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The narrative review article is focused on the strengths and limitations of modern imaging methods in the preoperative differential diagnosis of uterine mesenchymal tumours. In order to tailor the surgical procedures, imaging methods, namely ultrasound and magnetic resonance imaging (MRI), should be taken into account as well as clinical symptoms, age, and fertility plans. On ultrasound scans, uterine sarcomas have the appearance of large, usually solitary tumours of non-homogenous structure with irregular cysts, ill-defined outline borders (interrupted capsule), absence of calcifications with acoustic shadowing, and moderate to rich internal vascularisation. Rapid growth between follow-ups or atypical growth in peri- or post-menopause is also a sign of malignancy. On MRI, uterine sarcomas are characterized by irregular borders, hyperintense areas on T1-weighted and T2- weighted images, and central non-enhancing necrotic areas. On diffusion-weighted imaging (DWI/MRI), sarcomas exhibit markedly restricted diffusion but there is a significant overlap with some variants of fibroids. Core-needle or hysteroscopic biopsy can be used preoperatively if suspicious features are detected on ultrasound or MRI scans, particularly before myomectomy if fertility preservation is required or when conservative management is considered in asymptomatic women. Other imaging methods, such as positron emission tomography fused with CT (PET-CT) or computed tomography (CT) have limited role to distinguish uterine sarcomas from myomas and are suitable only for staging purposes. The importance of tumour markers including lactate dehydrogenase in preoperative work-up have not been verified yet. Conclusion: Uterine sarcomas can be distinguished from much more common myomas based on a combination of malignant features on ultrasound or MR imaging. In these suspicious cases the type and extent of surgery should be adjusted, avoiding intraperitoneal morcellation, which could lead to iatrogenic tumour spread and worsening of the patient's prognosis.

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Introduction: This study seeks to investigate the barriers to care that exist for patients presenting with sarcomas of musculoskeletal origin. Understanding the roots of delays in care for patients with musculoskeletal sarcoma is particularly important given the necessity of prompt treatment for oncologic diagnoses. Investigators reviewed relevant studies of publications reporting barriers to care in patients undergoing diagnosis and treatment of musculoskeletal tumors. Methods: A comprehensive literature search was conducted using Scopus, Embase, Web of Science, and PubMed-MEDLINE. Twenty publications were analyzed, including a total of 114,056 patients. Results: Four barrier subtypes were identified: Socioeconomic Status, Geographic Location, Healthcare Quality, Sociocultural Factors. Socioeconomic status included access to health insurance and income level. Geographic location included distance traveled by patients, access to referral centers, type of hospital system and resource-challenged environments. Healthcare quality included substandard imaging, access to healthcare resources, and healthcare utilization prior to diagnosis. Sociocultural factors included psychological states, nutrition, education and social support. Conclusion: After identifying the most significant barriers in this study, we can target specific public health issues within our community that may reduce delays in care. The assessment of barriers to care is an important first step for improving the delivery of oncologic patient care to this patient population.

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Sarcoma is a rare tumor derived from the mesenchymal tissue and mainly found in children and adolescents. The outcome for patients with sarcoma is relatively poor compared with that for many other solid malignant tumors. Sarcomas have a highly heterogeneous pathogenesis, histopathology and biological behavior. Dysregulated signaling pathways and various gene mutations are frequently observed in sarcomas. The telomere maintenance mechanism (TMM) has recently been considered as a prognostic factor for patients with sarcomas, and alternative lengthening of telomeres (ALT) positivity has been correlated with poor outcomes in patients with several types of sarcomas. Therefore, telomeres and telomerases may be useful targets for treating sarcomas. This review aims to provide an overview of telomere and telomerase biology in sarcomas.

