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Background: Pulmonary bronchial leiomyosarcoma is an extremely rare malignant tumour of the lung originating from the mesenchymal tissue. The retroperitoneal region is the most common site of leiomyosarcoma. It exhibits a high degree of malignancy and a poor prognosis, thereby highlighting the significance of early diagnosis of this disease., Case Demonstration: A 61-year-old male with a smoking history of 40 + years was admitted to the hospital due to cough, expectoration, and shortness of breath that extended for half a year, which aggravated for two months and was succeeded by intermittent bloody sputum for one month. Chest computed tomography (CT) revealed a mass in the upper lobe of the right lung, which was identified as a malignant tumour lesion involving the upper thoracic oesophagus. The boundaries of the mass with the trachea, brachiocephalic artery, and right brachiocephalic vein were unclear. Brush liquid-based cytology of the right middle lobe bronchus was performed, followed by histopathological biopsy. The pathological diagnosis was leiomyosarcoma in the middle lobe of the right lung. After the diagnosis, he underwent chemotherapy in another hospital, and the case was followed up for six months, during which no recurrence was noted., Conclusions: Pulmonary and bronchial leiomyosarcomas often occur in young males with rapid progression and poor prognosis, but metastasis is rare. Surgical resection is the first-line treatment. We report a case of pulmonary bronchial leiomyosarcoma. The cytological, histological, immunohistochemical, and differential diagnoses were analysed to improve the understanding of the disease. Presently, there exist some challenges in diagnosing and treating pulmonary bronchial leiomyosarcoma. Early detection and treatment are important to improve prognosis., Competing Interests: Declarations. Ethics approval and consent to participate: This case report was approved by the Ethics Committee of the Affiliated Hospital of Zunyi Medical University. Written informed consent was obtained from the patient and the patient’s family for publication of this clinical case report. Consent for publication: Written informed consent was obtained from the patient and the patient’s family for publication of this case report and any accompanying images. Competing interests: The authors declare no competing interests., (© 2025. The Author(s).)

Primary renal leiomyosarcoma is a rare but highly aggressive tumor with a poor prognosis. Early detection and surgical excision provide the most effective initial treatment. However, vigilant postoperative surveillance is critical due to the elevated risk of tumor recurrence and metastasis, as seen in this case., Competing Interests: The authors declare no conflicts of interest., (© 2025 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: Superficial leiomyosarcoma is a rare malignant soft tissue tumor arising from smooth muscle cells, accounting for 2-3% of superficial sarcomas, with limited literature available on the subject. It is typically observed in patients aged 50-60 years and affects both men and women equally in the subcutaneous subtype, whereas the cutaneous subtype predominantly affects men., Objective: This study aims to examine the clinicopathological features and therapeutic outcomes of patients with leiomyosarcoma., Method: This is a descriptive retrospective study of 26 cases of cutaneous and subcutaneous leiomyosarcomas, with histological confirmation., Results: We identified 10 (38.5%) subcutaneous leiomyosarcomas and 16 (61.5%) cutaneous leiomyosarcomas. The majority of tumors were located in the lower limbs, accounting for 13 (50%) cases. During follow-up, 6 patients experienced recurrence, and 7 developed metastases, including 2 of the 7 patients who had R0 resection margins. Among these, 3 out of the 6 recurrent cases and 3 out of the 7 metastatic cases were subcutaneous leiomyosarcomas. The average time to recurrence was 6.2 years., Conclusion: The observed risk of metastases and recurrences, despite clear surgical margins, in both cutaneous and subcutaneous leiomyosarcomas, along with the delayed onset of these events, justifies prolonged patient follow-up. The lungs, bones and liver have been identified as the most common site of metastasis., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background: In this single-arm, multicenter, phase 2 trial, the authors evaluated the efficacy and safety of avelumab plus gemcitabine in patients with leiomyosarcoma (LMS) who failed on first-line chemotherapy., Methods: Patients with advanced LMS received avelumab 10 mg/kg on days 1 and 15 (for up to 24 months) plus gemcitabine 1000 mg/m 2 on days 1, 8, and 15 of a 28-day cycle until they developed disease progression or intolerable toxicity. The primary end point was the objective response rate (ORR)., Results: In total, 38 patients were enrolled. Of these, 35 patients were evaluable, and the ORR was 20% (95% confidence interval; [CI], 8%-37%). The disease control rate was 71%, and the median duration of response was 21.8 months (range, 7.6 to ≥43.3 months). The median progression free-survival was 5.6 months (95% CI, 4.5-6.8 months), and the median overall survival was 27.5 months (95% CI, 20.4-34.6 months). Grade 3-4 adverse events occurred in 70% of patients, of which neutropenia was the most common (54%). Immune-mediated adverse events occurred in five patients (14%; hypothyroidism, n = 3; hepatitis, n = 2). Patients who had a higher density of tumor-infiltrating lymphocytes (greater than the median) exhibited better ORR (35% vs. 8%; p = .104), progression-free survival (median, 7.3 vs. 3.3 months; p = .024), and overall survival (median, not reached vs. 21.5 months; p = .027)., Conclusions: The combination of avelumab and gemcitabine demonstrated promising efficacy and manageable safety in patients with advanced LMS who progressed on first-line therapy. Tumor-infiltrating lymphocyte density may be an important factor in predicting the response to combining immunotherapy with chemotherapy., (© 2024 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.)

