Etiology, pathogenesis, and management of angiosarcoma associated with implants and foreign body: Clinical cases and research updates.

Angiosarcomas are rare and highly malignant soft tissue sarcomas originating from endothelial cells lining the lymphatic or vascular system. While they predominantly emerge from (sub)cutaneous regions, occurrences have been reported throughout the body. The etiology of angiosarcoma remains elusive in most clinical cases. Nevertheless, several prognosis risk factors play a pivotal role, including chronic lymphedema, therapeutic irradiation, environmental carcinogens, familial syndromes, and the presence of foreign materials like metallic objects and biomedical implants. Despite evidence implicating retained foreign material in angiosarcoma development, understanding its prognosis and pathogenesis remains limited. The pathogenesis of angiosarcoma appears to involve a complex interplay of chronic inflammation, tissue remodeling, and genetic factors that create a conducive microenvironment for malignant transformation. Management of these sarcomas remains challenging due to their infiltrative nature owing to the high chance of metastasis and local recurrence. The primary treatment modalities currently include surgery, radiotherapy, and chemotherapy, but recent advances in targeted immunotherapy and gene therapy hold promise for more effective approaches. This comprehensive review delves into the potential etiological and pathogenic roles of foreign materials, such as metallic objects, biomedical implants, and biomaterials, in the development of angiosarcoma. Further research into the underlying molecular mechanisms could provide valuable insights for tailored management and developing novel targeted therapeutic strategies.

Intracranial malignant peripheral nerve sheath tumor: A case report and comprehensive literature review.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare malignant soft-tissue sarcomas arising from peripheral nerves. Little data exist regarding MPNST originating intracranially. Here, we present a 7th/8th nerve complex MPNST, discuss the treatment strategy and patient outcome, and provide a comprehensive review of existing literature. Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, PubMed and crossed references were queried, yielding 37 publications from 1952 to the present. Fifty-three cases of primary intracranial and extra-axial MPNST were identified. We additionally report a 40-year-old female presented with acute onset dizziness and subsequent hearing loss with associated right-sided facial numbness. Magnetic resonance imaging revealed a 0.5 cm × 1.7 cm enhancing lesion within the right internal auditory canal extending into the cerebellopontine angle. The patient was initially treated with retro sigmoid craniotomy for tumor resection followed by a trans labyrinth approach for residual tumor resection. She completed adjuvant fractionated radiation therapy and underwent facial nerve transfer to restore complete hemifacial paralysis. The most common cranial nerves involved were V and VIII (43.4% each), with 66% of patients male and 34% female. The average age was 43.4 ± 17.4 years. The mean survival time for reported non-survivors after tissue diagnosis was 15 ± 4 months. Two-year survival for patients receiving gross total resection was 33.3% versus 22.8% with subtotal resection. MPNSTs comprise a group of highly aggressive neoplasms that rarely arise intracranially. Gross total surgical resection should be pursued when feasible.

Synovial sarcoma: the misdiagnosed sarcoma.

Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.

Association between periodontal disease and malignant soft tissue sarcomas.

Malignant soft tissue sarcoma (MSTS) is a rare disease, but is seen in patients undergoing orthopedic surgery. Although the association of periodontal disease with various cancers occurring in the oral cavity, gastrointestinal tract, lungs, and prostate, has been reported, the association between periodontal disease and MSTS remains unclear. This study investigated the association between periodontal disease and MSTS in patients undergoing orthopedic surgery. One hundred fifteen patients who underwent orthopedic surgery between 2017 and 2021 were retrospectively enrolled (mean age = 66.8 ± 10.7 years). The patient background was adjusted by the propensity score (PS). Subsequently, the association of periodontal disease with MSTS was analyzed using PS inverse probability of treatment weighting (IPTW). Periodontal status was determined by evaluating the periodontal inflamed surface area, which was calculated by measuring the periodontal probing pocket depth and detecting bleeding on probing. Multivariate logistic regression analysis after adjustment by the PS showed that severe periodontitis was significantly associated with MSTS (odds ratio 2.81, p = 0.04). Furthermore, IPTW showed that severe periodontitis was significantly associated with MSTS (odds ratio 3.21, p = 0.01). The results indicate an association between periodontal inflammation and MSTS.

