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Objectives: Early, accurate diagnosis is crucial for the prognosis of patients with soft tissue sarcomas. To this end, standardization of imaging algorithms, technical requirements, and reporting is therefore a prerequisite. Since the first European Society of Musculoskeletal Radiology (ESSR) consensus in 2015, technical achievements, further insights into specific entities, and the revised WHO-classification (2020) and AJCC staging system (2017) made an update necessary. The guidelines are intended to support radiologists in their decision-making and contribute to interdisciplinary tumor board discussions. Materials and methods: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements were scored online by level of agreement (0 to 10) during two iterative rounds. Either "group consensus," "group agreement," or "lack of agreement" was achieved. Results: Eight sections were defined that finally contained 145 statements with comments. Overall, group consensus was reached in 95.9%, and group agreement in 4.1%. This communication contains the first part consisting of the imaging algorithm for suspected soft tissue tumors, methods for local imaging, and the role of tumor centers. Conclusion: Ultrasound represents the initial triage imaging modality for accessible and small tumors. MRI is the modality of choice for the characterization and local staging of most soft tissue tumors. CT is indicated in special situations. In suspicious or likely malignant tumors, a specialist tumor center should be contacted for referral or teleradiologic second opinion. This should be done before performing a biopsy, without exception. Clinical relevance: The updated ESSR soft tissue tumor imaging guidelines aim to provide best practice expert consensus for standardized imaging, to support radiologists in their decision-making, and to improve examination comparability both in individual patients and in future studies on individualized strategies. Key Points: • Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors. • MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy. • In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy. [ABSTRACT FROM AUTHOR]

Lipofibromatosis‐like neural tumors (LPF‐NTs) are a recently discovered group of spindle cell tumors defined by the presence of a lipofibromatosis‐like pattern, CD34 and/or S100 reactivity, and frequent neurotrophic receptor tyrosine kinase 1 (NTRK1) gene rearrangements. As new cases emerge, the spectrum of features observed in LPF‐NTs continues to evolve. Here we describe the case of an 11‐year‐old with LPF‐NT with a dermatofibrosarcoma protuberans‐like honeycomb pattern, CD34 and S100 co‐expression, and an NTRK1 rearrangement. We also review the clinical and molecular features of the 73 cases of LPF‐NT previously described in the literature. [ABSTRACT FROM AUTHOR]

Pseudomyogenic hemangioendothelioma (PHE) is a rare neoplasm with vascular origin mostly seen on the extremities. Up to date, the terminology which represents PHE has been a subject to debate. According to the World Health Organization, PHE is classified among the soft tissue tumors. PHE is mostly seen in soft tissue planes and the distal lower extremities are the most affected location of the body. Localization of this tumor on the head and neck region is uncommon. PHE, being a sarcoma does not favor lymphogenic metastasis. As far as we are concerned, this is the first described case of PHE of the external ear and retro auricular sulcus. In this paper, the management of this case and the current treatment options of PHE are discussed. [ABSTRACT FROM AUTHOR]

Patients with locally extensive high-grade extremity soft tissue sarcomas (eSTS) are often presented in multidisciplinary teams to decide between ablative surgery (amputation) or limb-salvage surgery supplemented with either neo-adjuvant radiotherapy (RT) or induction isolated limb perfusion (ILP). In The Netherlands, ILP typically aims to reduce the size of tumors that would otherwise be considered irresectable, whereas neo-adjuvant RT aims mainly at improving local control and reducing morbidity of required marginal margins. This study presents a 15-year nationwide cohort to describe the oncological outcomes of both pre-operative treatment strategies. All consecutive patients with locally extensive primary high-grade eSTS surgically treated between 2000 and 2015 at five tertiary sarcoma centers that received neo-adjuvant ILP or RT were included. 169 patients met the inclusion criteria (89 ILP, 80 RT). Median follow-up was 7.3 years. Limb salvage was achieved in 84% of cases in the ILP group (80% for patients with amputation indication) and 96% of cases in the RT group. 5-Year overall survival was 47% in the ILP group, 69% in the RT group. 5-Year local recurrence rate was 14% in the ILP group, 10% in the RT group. Distant metastasis rate was 55% in the ILP group, 36% in the RT group. We find oncological outcomes and limb salvage rates in line with existing literature for both treatment modalities. Whether the tumor was locally advanced with an indication for induction therapy to prevent amputation or morbid surgery appeared to be the main determinant in choosing between neo-adjuvant ILP or RT. [ABSTRACT FROM AUTHOR]