Background: Uterine fibroids are benign monoclonal neoplasms of the myometrium, representing the most common female pelvic neoplasms globally. Treatments may be invasive, such as hysterectomy and myomectomy, non-invasive, such as medical therapy or focused ultrasound, or minimally invasive, such as transcervical radiofrequency ablation (TFA). To date, more than 12,000 women have been treated worldwide using TFA with the Sonata ® System. Case Presentation: We present the first case report of TFA on a presumptive fibroid that was initially reclassified as a STUMP (smooth muscle tumor of uncertain malignant potential) and, after additional surgical treatment, leiomyosarcoma. Conclusion: This case highlights that, while uterine sarcoma is rare, inadvertent treatment may still result due to a lack of reliable diagnostic modalities. Nonetheless, TFA with the Sonata System represents a minimally invasive option that might not alter the prognosis of an undiagnosed uterine sarcoma as this treatment is not intraperitoneal and does not resect/morcellate tissue.

The distinction between a uterine leiomyosarcoma (uLMS) and a perivascular epithelioid cell neoplasm (PEComa) can be quite challenging. Here we report a 39-year-old woman who underwent a hysteroscopic myomectomy. An intraoperative frozen section pathological examination revealed that the mass was likely to be a mesenchymal malignancy. After consultation with her family, a total hysterectomy and bilateral salpingo-oophorectomy were performed. Postoperative pathological examinations suggested leiomyosarcoma but a malignant PEComa cannot be completely excluded. Combining the present case and prior studies, we summarized the clinical manifestations, pathological features, genomic characterization, and treatment of LMS and PEComa., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Chen, Zheng and Lin.)

Leiomyomas are uncommon tumors of the gastrointestinal system, representing around 0.03% to 0.05% of all rectal tumors. They tend to have a benign biological behavior and are mostly asymptomatic. When leiomyomas are large in size, they can cause symptoms and complications, including abdominal pain, perforation, intestinal obstruction, and bleeding. We herein present a case of a 57-year-old male patient presenting for colonoscopic evaluation following a positive screening with a fecal occult blood test. The patient was found to have a 10 mm pedunculated polyp at the level of the recto-sigmoid junction, which was removed by hot snare polypectomy and was found to have spindle cells that were positive for desmin, consistent with the rare diagnosis of recto-sigmoid leiomyoma. That being said, this case evokes a rare entity that endoscopists should keep in mind when approaching a recto-colonic polyp., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