A novel patient-derived immortalised cell line of myxofibrosarcoma: a tool for preclinical drugs testing and the generation of near-patient models

BackgroundMyxofibrosarcoma is a rare malignant soft tissue sarcoma characterised by multiple local recurrence and can become of higher grade with each recurrence. Consequently, myxofibrosarcoma represents a burden for patients, a challenge for clinicians, and an interesting disease to study tumour progression. Currently, few myxofibrosarcoma preclinical models are available.MethodsIn this paper, we present a spontaneously immortalised myxofibrosarcoma patient-derived cell line (MF-R 3). We performed phenotypic characterization through multiple biological assays and analyses: proliferation, clonogenic potential, anchorage-independent growth and colony formation, migration, invasion, AgNOR staining, and ultrastructural evaluation.ResultsMF-R 3 cells match morphologic and phenotypic characteristics of the original tumour as 2D cultures, 3D aggregates, and on the chorioallantoic membrane of chick embryos. Overall results show a clear neoplastic potential of this cell line. Finally, we tested MF-R 3 sensitivity to anthracyclines in 2D and 3D conditions finding a good response to these drugs.ConclusionsIn conclusion, we established a novel patient-derived myxofibrosarcoma cell line that, together with the few others available, could serve as an important model for studying the molecular pathogenesis of myxofibrosarcoma and for testing new drugs and therapeutic strategies in diverse experimental settings.

PAEDIATRIC-06 PEDIATRIC RECURRENT PRIMARY OCCIPITAL SYNOVIAL SARCOMA: A CASE REPORT

Abstract BACKGROUND Synovial sarcoma is one of the malignant soft tissue sarcomas with an unknown histological origin. It has a unique t(X;18) (p11.2; q11.2) chromosomal translocation, resulting in the SYT-SSX fusion protein. The intracranial occurrence is mainly metastatic, and primary intracranial synovial sarcoma is extremely rare, with few case reports and case series in the literature.Surgical resection of the tumor followed by adjuvant radiotherapy and chemotherapy, regardless of the extent of tumor resection, has been shown to have a significant benefit. CASE PRESENTATION Here we report the case of an eleven-year-old male patient who presented with a four-month history of swelling at the occipital area and intermittent headache. Imaging showed a destructive skull vault lesion with infiltration of the ipsilateral cerebellum. A subtotal resection of the tumor was achieved, and histopathology examination revealed a biphasic variant of synovial sarcoma. The patient disappeared from follow-up without receiving adjuvant radiotherapy and presented two years later with a swelling at the previous surgical site. After imaging was done, a recurrence was diagnosed, and he was reoperated with near total excision. CONCLUSION Primary intracranial synovial sarcoma is an extremely rare malignant tumor. Surgical resection with adjuvant radiation should be the standard of care to prevent recurrence. In low-income countries, patients disappear from follow-up, making post-operative management difficult. Furthermore, radiation services should be made widely available to address issues with compliance.

Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?

Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body's connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. Considering the ongoing trend of increasing lifespans across many nations, MFS is likely to become the most common musculoskeletal sarcoma in the future. Although MFS patients have a lower risk of developing distant metastases compared with other STS cases, MFS is characterized by a high frequency of local recurrence. Notably, in 40-60% of the patients where the tumor recurs, it does so multiple times. Consequently, patients may undergo multiple local surgeries, removing the risk of potential amputation. Furthermore, because the tumor relapses generally have a higher grade, they exhibit a decreased response to radio and chemotherapy and an increased tendency to form metastases. Thus, a better understanding of MFS is required, and improved therapeutic options must be developed. Historically, preclinical models for other types of tumors have been instrumental in obtaining a better understanding of tumor development and in testing new therapeutic approaches. However, few MFS models are currently available. In this review, we will describe the MFS models available and will provide insights into the advantages and constraints of each model.