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Soft tissue sarcomas (STS) have long been a formidable challenge in oncology, partly because of their rarity and diversity, which complicates large-scale studies and slows the advent of new treatments. Traditionally anchored by anthracycline-based chemotherapy, the landscape of STS treatment hasn't shifted dramatically in the past twenty years. However, recent strides in research are starting to paint a more hopeful picture. Leveraging advanced molecular profiling, researchers are now tailoring treatments to the unique genetic makeup of tumors, with targeted therapies showing promise. Innovations such as NTRK inhibitors for NTRK-rearranged sarcomas and gamma-secretase inhibitors for desmoid tumors are changing clinical practices. The rise of immunotherapy, including novel agents like LAG-3 inhibitors and bifunctional proteins that target both TGF-ß and PD-L1, offers new avenues for treatment, particularly when combined with traditional therapies like chemotherapy. Meanwhile, the approval of epigenetic treatments for specific sarcoma subtypes heralds a new wave of strategy based on histological specificity, which could lead to more personalized and effective care. While challenges remain, the field of STS treatment is evolving, driven by a deeper understanding of the disease's biological underpinnings and a commitment to innovative research approaches.

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CASE: This case report describes a patient who presented with clinical and radiographic features of a soft tissue sarcoma of the shoulder. Despite having a painless and relatively large mass, a biopsy and resection revealed nodular fasciitis (NF). CONCLUSION: This is an unusual case of a painless 10 cm mass that histopathologically was diagnosed as NF in the upper extremity with proximity to the axillary nerve and posterior humeral circumflex vessels. The USP6 rearrangement was helpful in confirming the diagnosis. Careful clinical, radiographic, and pathologic correlation is necessary in diagnosing these relatively rare tumors. In cases where there are discordant findings, molecular markers can be very helpful.

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Importance: Improved prognostic tools are needed for patients with locally recurrent extremity or truncal soft tissue sarcoma (STS). Objective: To examine the association between average local recurrence (LR) growth rate and outcomes following resection of locally recurrent extremity or truncal STS. Design, Setting, and Participants: This retrospective cohort study used a prospectively maintained database from a single high-volume tertiary sarcoma referral center in the US to identify patients 16 years of age or older who underwent repeat resection of a locally recurrent extremity or truncal STS between July 1, 1982, and December 31, 2021. Patients with atypical lipomatous tumors, desmoid tumors, dermatofibrosarcoma protuberans, angiosarcomas, and prior or synchronous distant recurrence were excluded. Data were analyzed from November 1, 2022, to June 17, 2024. Exposure: Average LR growth rate, defined as the sum of recurrent tumor maximal diameters divided by the disease-free interval after index operation. Main Outcomes and Measures: The primary outcomes were cumulative incidences of disease-specific death (DSD), with death from other causes as a competing risk, and second LR, with death from any cause as a competing risk. Results: The study cohort included 253 patients (median [IQR] age, 64 [51-73] years; 140 [55.3%] male). The 5-year cumulative incidence of DSD after repeat resection was 29%. Multivariable analysis indicated that LR growth rate (hazard ratio [HR], 1.12 [95% CI, 1.08-1.18]; P < .001), younger age (HR, 0.98 [95% CI, 0.97-0.99]; P = .002), R1 or R2 margins (HR, 1.71 [95% CI, 1.03-2.84]; P = .04), high LR grade (HR, 2.90 [95% CI, 1.17-7.20]; P = .02), and multifocality (HR, 2.92 [95% CI, 1.70-5.00]; P < .001) were independently associated with higher incidence of DSD. Using the minimum P value method, the optimal cutoff for growth rate was found to be 0.68 cm/mo. Patients with values above this cutoff had higher 5-year incidences of DSD following repeat resection (63% vs 19%; permutation test P < .001) and higher amputation rates (19% vs 7%; P = .008). Only R1 margins were independently associated with higher incidence of second LR (HR, 1.81 [95% CI, 1.19-2.78]; P = .006). Conclusions and Relevance: In this cohort study of patients undergoing resection of a locally recurrent extremity or truncal STS, LR growth rate was independently associated with DSD. These findings suggest that patients with growth rates higher than 0.68 cm/mo who undergo LR resection may have high disease-specific mortality and amputation rates and should be considered for perioperative systemic therapy.