This study evaluates the H2AX/γ-H2AX expression in soft tissue sarcomas (STS), its implications for biological behavior and immune environment, and its potential as a prognostic biomarker. RNA-Seq data from 237 STS were obtained from The Cancer Genome Atlas project. Patients were stratified by H2AX mRNA expression using a survival-associated cutoff. Differentially expressed genes and pathways as well as immune signatures between H2AX high - and H2AX low tumors were identified with DESeq2 analysis, gene set enrichment analyses (GSEA), Enrichr pathway analysis and CIBERSORTx. Tissue microarrays of a different cohort of 291 STS were generated for immunohistochemical staining to assess γ-H2AX protein expression, followed by statistical evaluation. High H2AX mRNA expression was associated with shorter overall survival (OS) in STS (p = 0.02), particularly in leiomyosarcomas (LMS) (p < 0.001), and was a negative prognostic factor in LMS (HR 11.15, p < 0.001). H2AX high LMS tumors showed upregulation of cell cycle-related pathways, while H2AX low LMS exhibited increased inflammatory activity, including elevated M1 macrophage signatures and resting mast cell signatures (both p < 0.001). High γ-H2AX protein levels were an independent negative prognostic factor in the total LMS cohort (HR 12.12, p = 0.025) and in the subgroup of non-uterine LMS (HR 153.80, p = 0.013). Consistent with CIBERSORTx analysis, γ-H2AX low LMS showed higher mast cell infiltration than γ-H2AX high LMS (p = 0.038). In conclusion, H2AX mRNA and γ-H2AX protein expression are associated with distinct biological behavior, differences in the immune cell environment, and might serve as useful prognostic biomarkers in LMS., (© 2024 The Author(s). International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Sigmoid leiomyosarcoma is a rare type of cancer that originates from smooth muscles of the sigmoid colon. This case report details a 50-year-old female found to have this condition, presenting to the emergency department with intussusception. A CT scan revealed two intraluminal masses, with the largest 10 cm from the anal verge. A colonoscopy and biopsy revealed a large obstructing mass with atypical spindle-shaped cells. This case shows a rare type of cancer presenting in a rare complication and highlights the approach done in the multi-disciplinary tumor board meeting involving gastroenterologists, radiologists, surgeons, and oncologists, leading to prompt diagnosis and surgical resection., Competing Interests: All authors have no potential conflict of interest relevant to this article., (Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2024.)

Introduction: Primary leiomyosarcoma affecting the bone is an uncommon destructive tumor that primarily affects the elderly. If leiomyosarcoma is discovered in bone, it should be explored as a possible metastasis from a distant tumor. Surgical excision with broad margins is used as therapy., Case Report: A 50-year-old man reported to outpatient department with a 5-month chief complaint of a painful mass involving his left carpus that had grown. No history of trauma was present. Examination demonstrated a painful restriction of wrist motions, and ill-defined, immobile, tender hard mass measuring 5 × 3 cm along the ulnar border of the left wrist. All necessary investigations were done and were diagnosed as primary leiomyosarcoma of the distal ulna. Patient was managed surgically with resection with wide en bloc resection with a margin of at least 2 mm around the tumor and radioulnar stabilization with the help of 1 K-wire (2.5 mm). Post-operative period was uneventful., Conclusion: Osseous leiomyosarcoma is an uncommon tumor, it should be evaluated in the diagnosis of any solely lytic aggressive osseous lesion in a middle age, Furthermore, the existence of extraosseous main lesions should be ruled out for its diagnosis. At present, surgery is the most effective therapy option., Competing Interests: Conflict of Interest: Nil, (Copyright: © Indian Orthopaedic Research Group.)

Primary inferior vena cava(IVC) leiomyosarcoma (LMS) is an extremely rare retroperitoneal mesenchymal tumour with < 500 cases reported in literature so far. Patients always present with nonspecific complaints, hence delaying the actual diagnosis, and they are associated with poor oncological outcomes. Differentiating primary leiomyosarcoma from other pathologies affecting IVC-like bland thrombus, retroperitoneal liposarcomas and other tumours infiltrating IVC secondarily may be difficult at times. We present here our experience with five cases of IVC leiomyosarcoma, who presented to us during a 6-year period., Competing Interests: Conflict of InterestThe authors declare no competing interests., (© The Author(s), under exclusive licence to Indian Association of Surgical Oncology 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Uterine leiomyomas are the most common benign, monoclonal, gynaecological tumors in a woman's uterus, while leiomyosarcoma is a rare but aggressive condition caused by the malignant transformation of the myometrium. To overcome the common obstacles related to the methods usually used to study these pathologies, we aimed to devise three-dimensional models of myometrium, uterine leiomyoma and leiomyosarcoma cell lines, using two different types of biocompatible scaffolds. Specifically, we exploited the agarose gel matrix in common 6-well plates and the alginate matrix using Bioprinting INKREDIBLE + (CELLINK), a pneumatic extruded base equipped with a system with double printheads, and a UV printer LED curing system. Both methods allowed the development of 3D spheroids of all three cell types, that were also suitable for morphological investigations. We showed that all cell types embedded in both agarose and alginate formed spheroids in their growth medium. The spheroids successfully proliferated and self-organized into complex structures, developing a sustainable system that emulated the condition of the tissues through the accumulation of extracellular matrix. These models could be useful for a better understanding of pathophysiology, etiopathogenesis, and testing new methods or molecules from a preventive and therapeutic point of view., Competing Interests: Declarations. Conflict of Interest: On behalf of all authors, the corresponding author states that there is no conflict of interest., (© 2024. The Author(s), under exclusive licence to Society for Reproductive Investigation.)