P11.45.B LOCAL RECURRENCE IN MALIGNANT PERIPHERAL NERVE SHEATH TUMORS: A MULTICENTER COHORT STUDY

Abstract BACKGROUND Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare and aggressive malignant soft-tissue sarcomas (STS) and compromise 5-10% of STS. About 40% of MPNST cases are associated with neurofibromatosis type 1 (NF1). High-grade MPNSTs have a high probability rate of a local recurrence (LR). Risk factors vary in current literature due to its rarity, as does the optimal treatment for LRs. Factors include the role of radiotherapy, surgical margins and NF1. This study investigated both gaps in knowledge. MATERIAL AND METHODS Surgically treated primary MPNSTs between 1988 to 2019 in the MONACO multicenter cohort were included. Multivariate Cox regression analyses were performed to identify factors associated with LR and overall survival (OS) after a LR diagnosis. Treatment of LR was evaluated. RESULTS A total of 507 patients were included in this analysis, of which 142 developed a first LR. A high-grade (HR 3.40; 95% CI, 1.32-8.70) , microscopically positive margins (R1) (HR 3.6; 95% CI, 2.16-6.00), and tumor size (HR 2.14; 95% CI, 1.04-4.42) were independent risk factors for the development of a LR. Radiotherapy (HR 0.43; 95% CI, 0.23-0.79), on the other hand, reduced the risk. Patients with a LR had significantly worse OS. Most LRs (50.7%) were treated surgically without additional therapy. Only a small amount of patients (11.3%) received additional radiotherapy. CONCLUSION This analysis provides important insights into clinicopathologic, and treatment factors associated with a LR and outcomes in MPNST patients. NF1 is not associated with an increased risk of developing a LR1 whereas other clinical factors are.

P11.44.A THE ROLE OF RADIOTHERAPY IN MALIGNANT PERIPHERAL NERVE SHEATH TUMORS: A MULTICENTER COHORT STUDY

Abstract BACKGROUND Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare and aggressive malignant soft-tissue sarcomas (STS), of which 40% are associated with neurofibromatosis type 1 (NF1). Current treatment in localized disease mainly revolves around surgical excision, yet MPNSTs harbor a high probability of local recurrence (LR). Radiotherapy (RT) is increasingly being used, as it improves local control in STS, yet does not impact survival. Nevertheless, its use is controversial in some cases as major wound complications can be much higher and NF1 patients harbor a potential higher risk for secondary malignancies as MPNST commonly arise within plexiform neurofibromas. This study evaluates the use of RT in these rare tumors and tries to understand its impact in local control, especially in the NF1 setting. MATERIAL AND METHODS Surgically treated primary MPNSTs between 1988 to 2019 in the MONACO multicenter cohort were included. Demographic and treatment differences, especially the use of RT, between NF1 and non-NF1 were analyzed. Univariate and multivariable logistic regression analyses were used to identify factors independently associated with the use of radiotherapy. Multivariate Cox regression analyses were performed to identify factors associated with LR in NF1 patients. RESULTS A total of 528 patients (31.8% NF1) were included, of which 149 (28.2%) developed a LR. RT was administered in 53.2% of patients (55.6% in NF1). Of these, 26.7% received neoadjuvant RT, and 71.2% adjuvant RT. The use of both treatment modalities was similar between patients with and without NF1. Multivariable regression showed that microscopically positive margins and high-grade tumors were independent factors for the use of RT. The rate of RT use in NF1 did not alter after correcting for tumor-, patient- and surgical factors. RT did not impact overall survival in sporadic and NF1 associated MPNST. In NF1 patients, microscopically positive margins (R1) (HR 3.2; 95% CI, 1.41-7.62), tumor size >5cm (HR 2.2; 95% CI, 1.14-4.3), and a high grade (HR 3.13; 95% CI, 1.23-7.98) were independent risk factors for the development of a LR. Correcting for tumor-, patient- and surgical factors, use of RT was not associated lower rates of LR, in contrast to the sporadic population. CONCLUSION Radiotherapy is commonly used in MPNST treatment regardless of its origin in NF1. While it may impact LR rate in sporadic patients, this is less clear in NF1 patients. Further studies are needed to evaluate its role and indications in NF1 associated MPNST.