Ectomesenchymal chondromyxoid tumor is a rare and benign neoplasm with a predilection for the anterior dorsal tongue. Despite morphologic heterogeneity, most cases are characterized by a proliferation of bland spindle cells with a distinctive reticular growth pattern and myxoid stroma. The immunophenotype of these neoplasms is likewise variable; most cases express glial fibrillary acid protein and S100 protein, with inconsistent reports of keratin and myoid marker expression. The molecular pathogenesis is poorly understood; however, a subset of cases has been reported to harbor EWSR1 gene rearrangement. Following identification of an RREB1-MKL2 fusion gene by RNA Sequencing in an index patient, a retrospective review of additional cases of ectomesenchymal chondromyxoid tumors was performed to better characterize the clinical, immunohistochemical, and molecular attributes of this neoplasm. A total of 21 cases were included in this series. A marked predisposition for the dorsal tongue was confirmed. Most cases conformed to prior morphologic descriptions; however, hypercellularity, hyalinized stroma, and necrosis were rare attributes not previously emphasized. The neoplastic cells frequently coexpressed glial fibrillary acid protein, S100 protein, keratin, smooth muscle actin, and/or desmin; a single case was found to contain significant myogenin expression. An RREB1-MKL2 fusion product was identified in 19 tumors (90%), a single tumor (5%) had an EWSR1-CREM fusion product, and the remaining case lacked any known fusion gene by RNA Sequencing. The latter 2 cases subtly differed morphologically from many in the cohort. This series illustrates that recurrent RREB1-MKL2 fusions occur in most, perhaps all, cases of ectomesenchymal chondromyxoid tumor.

Objectives: The revised European Society of Musculoskeletal Radiology (ESSR) consensus guidelines on soft tissue tumor imaging represent an update of 2015 after technical advancements, further insights into specific entities, and revised World Health Organization (2020) and AJCC (2017) classifications. This second of three papers covers algorithms once histology is confirmed: (1) standardized whole-body staging, (2) special algorithms for non-malignant entities, and (3) multiplicity, genetic tumor syndromes, and pitfalls., Materials and Methods: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements that had undergone interdisciplinary revision were scored online by the level of agreement (0 to 10) during two iterative rounds, that could result in 'group consensus', 'group agreement', or 'lack of agreement'., Results: The three sections contain 24 statements with comments. Group consensus was reached in 95.8% and group agreement in 4.2%. For whole-body staging, pulmonary MDCT should be performed in all high-grade sarcomas. Whole-body MRI is preferred for staging bone metastasis, with [ 18 F]FDG-PET/CT as an alternative modality in PET-avid tumors. Patients with alveolar soft part sarcoma, clear cell sarcoma, and angiosarcoma should be screened for brain metastases. Special algorithms are recommended for entities such as rhabdomyosarcoma, extraskeletal Ewing sarcoma, myxoid liposarcoma, and neurofibromatosis type 1 associated malignant peripheral nerve sheath tumors. Satisfaction of search should be avoided in potential multiplicity., Conclusion: Standardized whole-body staging includes pulmonary MDCT in all high-grade sarcomas; entity-dependent modifications and specific algorithms are recommended for sarcomas and non-malignant soft tissue tumors., Clinical Relevance Statement: These updated ESSR soft tissue tumor imaging guidelines aim to provide support in decision-making, helping to avoid common pitfalls, by providing general and entity-specific algorithms, techniques, and reporting recommendations for whole-body staging in sarcoma and non-malignant soft tissue tumors., Key Points: An early, accurate, diagnosis is crucial for the prognosis of patients with soft tissue tumors. These updated guidelines provide best practice expert consensus for standardized imaging algorithms, techniques, and reporting. Standardization can improve the comparability examinations and provide databases for large data analysis., (© 2024. The Author(s).)