Introduction and Importance: Scrotal Leiomyoma is a benign and rare mesenchymal tumor that grows slowly. It was reported for the first time in 1858 by Forster. The Patients with Scrotal Leiomyoma is an asymptomatic painless mass., Case Presentation: The patient was a 45-year-old man, who presented with a painless scrotal mass to our hospital. On physical examination, he had a single, firm, and mobile mass about 2-3 cm in diameter within the right scrotum without tenderness. The left side was normal. Pre-operative scrotal sonography, a 2.5 cm hypoechoic extra testicular mass with sharp borders was reported. Intraoperatively The mass was excised totally and the testis was preserved. On microscopic examination revealed interlacing bundles of spindle-shaped cells with no mitotic figures or nuclear pleomorphism. On immunohistochemical analysis, the spindle cells were positive for SMA and negative for S100, and leiomyoma was confirmed., Clinical Discussion: For diagnosing leiomyoma, scrotal ultrasound is a first-line and noninvasive method that is confirmed by histologic examination. In histology, smooth muscle bundles are seen. Immunohistochemistry studies can confirm the diagnosis by identifying specific markers like vimentin, desmin, and smooth muscle actin. It is important to differentiate leiomyoma from a malignant type known as leiomyosarcoma., Conclusion: We report a rare case presentation of a 45-year-old man with scrotal leiomyoma that is confirmed by histology. It is important to differentiate leiomyoma from malignant course., Competing Interests: Conflict of interest statement The authors declare no conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Competing Interests: Conflicts of Interest There are no conflicts of interest to disclose.

Renal leiomyosarcoma metastasis to the pancreas is exceptionally rare. Here, we present a case of metastatic recurrence in the pancreas seven years after renal leiomyosarcoma resection. A 73-year-old female with a history of renal leiomyosarcoma surgery seven years prior presented with a well-defined 40 × 30 mm pancreatic tail tumor detected by a computed tomography (CT) scan. The tumor exhibited hypo-enhancement in the arterial phase and a progressive enhancement pattern toward the equilibrium phase, similar to pancreatic cancer. Endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) revealed bundles of spindle cells that matched those in the previously resected renal sample. Immunohistochemistry showed positive staining for desmin, confirming the diagnosis of pancreatic metastasis from renal leiomyosarcoma. The patient underwent a distal pancreatectomy to remove the metastatic lesion. The extended interval of seven years before the detection of metastasis underscores the challenges in monitoring and diagnosing metastatic patterns of renal leiomyosarcoma. EUS-FNB can assist in distinguishing metastatic pancreatic leiomyosarcoma from primary pancreatic cancer, thus influencing treatment decisions., (© 2024. Japanese Society of Gastroenterology.)