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Malignant melanoma, a cancer type with a high mortality rate and increasing incidence worldwide, primarily affects the skin but can also occur in various other organs and tissues, including the respiratory tract. Although primary lung malignant melanomas are rare, they are often diagnosed at advanced stages due to their asymptomatic nature, and their atypical presentations may lead to misdiagnosis as other malignancies. In this case report, a mass lesion almost completely filling the right lung initially led to the consideration of small cell lung carcinoma, but a definitive pathological diagnosis could not be obtained in subsequent studies. The diagnosis was confirmed by soft tissue biopsy taken from the anterior thoracic wall. Considering clinical, pathological, and radiological evaluations, possible diagnoses included sarcoma, small-cell lung cancer, and melanoma. The patient, diagnosed through multiple tissue sampling and detailed dermatological examination, presents an interesting atypical case. This case highlights that rare variants of melanoma can be mistaken for other cancer types, such as lung cancer, sarcoma, or lymphoma. Patients may not always present with a noticeable skin lesion; therefore, a meticulous skin examination is crucial in such cases. Malignant melanoma is a noteworthy disease, considering its increasing incidence and early diagnosis holds vital importance for appropriate treatment and management.

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Primary mammary sarcomas are very rare, histologically heterogeneous non-epithelial malignant neoplasms. Primary undifferentiated pleomorphic sarcoma is a rare malignant mesenchymal tumor in the breast. It is characterized by marked cellular atypism and pleomorphism. Isolated cases with an aggressive course and poor prognosis have been commonly described in the literature. We present a rare case of a 62-year-old woman with an 18-cm solid tumor of the left breast, 6 months old, which grew rapidly during the last month. Physical examination and mammography revealed no enlarged lymph nodes in the left axilla. A total mastectomy was performed. The diagnosis of undifferentiated pleomorphic sarcoma is challenging due to the lack of specific immunohistochemical markers. It can only be made after excluding other types of soft tissue sarcomas. This report discusses the histopathological and immunohistochemical studies that were conducted. Our case is distinguished from others with the same diagnosis by the atypical clinical presentation, which is painless, and the spontaneous bleeding, as well as the large size of the tumor.

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INTRODUCTION: The use of free-style and propeller perforator-based flaps has been popularized for the reconstruction of moderate size defects in the trunk and extremities, while their application in the field of abdominal reconstruction is seldom reported. The purpose of this report is to describe the authors experience with the use of pedicled perforator-based flaps in abdominal wall reconstruction, presenting the innovative concept of transition from angiosomal to bi-angiosomal and extra-angiosomal perforator flaps and showing applications of the different flap designs according to the multiple clinical scenarios. PATIENTS AND METHODS: A total of 15 patients underwent abdominal wall reconstruction with angiosomal, bi-angiosomal, and extra-angiosomal pedicled perforator-based flaps harvested from the surrounding abdominal subunits for superficial or full thickness defects of the abdominal wall of moderate and large dimensions. The defects were consequent to soft-tissue sarcomas (STS) and non-melanoma skin cancer (NMSC) resection in 11 and 4 cases, respectively. Operative data, post-operative course, and complications were recorded. Moreover, at 12 months follow-up, patients were asked to rate the esthetic and functional outcomes of the reconstructive procedure on a 5-point Likert scale. RESULTS: Ten angiosomal perforator flaps (4 DIEP, 4 SCIP, 1 SEAP, and 1 LICAP flaps) and 5 bi-angiosomal and extra-angiosomal conjoined perforator flaps including different vascular territories (3 bilateral DIEP, 1 bilateral SEAP, and 1 ipsilateral DIEP-SEAP flap) were successfully transferred in 15 patients. In two patients, microsurgical anastomoses were performed to guarantee proper vascularization of the additional cutaneous territory. Mean age was 59.3 years. Defect sizes ranged from 98 to 408 cm2 (mean size was 194.7 cm2). Mean operative time was 280 min. Flap surface ranged from 108 to 336 cm2 (mean surface was 209.3 cm2). No major complications were registered. One bi-angiosomal bilateral DIEP flap suffered from partial necrosis and required an additional flap reconstruction. All patients underwent a 12-month follow-up except one, who did not show for clinical follow-up but responded at the Likert scale at clinical follow-up at 9 months. Overall patients' satisfaction was high, with mean esthetic and functional ratings of 4.27 and 3.87. CONCLUSION: The use of local tissues is an under-utilized solution in the field of abdominal wall reconstruction. Angiosomal, bi-angiosomal, and extra-angiosomal perforator flaps proved to be a reliable option to provide the transfer of a significant amount of tissue and offer like with like reconstruction while maximizing flap survival.