The Value of MRI-Based Radiomic Nomograms in Differential Diagnosis and Metastasis Prediction of Rhabdomyosarcoma and Neuroblastoma in Children

Background: Rhabdomyosarcoma (RMS) and Neuroblastoma (NB) are highly malignant soft tissue sarcoma with tendency to metastasize. Due to the similarities in clinical manifestations and imaging features between RMS and NB, they are often misdiagnosed, which resulted in improper treatment progression of the mass. On the other hand, the treatment paradigm for patients with metastasis RMS/NB and non-metastasis RMS/ NB is different.

Perspectives of Cell Sensitivity/Resistance Assay in Soft Tissue Sarcomas Chemotherapy.

Treatment of highly malignant soft tissue sarcomas (STSs) requires multicomponent therapy including surgery, radiotherapy, and chemotherapy. Despite the advancements in targeted cancer therapies, cytostatic drug combinations remain the gold standard for STS chemotherapy. The lack of algorithms for personalized selection of STS chemotherapy leads to unhelpful treatment of chemoresistant tumors, causing severe side effects in patients. The goal of our study is to assess the applicability of in vitro chemosensitivity/resistance assays (CSRAs) in predicting STS chemoresistance. Primary cell cultures were obtained from 148 surgery samples using enzymatic and mechanical disaggregation. CSRA was performed using resazurin-based metabolic activity measurement in cells cultured with doxorubicin, ifosfamide, their combination and docetaxel, gemcitabine, and also their combination for 7 days. Both the clinical data of patients and the CSRA results demonstrated a higher resistance of some cancer histotypes to specific drugs and their combinations. The correlation between the CSRA results for doxorubicin and ifosfamide and clinical responses to the combination chemotherapy with these drugs was demonstrated via Spearman rank order correlation. Statistically significant differences in recurrence-free survival were also shown for the groups of patients formed, according to the CSRA results. Thus, CSRAs may help both practicing physicians to avoid harmful and useless treatment, and researchers to study new resistance markers and to develop new STS drugs.

Cancer incidence and type of treatment hospital among children, adolescents, and young adults in Japan, 2016-2018.

Cancer in children, adolescents, and young adults (AYAs) although rare, is the leading disease-specific cause of death in Japan. This study aims to investigate cancer incidence and type of treatment hospital among children and AYAs in Japan. Cancer incidence data (2016-2018) for those aged 0-39 years were obtained from the Japanese population-based National Cancer Registry. Cancer types were classified according to the 2017 update of the International Classification of Childhood Cancer (Third Edition), and AYA Site Recode 2020 Revision. Cases were also categorized into three groups: those treated at core hospitals for pediatric cancer treatment (pediatric cancer hospitals [PCHs]), those treated at designated cancer care hospitals, and those treated at nondesignated hospitals. The age-standardized incidence rate was 166.6 (per million-person years) for children (age 0-14 years) and 579.0 for AYAs (age 15-39 years) (including all cancers and benign or uncertain-behavior central nervous system [CNS] tumors). The type of cancer varied with age: hematological malignancies, blastomas, and CNS tumors were common in children under 10 years, malignant bone tumors and soft tissue sarcomas were relatively common in teenagers, and in young adults over 20 years, carcinomas in thyroid, testis, gastrointestinal, female cervix, and breast were common. The proportion of cases treated at PCHs ranged from 20% to 30% for children, 10% or less for AYAs, and differed according to age group and cancer type. Based on this information, the optimal system of cancer care should be discussed.

Synovial sarcoma of female urethra: a case report and review of the literature

Synovial sarcoma (SS) is a rare malignant soft tissue sarcoma that originates from primitive mesenchymal cells with epithelial differentiation potential. It is most commonly found in the limbs and trunk. In the urinary system, it is mostly found in the kidneys. However, synovial sarcomas originating from the external urethra are extremely rare. Only one case of synovial sarcoma arising from the vulvar urethral orifice has been reported previously, and we report a second case of synovial sarcoma of the urethral orifice. In addition, a total of 16 vulvar synovial sarcomas were identified and the literature are analyzed in this report reviews from 1966 to the present.

Postoperative Limb Function and QOL in Elderly Patients With Malignant Bone Tumor/Soft Tissue Sarcoma.