Purpose: Machine learning (ML) models have been used to predict cancer survival in several sarcoma subtypes. However, none have investigated extremity leiomyosarcoma (LMS). ML is a powerful tool that has the potential to better prognosticate extremity LMS., Methods: The Surveillance, Epidemiology, and End Results (SEER) database was queried for cases of histologic extremity LMS (n = 634). Patient, tumor, and treatment characteristics were recorded, and ML models were developed to predict 1-, 3-, and 5-year survival. The best performing ML model was externally validated using an institutional cohort of extremity LMS patients (n = 46)., Results: All ML models performed best at the 1-year time point and worst at the 5-year time point. On internal validation within the SEER cohort, the best models had c-statistics of 0.75-0.76 at the 5-year time point. The Random Forest (RF) model was the best performing model and used for external validation. This model also performed best at 1-year and worst at 5-year on external validation with c-statistics of 0.90 and 0.87, respectively. The RF model was well calibrated on external validation. This model has been made publicly available at https://rachar.shinyapps.io/lms&#95;app/ CONCLUSIONS: ML models had excellent performance for survival prediction of extremity LMS. Future studies incorporating a larger institutional cohort may be needed to further validate the ML model for LMS prognostication., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Purpose: Leiomyosarcoma (LMS) is an aggressive mesenchymal malignant tumor with poor therapeutic options, but the molecular mechanisms underlying LMS remain largely unknown. Increasing evidence indicates that transient receptor potential vanilloid 4 (TRPV4) levels are closely related to the advancement of various malignant tumors through diverse molecular mechanisms. However, the roles and regulatory mechanisms of TRPV4 in LMS progression remain unclear., Methods: Immunohistochemistry, Western blot, and immunofluorescence were used to investigate the relationship between TRPV4 expression and LMS. Survival analysis was conducted to evaluate the association between TRPV4 levels and prognosis in LMS patients. Intracellular Ca 2+ measurement, colony formation, CCK-8, wound healing and Transwell assays and peritoneal metastasis mouse model were used to verify the effect of TRPV4 activity and expression on LMS proliferation and metastasis. RNA-seq and proteomics were performed to explore the underlying mechanism., Results: TRPV4 was upregulated in LMS tissues and cells and served as a novel prognostic factor. Moreover, TRPV4 overexpression enhanced cell proliferation, cell migration and invasion of LMS cells in vitro, as well as promoted tumor metastasis in vivo, which could be blocked by HC067047 intervention or TRPV4 knockdown. Combined RNA-seq and proteomics analysis of KEGG pathway indicated that ECM receptor interaction was obviously activated. Extracellular matrix protein 1 (ECM1) was identified as downstream gene of TRPV4. Mechanistically, TRPV4 overexpression increased ECM1 level and activated the FAK/PI3K/AKT/GSK3β pathway, which could be reversed by TRPV4 knockdown or LY294002 treatment. Moreover, ECM1 overexpression enhanced the activation of FAK/PI3K/AKT/GSK3β pathway. And simultaneous overexpression of TRPV4 and ECM1 synergistically activated this pathway., Conclusion: Our findings provide a novel mechanism by which TRPV4 directly activates Ca 2+ /FAK/PI3K/AKT/GSK3β pathway and further indirectly enhances the FAK/PI3K/AKT/GSK3β pathway through the promotion and secretion of ECM1 to promote LMS malignant progression. Targeting the TRPV4/FAK axis might be a promising potential strategy for prognosis and treatment of LMS., Competing Interests: Declarations. Ethical approval: The study protocol received approval from the Investigation Ethical Committee of Zhongshan Hospital of Fudan University. Informed consent: Informed consent was obtained from all participants or their legal guardians. Animal studies: The study was approved by the Animal Care Ethical Committee of Zhongshan Hospital, Fudan University and carried out according to the guidelines outlined in the Care and Use of Laboratory Animals by the US National Academy of Science. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

A ​small intestinal leiomyosarcoma (LMS) is a rare malignancy that accounts for a small proportion of small bowel tumors and is often difficult to diagnose early due to its variable symptoms. This case report describes a 75-year-old female patient, previously suspected to have a gastrointestinal stromal tumor (GIST), presenting to the ED with abdominal pain and elevated inflammatory markers. A CT scan was performed which revealed a perforated mass in the distal ileum with surrounding collection. Consequently, she underwent laparoscopic washout, bowel resection, and primary anastomosis. Postoperatively, she developed paralytic ileus, which resolved with conservative management, and she was eventually discharged postoperative day 14. Histological and immunohistochemical analysis confirmed the diagnosis of an LMS, showing smooth muscle actin positivity, strong caldesmon labeling, and focal desmin expression with negative c-kit and S-100. The diagnosis of LMSs can be difficult, often misdiagnosed as GISTs due to overlapping features. Imaging studies, including CT scans, are valuable but not definitive. This case highlights the necessity of recognizing primary small intestinal LMSs as a potential cause of bowel perforation. Due to the rarity of LMSs and their potential for misclassification, accurate diagnosis and multidisciplinary management are crucial. Given the high risk of recurrence and metastasis, particularly due to perforation, long-term follow-up is also recommended., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Busada et al.)

Primary hepatic leiomyosarcoma (PHLS) is an extremely rare malignant tumor, which is often elusive in early diagnosis due to its rarity and nonspecific clinical and imaging presentations. Herein, we present a case of PHLS in a 66-year-old male and a review of the English literature from January 2000 to December 2023, focusing on the clinical and imaging characteristics of 30 patients with PHLS. The present patient was admitted to our hospital with complaints of abdominal distension, with history of hepatitis B. Tumor markers, including alpha-fetoprotein, carcinoembryonic antigen, and CA 19-9, were within the normal range. A hepatic tumor was incidentally identified during an abdominal ultrasound examination, further evaluated by contrast-enhanced CT and MR scans, which was preliminarily misdiagnosed as hepatocellular carcinoma. The tumor was surgically excised and definitively diagnosed as PHLS, characterized by two distinct areas with varying imaging features on contrast-enhanced CT and MR images. PHLS typically manifests as a well-defined, heterogeneously hypo- or iso-dense mass on CT, with a slightly prolonged T2 signal on MRI, and exhibits gradual enhancement during dynamic contrast-enhanced imaging. We advocate that the possibility of PHLS should be considered when the aforementioned imaging features are observed., Competing Interests: The authors declare the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Yan, Huang, Chen, Chen and Li.)