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BACKGROUND: The antigen processing machinery (APM) plays a critical role in generating tumor-specific antigens that can be recognized and targeted by the immune system. Proper functioning of APM components is essential for presenting these antigens on the surface of tumor cells, enabling immune detection and destruction. In many cancers, defects in APM can lead to immune evasion, contributing to tumor progression and poor clinical outcomes. However, the status of the APM in sarcomas is not well characterized, limiting the development of effective immunotherapeutic strategies for these patients. METHODS: We investigated 126 patients with 8 types of bone and soft tissue sarcoma operated between 2001-2021. Tissue microarrays mapped 11 specific areas in each case. The presence/absence of APM protein was determined through immunohistochemistry. Bayesian networks were used. RESULTS: All investigated sarcomas had some defects in APM. The least damaged component was HLA Class I subunit ß2-microglobulin and HLA Class II. The proteasome LMP10 subunit was defective in leiomyosarcoma (LMS), myxoid liposarcoma (MLPS), and dedifferentiated liposarcoma (DDLPS), while MHC I transporting unit TAP2 was altered in undifferentiated pleomorphic sarcoma (UPS), gastrointestinal stromal tumor (GIST), and chordoma (CH). Among different neoplastic areas, high-grade areas showed different patterns of expression compared to high lymphocytic infiltrate areas. Heterogeneity at the patient level was also observed. Loss of any APM component was prognostic of distant metastasis (DM) for LMS and DDLPS and of overall survival (OS) for LMS. CONCLUSION: Sarcomas exhibit a high degree of defects in APM components, with differences among histotypes and tumoral areas. The most commonly altered APM components were HLA Class I subunit ß2-microglobulin, HLA Class I subunit α (HC10), and MHC I transporting unit TAP2. The loss of APM components was prognostic of DM and OS and clinically relevant for LMS and DDLPS. This study explores sarcoma molecular mechanisms, enriching personalized therapeutic approaches.

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OBJECTIVES: To investigate the clinicopathological characteristics and prognosis of patients with uterine sarcoma treated following surgery for presumed benign disease. METHODS: We identified all patients with uterine sarcoma found incidentally after primary surgery for presumed benign disease who presented to our institution and received re-exploration for completion surgery from January 1, 2004 to January 1, 2021. We analyzed the clinicopathological characteristics and prognosis. RESULTS: Overall, 95 patients were included in our study. For the initial surgery, myomectomy was performed in 50 (52.6%, 50/95) patients, hysterectomy was performed in 45 (47.4%, 45/95) patients. All patients were re-explored to complete the staging operation. The median time to the staging surgery was 40 days (range 15-90 days). There were 29 patients (30.5%, 29/95) had remnant sarcomas, with 17 patients (17/95, 17.9%) on the remaining uterus, 9 patients (9/95, 9.5%) had disseminated diseases, and 4 patients (4/95, 4.2%) had positive lymph nodes. About 40 patients (42.1%) received adjuvant chemotherapy, 55.2% (16/29) and 36.4% (24/66) patients with/without remnant diseases received adjuvant chemotherapy, respectively (P = 0.087). The median follow-up duration was 76.7 months (IQR: 34.8-118.1 months). And 17 patients (17.9%) had recurrence following re-exploration surgery. 5-year progression-free survival (PFS) and 5-year overall survival (OS) for the entire cohort was 81.7% and 92.1%, respectively. Patients with remnant sarcomas had a tendency towards a worse 5-year PFS and 5-year OS, compared with those without (5-year PFS: 75.6% vs. 84.5%, P = 0.224; 5-year OS: 85.5% vs. 95.1%, P = 0.217). Patients with disseminated diseases had a worse 5-year OS (62.5% vs. 95.1%, P = 0.007) and non-significantly worse 5-year PFS (64.8% vs. 83.4%, P = 0.153) compared with those without. CONCLUSIONS: Patients with uterine sarcoma treated following surgery for presumed benign disease have a favorable survival. Patients with disseminated diseases had a worse 5-year OS compared with those without. Surgical re-exploration may be valuable for removing remnant sarcomas and disseminated diseases.