Malignant bone tumors (MBT) and soft tissue sarcomas (STS) require wide excision. Although the number of elderly patients is increasing, wide excision may decrease limb function and quality of life (QOL) for elderly patients. However, no detailed evaluation of the functional prognosis or QOL of elderly patients with sarcoma has been reported. This study evaluated postoperative limb function and QOL in elderly patients with MBT and STS. This retrospective study included 67 patients aged >70 years with MBT or STS who underwent surgery at a single institution. The Toronto Extremity Salvage Score (TESS), EuroQoL 5-dimension 5-level (EQ-5D-5L) questionnaire, Musculoskeletal Tumor Society (MSTS) score, and psoas muscle index (PMI) were evaluated. We also assessed factors associated with the postoperative TESS and EQ-5D-5L index. Detailed examination of the MSTS items perioperatively revealed significant decline in manual dexterity/walking ability and support but significant improvement in pain and emotional acceptance. The mean PMI decreased significantly from 4.7 to 4.23 perioperatively. The postoperative mean TESS and EQ-5D-5L index was 76.9 and 0.74, respectively. Patients with good performance status and clinical frailty scale scores preoperatively had better postoperative TESS and EQ-5D-5L scores. The current study strongly suggests the possibility of maintaining postoperative limb function, satisfaction, and QOL in patients with MBT and STS by choosing patients in good condition and the appropriate procedure that the patient desires. However, perioperative progression of sarcopenia should be noted.

Health-Related Quality of Life of Bone and Soft-Tissue Tumor Patients around the Time of Diagnosis.

Bone and soft-tissue tumor patients experience long-lasting physical and psychological challenges. It is unknown to what extent Health-Related Quality of Life (HRQoL) is already affected during the diagnostic process. This study assesses the HRQoL of bone and soft-tissue tumor patients around time of diagnosis and explores which patient or tumor characteristics are associated with a reduced HRQoL. All patients with a suspected benign/malignant bone tumor (BT), benign soft-tissue tumor (STT), or malignant soft-tissue sarcoma (STS) visiting the Leiden University Medical Center between 2016 and 2020 were invited to complete the Patient-Reported Outcomes Measurement Information System (PROMIS) 29-item profile questionnaire. Mean scores of all included patients and per diagnosis group were compared to mean scores of the general population using one-sample t-tests. Overall, patients (n = 637) reported statistically significantly worse HRQoL-scores on anxiety (51.3 ± 9.6), pain (55.3 ± 10.1), physical functioning (46.0 ± 9.7), and social functioning (48.1 ± 10.8) with the difference in pain and physical functioning being clinically relevant (based on a 3-point difference on t-metric). HRQoL-scores differed between diagnosis subgroups, i.e., patients with malignant tumors had higher anxiety levels and experienced more pain, where patients with bone tumors had worse physical functioning. The HRQoL of patients with suspected bone and soft-tissue tumors is already affected during the diagnostic process.

Inovo chorioallantoic membrane assay as a xenograft model for pediatric rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common highly malignant pediatric soft tissue sarcoma. While recent multidisciplinary treatments have improved the 5‑year survival rate of low/intermediate‑risk patients to 70‑90%, there are various complications that arise due to treatment‑related toxicities. Immunodeficient mice‑derived xenograft models have been widely used in cancer drug research; however, these models have some limitations, including i)they are time‑consuming and expensive, ii)their use needs to be approved by animal experimental ethics committees, and iii)the inability to visualize where tumor cells or tissues were engrafted. The present study performed a chorioallantoic membrane (CAM) assay in fertilized chicken eggs, which is time‑saving, simple, and easy to standardize and handle because of the high vascularization and the immature immune system of the fertilized eggs. The present study aimed to examine the usability of the CAM assay as a novel therapeutic model for the development of precision medicine for pediatric cancer. A protocol was developed for constructing cell line‑derived xenograft (CDX) models using a CAM assay by transplanting RMS cells on the CAM. It was then examined as to whether these CDX models could be used as therapeutic drug evaluation models using vincristine (VCR) and human RMS cell lines. After grafting and culturing the RMS cell suspension on the CAM, three‑dimensional proliferation over time was observed visually and by comparing volumes. VCR reduced the size of the RMS tumor on the CAM in a dose‑dependent manner. Currently, treatment strategies based on patient‑specific oncogenic backgrounds have not been adequately developed in the field of pediatric cancer. The establishment of a CDX model with the CAM assay may lead to the advancement of precision medicine and help formulate novel therapeutic strategies for intractable pediatric cancer.