Objective: To describe the MRI features of paediatric conventional central chondrosarcoma (CC-CS) and correlate with histological grade. Materials and methods: A retrospective review of children/adolescents with histologically confirmed CC-CS. Data collected included age, sex, skeletal location, and histology from needle biopsy or resection, which was classified as atypical cartilaginous tumours/grade 1 CS (ACT/Gd 1 CS), high-grade chondrosarcoma (HGCS), and dedifferentiated chondrosarcoma (DD-CS). MRI studies were reviewed independently by 2 radiologists blinded to the histology grade, who graded the tumours as ACT/Gd 1 CS, HGCS, and DD-CS based on MRI features. Results: The study included 7 males and 10 females with mean age 13.9 years (range 6–18 years). Tumours were located in the femur (n = 6), humerus (n = 3), tibia, ilium, scapula, and ulna (n = 1 each), and the small bones of the hands or feet (n = 4). Final histology grade was ACT/Gd 1 CS in 15 cases and HGCS in 2 (both grade 1 CS with focal transition to grade 2), 15 based on surgical specimens, 1 based on open biopsy, and 1 on needle biopsy alone. Predicted MRI grade for the 2 readers was ACT/Gd 1 CS in 11 cases each and HGCS in 6 cases each, indicating a mismatch between predicted MRI grade and histological grade in 8 (47%) cases (4 cases with one reader mismatch and 4 cases with both). Conclusions: MRI findings in paediatric CC-CS may be misleading, showing features suggestive of HGCS 7 of 17 (41.2%) of cases. This should be taken into consideration when planning surgical treatment. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Sarcomas account for less than 1% of all cancers. Undifferentiated Pleomorphic Sarcoma, formerly called Malignant Fibrous Histiocytoma, is a rare subtype identified by a lack specific immunohistochemical markers for a specific lineage of differentiation. These soft tissue tumors are aggressive and rapidly enlarge. Risk for metastasis increases almost linearly as the tumor increases in size, emphasizing the importance of early detection, treatment, and post-resection monitoring. Case Report: This article reports a case of a large undifferentiated pleomorphic sarcoma of the posterior thigh in a 62-yearold female. Given the patient's history of thrombotic thrombocytopenic purpure, her initial mass was thought to be a hematoma following a hernia repair surgery. After diagnosis of undifferentiated pleomorphic sarcoma, she underwent radical excision revealing a 24×9.5×7cm lesion - one of the largest reported in the literature. Conclusions: Sarcomas are very rare soft tissue neoplasms, but they should not be excluded in a physician's differentials when a patient presents with an enlarging soft tissue mass. Because sarcomas enlarge rapidly, delay in evaluation and management should be avoided and these patients should be quickly referred to a center specializing in sarcoma treatment. Magnetic Resonance Imaging (MRI) is the recommended initial imaging for all soft tissue masses of the extremities, trunk, and head and neck while Computed Tomography (CT) is the recommended imaging choice for retroperitoneal and visceral masses. After successful surgical excision with clean margins, patients should undergo serial monitoring by CT or MRI for surveillance of recurrence or late pulmonary metastases. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinicopathological and morphological features of gastrointestinal stromal tumours (GISTs) on multi-detector computed tomography (MDCT). Material and methods: MDCT (plain and post contrast) images of 25 confirmed cases of GISTs were retrospectively evaluated from our hospital database. The images were analysed for the location, size, growth pattern, attenuation pattern, relation to adjacent structures, presence or absence of ulceration, calcification, metastases, lymphadenopathy, and for any complications such as haemorrhage, intestinal obstruction, etc. Institutional Ethics Committee clearance was obtained prior to the commencement of the study. Statistics used included percentage frequency. Results: Our study group comprised 14 males and 11 females. The mean age of our study population was 60 years (age range: 40 to 82 years). The mean tumour size was 11.7 cm. The stomach and small bowel accounted for 76% of the primary tumour site. The commonest imaging appearance of GIST observed in our study was that of an exophytic mass (76%) with a heterogenous pattern of enhancement (96%) with intratumoural necrosis (76%). MDCT demonstrated complications in three patients (12%). Six patients presented with metastatic foci (five to the liver and one to the lung), while lymphadenopathy was observed in five patients (20%). Associated complications included intestinal obstruction (8%) and retrogastric haematoma (4%). Incidental findings included uterine fibroid (n = 1), ovarian dermoid (n = 1), and chronic pancreatitis (n = 1). Conclusions: GISTs are predominantly large tumours with a well circumscribed and exophytic pattern on MDCT, with or without cystic/necrotic areas, and they mostly show a heterogenous pattern of enhancement on post-contrast administration. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) plays a critical role in the management pathway of both soft tissue and bone neoplasms, from diagnosis through to post-treatment follow-up. There are a wide range of surgical, oncological, and combined treatment regimes but these rely on accurate histopathological diagnosis. This article reviews the role of MRI in the planning of image-guided needle biopsy for suspected soft tissue and bone tumors. [ABSTRACT FROM AUTHOR]