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Bone sarcomas are malignant tumors of mesenchymal origin. Complete surgical resection is the cornerstone of multidisciplinary treatment. However, advanced, unresectable forms remain incurable. A crucial step towards addressing this challenge involves comprehending the molecular mechanisms underpinning tumor progression and metastasis, laying the groundwork for innovative precision medicine-based interventions. We previously showed that tyrosine kinase receptor Ephrin Type-A Receptor 2 (EphA2) is overexpressed in bone sarcomas. EphA2 is a key oncofetal protein implicated in metastasis, self-renewal, and chemoresistance. Molecular, genetic, biochemical, and pharmacological approaches have been developed to target EphA2 and its signaling pathway aiming to interfere with its tumor-promoting effects or as a carrier for drug delivery. This review synthesizes the main functions of EphA2 and their relevance in bone sarcomas, providing strategies devised to leverage this receptor for diagnostic and therapeutic purposes, with a focus on its applicability in the three most common bone sarcoma histotypes: osteosarcoma, chondrosarcoma, and Ewing sarcoma.

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Sarcomas are challenging and conventionally referred to sarcoma specialist centres. In select cases with required surgical expertise, collaboration with a quaternary sarcoma centre rather than an upfront transfer of care may reduce logistic challenges without compromising patient care.We present a case series of three rare tumours of hepatobiliary origin-two cases of undifferentiated embryonal liver sarcoma in adults and one case of follicular dendritic sarcoma of the cystic lymph node.All three patients underwent surgery in a non-sarcoma specialist centre by hepatobiliary specialist surgeons with concurrent remote referrals to a sarcoma specialist quaternary centre. Both centres belong to the same cluster. R0 resection and no significant postoperative morbidity were achieved. All three patients currently remain disease-free.The unique and integrated healthcare systems within Singapore render cross-institution management possible. This case series suggests that an established setup for cross-centre collaboration facilitates wholistic patient care with good outcomes.

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PURPOSE: To investigate the value of multi-parametric MRI-based radiomics for preoperative prediction of lung metastases from soft tissue sarcoma (STS). METHODS: In total, 122 patients with clinicopathologically confirmed STS who underwent pretreatment T1-weighted contrast-enhanced (T1-CE) and T2-weighted fat-suppressed (T2FS) MRI scans were enrolled between Jul. 2017 and Mar. 2021. Radiomics signatures were established by calculating and selecting radiomics features from the two sequences. Clinical independent predictors were evaluated by statistical analysis. The radiomics nomogram was constructed from margin and radiomics features by multivariable logistic regression. Finally, the study used receiver operating characteristic (ROC) and calibration curves to evaluate performance of radiomics models. Decision curve analyses (DCA) were performed to evaluate clinical usefulness of the models. RESULTS: The margin was considered as an independent predictor (p < 0.05). A total of 4 MRI features were selected and used to develop the radiomics signature. By incorporating the margin and radiomics signature, the developed nomogram showed the best prediction performance in the training (AUCs, margin vs. radiomics signature vs. nomogram, 0.609 vs. 0.909 vs. 0.910) and validation (AUCs, margin vs. radiomics signature vs. nomogram, 0.666 vs. 0.841 vs. 0.894) cohorts. DCA indicated potential usefulness of the nomogram model. CONCLUSIONS: This feasibility study evaluated predictive values of multi-parametric MRI for the prediction of lung metastasis, and proposed a nomogram model to potentially facilitate the individualized treatment decision-making for STSs.