Oncologic outcomes in myxofibrosarcomas: the role of a multidisciplinary approach and surgical resection margins.

Myxofibrosarcomas (MFS) are malignant soft tissue sarcomas with an infiltrative growth pattern and propensity for local recurrence(LR).We aimed to assess our management of MFS and make recommendations about the role of a multidisciplinary team approach and margin widths. Fifty-seven patients were identified with MFS treated at a single sarcoma centre between 1998 and 2020. Patients were stratified based on whether they presented for a planned resection (59.6%) or after an unplanned resection (40.4%) performed at a non-specialized facility. All patients underwent radiotherapy before definitive surgery. 73.7% underwent a combined onco-plastic approach. The 5year LRFS rate was 78.2% (84.4%, planned, versus 70.1%, unplanned, P =0.194) and found comparable oncological outcomes between the planned and unplanned groups for the 5year metastasis free survival (74.5% versus 86.1%, P=0.257), disease free survival (70.1% versus 72.4%, P=0.677), and Overall Survival (64.5% versus 75.9%, P=0.950). Margin width ≥ 2cm was obtained in 84.2% of cases and improved local control (HR=0.22; 95% CI 0.06-0.81; P=0.023), metastasis (HR=0.24; 95% CI 0.07-0.80; P=0.019) and mortality rates (HR=0.23; 95% CI 0.09, 0.61; P=0.003) compared to <2cm. Margin width > 3cm did not further affect oncological outcomes. Our study shows that a multidisciplinary team approach allows the achievement of low local recurrence rate and good oncological outcomes of myxofibrosarcomas, regardless of presentation status. We recommend a minimum of 2cm margin width.

Does the apparent diffusion coefficient from diffusion-weighted MRI imaging aid in the characterization of malignant soft tissue tumors and sarcomas.

To identify trends in apparent diffusion coefficient (ADC) measurements from diffusion-weighted imaging (DWI) with respect to tumor type classification and other tumor characteristics whether common malignant soft tissue tumors can be distinguished. A consecutive series of extremity malignant soft tissue tumors and soft tissue sarcomas (STS) among 78 adult patients with conventional MRI and DWI were included. Each case was evaluated with respect to T1/T2 signal alterations and heterogeneity, presence of peritumoral edema, necrosis, cystic changes, internal hemorrhage, and maximum longitudinal dimension blinded to the histology. The ADC mean and minimum were obtained using a free-hand region of interest of the whole tumor and the darkest (lowest signal area) ADC area of the tumor. Kruskal-Wallis and Wilcoxon Rank-Sum Tests were used to determine associations and significance between tumor subtypes. Intraclass correlation (ICC) and kappa calculations were utilized to assess inter-reader agreements for ADC values and reader diagnosis. Liposarcomas showed more heterogenous T1W images with hyperintense T1W signal when compared to tumors not classified as liposarcoma (P = 0.046 and P = 0.010, respectively). Liposarcomas were relatively consistent in demonstrating an absence of hemorrhage (81.8%) while undifferentiated pleomorphic sarcomas consistently showed intralesional hemorrhage (90%). When comparing individual tumor classifications against the rest of the samples, lymphomas registered lower mean and minimum ADC values in the whole tumor and in themost hypointense area of the tumor for both readers (P < 0.05). The interobserver agreement between the two readers was good to excellent for all four ADC measurements (ICC = 0.65-0.98). Diffusion-weighted imaging generated ADC measurements are reproducible but currently offer limited insight in being able to differentiate among different malignant soft tissue tumor and sarcoma histologies.T1W and T2W signal characteristics also offer limited insight indifferentiating between soft tissue malignancies.