Introduction Ultrasonography is associated with a high error rate in the evaluation of soft tissue masses. The purposes of this study were to examine the nature of the diagnostic errors and to identify areas in which reporting could be improved. Methods Patients who had soft tissue tumours and received ultrasonography during a 10-year period (1999-2009) were identified from a local tumour registry. The sonographic and pathological diagnoses were categorised as either 'benign' or 'non-benign'. The accuracy of ultrasonography was assessed by correlating the sonographic with the pathological diagnostic categories. Recommendations from radiologists, where offered, were assessed for their appropriateness in the context of the pathological diagnosis. Results One hundred seventy-five patients received ultrasonography, of which 60 had 'non-benign' lesions and 115 had 'benign' lesions. Ultrasonography correctly diagnosed 35 and incorrectly diagnosed seven of the 60 'non-benign' cases, and did not suggest a diagnosis in 18 cases. Most of the diagnostic errors related to misdiagnosing soft tissue tumours as haematomas (four out of seven). Recommendations for further management were offered by the radiologists in 144 cases, of which 52 had 'non-benign' pathology. There were eight 'non-benign' cases where no recommendation was offered, and the sonographic diagnosis was either incorrect or unavailable. Conclusions Ultrasonography lacks accuracy in the evaluation of soft tissue masses. Ongoing education is required to improve awareness of the limitations with its use. These limitations should be highlighted to the referrers, especially those who do not have specific training in this area. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to evaluate the efficacy, adverse reaction and survival of MAID protocol as first-line treatment on 137 metastatic soft tissue sarcomas patients. Methods: One hundredand thirty-seven patients with metastatic soft tissue sarcoma were given MAID protocol chemotherapy which consists of ifosfamide at a total dose of 8-10 g/m in 4-5 days, adriamycin at a dose of 60 mg/m on first day and dacarbazine at a dose of 200 mg/m/d in 4-5 days. Twenty one days were regarded as a cycle and 2-6 cycles were completed. The median cycle number was 4. Results: Four cases had complete remission (CR) (2.9%), thirteen cases achieved partial remission (PR) (9.5%), eighty-eight cases maintained a stable disease (SD) (64.2%), and thirty-two cases had progressive disease (PD) (23.4%). The overall response rate (RR) was 12.4%, disease control rate (DCR) was 76.6%. The progression-free survival (PFS) was 2-14 months and median PFS was 5.00 ± 1.12 months. The overall survival (OS) was 3-19 months and median OS was 8.00 ± 1.32 months. Three months PFS rate was 56.9%, six months PFS rate was 27.2% and one year PFS rate was 7.8%. Three months OS rate was 100.0%, six months OS rate was 41.5% and one year OS rate was 10.7%. The main adverse reactions were bone marrow supression, nausea/vomiting and alopecia. Other adverse reactions were seldom observed. Conclusion: The effect of MAID protocol as first-line treatment on metastatic soft tissue sarcomas patients was exact, the treatment can control disease progression effectively and the side effects can be tolerable. It was obviously very worthy in clinical use as one of the first-line chemotherapy protocols on advanced soft tissue sarcomas. [ABSTRACT FROM AUTHOR]

Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis Intra-abdominal spindle cell lesions are uncommon and often present a diagnostic challenge. An important group of such lesions are the gastrointestinal stromal tumours. Other intra-abdominal spindle cell lesions include fibromatosis, various sarcomas—in particular, leiomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumour—and, in women, endometrial stromal sarcoma. Less common lesions are inflammatory myofibroblastic tumours, the mesenteric spindle cell reactive lesions, retroperitoneal fibrosis, and solitary fibrous tumour. A variety of intra-abdominal tumours of nonmesenchymal origin may have a spindle cell/sarcomatoid morphology; these include sarcomatoid carcinoma, malignant melanoma and, in women, sarcomatoid granulosa cell tumour. Finally, metastatic sarcomas from pelvic or extra-abdominal organs need also be considered. A set of practical aids to the diagnosis of intra-abdominal spindle cell lesions is presented to assist pathologists dealing with such lesions, particularly with regards to the consideration of differential diagnoses. [ABSTRACT FROM AUTHOR]

Introduction: The aims of this study were to report the presenting characteristics and identify how best to distinguish bone and soft-tissue infections that mimic sarcomas., Materials and Methods: A total of 238 (211 osteomyelitis and 27 soft-tissue infections) patients referred to a tertiary sarcoma multidisciplinary team with suspected sarcoma who were ultimately diagnosed with a bone or soft tissue infection were included. Data from a prospectively collated database was analysed retrospectively., Results: Of all possible bone and soft-tissue sarcoma referrals, a diagnosis of infection was made in 2.1% and 0.7%, respectively. Median age was 18 years in the osteomyelitis group and 46 years in the soft-tissue infection group. In the osteomyelitis group, the most common presenting features were pain (85.8%) and swelling (32.7%). In the soft-tissue infection group, the most common clinical features were swelling (96.3%) and pain (70.4%). Those in the soft-tissue group were more likely to have raised inflammatory markers. Radiological investigations were unable to discern between tumour or infection in 59.7% of osteomyelitis and 81.5% of soft-tissue infection cases. No organism was identified in 64.9% of those who had a percutaneous biopsy culture., Conclusions: This study has highlighted that infection is frequently clinically indistinguishable from sarcoma and remains a principle non-neoplastic differential diagnosis. When patients are investigated for suspected sarcoma, infections can be missed due to falsely negative radiological investigations and percutaneous biopsy. As no single clinical, biochemical or radiological feature or investigation can be relied upon for diagnosis, clinicians should have a low threshold for tissue biopsy and discussion in a sarcoma multidisciplinary team meeting.