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The coronavirus disease (COVID-19) pandemic negatively affected the diagnosis and treatment of several cancer types. However, this pandemic's exact impact and extent on bone and soft tissue sarcomas need to be clarified. We aimed to investigate the effect of the COVID-19 pandemic and emergency declaration by the local government on consultation behavior and clinical stage at diagnosis of bone and soft tissue sarcoma. A total of 403 patients diagnosed with bone and soft tissue sarcoma who initially visited three sarcoma treatment hospitals between January 2018 and December 2021 were included. The monthly number of newly diagnosed soft tissue sarcoma patients was reduced by 25%, and the proportion of soft tissue patients with stage IV disease at diagnosis significantly increased by 9% during the COVID-19 pandemic compared to before the COVID-19 pandemic. Furthermore, the monthly number of new primary bone and soft tissue sarcoma patients significantly decreased by 43% during the state of emergency declaration. The COVID-19 pandemic had a negative impact on soft tissue sarcoma patients' consultation behavior and increased the proportion of advanced-stage patients at initial diagnosis. An emergency declaration by the local government also negatively affected primary bone and soft tissue sarcoma patients' consultation behavior.

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Soft-tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin and account for only 1% of adult malignancies. They tend to occur most commonly in the lower extremities. Reconstruction after sarcoma resection can be challenging, especially when important structures are involved and recurrences occur. Additionally, more attention is now being paid to reconstructing the lymphatic system to prevent lymphatic complications. In this case report, we presented the management of recurrent medial thigh sarcoma that necessitated multiple challenging reconstructions to provide valuable insights for lectures on similar cases. A 50-year-old male patient was diagnosed with an undifferentiated pleomorphic cell sarcoma (UPS) of the anteromedial thigh. After preoperative radiotherapy, a mass of 23 × 15 cm was removed, and reconstruction with a pedicled deep inferior epigastric artery perforator (p-DIEP) flap-based lymphatic flow through (LyFT) was performed. Six months later, the patient developed the first local recurrence with the presence of a distant metastasis. Following the tumor resection, the medial part of the DIEP flap was de-epithelized and buried in the defect for dead space obliteration. Another local recurrence arose 7 months after the second surgery. Therefore, a major debulking surgery involving the femoral neurovascular bundle was performed. The femoral artery was reconstructed with a synthetic graft, and the femoral vein with the great saphenous vein harvested from the contralateral thigh. A composite myocutaneous neurotized anterolateral thigh (ALT) flap from the contralateral thigh was used to obliterate the defect and restore the loss of function of the quadriceps femoris. Two lymphaticovenular anastomoses (LVAs) were performed at the ankle to reduce the risk of lymphatic sequelae. This case report highlights the importance of integrating various techniques to create a tailored approach that effectively addresses complex surgical requirements to avoid limb amputation and maintain functionality.

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Fibrous dysplasia (FD) is a skeletal disorder characterized by the replacement of normal bone by fibrous tissue. Malignant transformation of FD is extremely rare and has been reported in both monostotic and polyostotic forms of FD. The most frequently reported malignant transformation is osteosarcoma. Among malignant bone tumors, spindle cell sarcomas are uncommon and difficult to diagnose. This report presents the case of a 30-year-old woman with an unusual presentation of a malignant undifferentiated spindle cell neoplasm secondary to fibrous dysplasia. The clinical features, radiological findings and management are discussed.

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