Incidence and survival of soft tissue sarcoma in England between 2013 and 2017, an analysis from the National Cancer Registration and Analysis Service.

There is a paucity of population-based data detailing the incidence and survival of patients with soft tissue sarcoma (STS), in part due to the heterogeneity of disease and changes to classification. Here, the incidence and survival of all STS subtypes registered in England between 2013 and 2017 were analysed using cancer registry data held by the National Cancer Registration and Analysis Service. Age-standardised incidence rates were calculated per 1 000 000 using the 2013 European Standard Population. Net survival was computed using Brenner's alternative method, with the Ederer II estimator. Age-specific overall survival was assessed using Kaplan-Meier. The influence of age, sex, socioeconomic deprivation and diagnostic routes on survival was assessed using Cox proportional hazards modelling. In total, 19 717 patients were diagnosed with STS, an average of 3943 patients per year and representing approximately 0.8% of malignancies. The most common histological diagnoses were Gastrointestinal Stromal Tumours (GIST), leiomyosarcoma and undifferentiated sarcoma, accounting for 20.2%, 13.3% and 12.7% of all sarcomas, respectively. Five-year net survival for all malignant STS was 65.0%; and was lowest for patients with vascular tumours at 39%. Patients from most deprived cohorts had 23% greater chance of dying within 5 years than patients in least deprived areas. This population-based study has allowed us for the first time to define the incidence and survival rates of prevalent STS subtypes in England such as GIST, liposarcoma and leiomyosarcoma, as well as rare entities and groups with inferior outcome. This data is invaluable for service provision, benchmarking and addressing inequality.

Multimodality imaging evaluation of nasal sinus alveolar rhabdomyosarcoma: Two case reports

Rhabdomyosarcoma (RMS) is a common pleomorphic malignant soft tissue sarcoma in children and adolescents that originates from rhabdomyoblasts or mesenchymal precursor cells. Alveolar rhabdomyosarcoma (ARMS) mostly occurs in adolescents aged 10-15 years and is characterized by more aggressive behaviors and worse prognosis than other sarcomas, prone to lymphatic and hematogenous metastasis in the early stage as well as metastasizing to breast, testis, pancreas, and other parts. ARMS often occurs in the limbs and genitourinary system, however, head and neck ARMS are relatively rare when involving the nasal cavity or sinuses. The role of MRI and 18F-FDG positron emission tomography combined with computed tomography (PET/CT) remains to be established in ARMS. Case 1: An 18-year-old male was found with a left submandibular mass of approximately 1 cm in diameter 2 months ago, which gradually increased in size. CT showed multiple soft tissue masses in maxillofacial and neck regions and the lesions invaded the frontal lobe and the inner wall of the left orbital lobe. MRI showed the masses with hypointensity on T1WI, hyperintensity on T2WI, and diffusion-weighted imaging (DWI) with significant enhancement. 18F-FDG PET/CT showed multiple hypermetabolic lesions located in the maxillofacial, neck region, 3rd lumbar vertebra, and the right sacrum. A nasal endoscopic tumor biopsy and molecular testing finally helped to diagnose the ARMS. Case 2: A 14-year-old male presented with left maxillary pain with nasal congestion and left ocular swelling 15 days ago. CT demonstrated a soft tissue mass in the nasal cavity and sinuses with local protrusion into the left orbit. MRI showed the masses with a slightly low signal on T1WI, a high signal on T2WI, and DWI with significant heterogenous enhancement. 18F-FDG PET/CT showed hypermetabolic lesions in the left maxillofacial and neck regions. ARMS was finally diagnosed by a nasal endoscopic tumor biopsy and molecular testing. The patient had a recurrence of the lesion after chemotherapy and surgical resection and is currently undergoing radiation therapy. Nasal sinus ARMS is highly malignant with a poor prognosis. Accurate diagnosis relies not only on histopathology and immunohistochemistry examination but also on genetic detection of characteristic chromosomal translocations and fusion genes. Imaging methods, such as MRI and PET/CT can accurately assess the extent of the lesions and metastases, assist in the diagnosis of the disease and the selection of treatment regimens, provide precise localization for surgery, and help with treatment monitoring and follow-up.