Background: Gemcitabine and docetaxel (GD) is a common chemotherapy regimen for metastatic soft tissue sarcoma (STS). The GeDDiS trial compared GD with doxorubicin in the first-line setting, using gemcitabine 675 mg/m 2 over a prolonged rate of 90 min-reporting a 20% response rate and 5.4-month median progression-free survival (PFS). We aimed to examine the real-world efficacy and toxicity of GD in our center, using a standardized dose of gemcitabine 900 mg/m 2 over 30 min on Days 1 and 8 and intravenous docetaxel 75 mg/m 2 over 60 min on Day 8 every 21 days., Methods: A retrospective analysis was conducted of patients with unresectable or metastatic STS receiving GD between July 2018 and October 2022. Data collected included patient and tumor characteristics, dose intensity, toxicity, response, PFS, and overall survival (OS)., Results: Thirty-eight patients were included. Median follow-up was 19 months (range 3-30). Line of treatment (n) was first line (10), second line (13), ≥ third line (15). The median number of cycles was 6. Response rate was 42%, including 5% with a complete response. At the time of data collection, 33 patients had disease progression and 24 patients had died. PFS (median, 6-month rate) was 4.5 months and 44%. OS (median, 12-month rate) was 15 months and 65%. Grade 3/4 toxicity included anemia (21%), neutropenia (5%), thrombocytopenia (5%), and febrile neutropenia (3%)., Conclusion: These data demonstrate the activity of gemcitabine using a standardized dose and rate with docetaxel comparable to other published data and favorable toxicity in a real-life patient population despite altered gemcitabine dosing and a heavily pretreated patient population., (© 2024 John Wiley & Sons Australia, Ltd.)

A 27-year-old female with a history of acute lymphoblastic leukemia in remission presented with chest pain, liver cirrhosis, and a thrombus in the hepatic vein on ultrasound. Further workup with computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass extending from the inferior vena cava to the right atrium, 3.4 x 3.4 x 7.0 cm in size. She underwent excision of the intracardiac mass with a full sternotomy and cardiopulmonary bypass successfully. Her postoperative course was uncomplicated. She was discharged home on postoperative day six. Subsequent histopathology confirmed high-grade leiomyosarcoma. Postoperative positron emission tomography CT showed no evidence of metastatic disease., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. IRB issued approval N/A. This research project dose not require review or approval by IRB. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Chong et al.)

Uterine carcinosarcoma (UCS) is a rare and highly aggressive gynecological malignancy characterized by poor prognosis. Due to its rarity, UCS remains relatively unexplored, and specific treatment guidelines are scarce. Despite standard treatments, including surgery, adjuvant chemotherapy, and radiotherapy, UCS has a high recurrence rate and poor overall prognosis. The aggressive nature of UCS is attributed to the metaplastic transformation of carcinomatous elements into sarcoma. This "biphasic" neoplasm features a mixture of epithelial and mesenchymal/tumor components, which partially share molecular signatures and exhibit a typical epithelial-to-mesenchymal transition (EMT) gene expression profile. Recent scientific advancements have highlighted the pivotal role of EMT in UCS progression and mortality. This review covers the epidemiology of UCS, theories regarding its origin, and the current state of clinical trials with more emphasis on the role of EMT drivers in UCS progression and scope of targeting these molecules. By shedding light on the molecular mechanisms supporting UCS, particularly emphasizing the importance of EMT, we aim to provide a more comprehensive understanding of the disease to support the development of more effective therapeutic strategies., (© 2024 Federation of American Societies for Experimental Biology.)

Dedifferentiated liposarcoma is a malignant lipomatous tumor that rarely occurs in the gastrointestinal tract, including the ileocecal region. In this case, computed tomography and magnetic resonance imaging showed no fatty mass located in the mesenteric or submucosal lesion, and positron emission tomography-computed tomography showed a high maximum standardized uptake value, collectively indicating the gastrointestinal stroma tumor and lymphoma. The pathological findings resemble leiomyosarcoma; the immunohistochemistry findings including mouse double minute 2 homolog and cyclin D-dependent kinase-4 and amplification of mouse double minute 2 homolog in fluorescence in situ hybridization just favored the diagnosis of dedifferentiated liposarcoma with leiomyosarcoma phenotype and not leiomyosarcoma. Recently, a new inhibitor for mouse double minute 2 homolog and cyclin D-dependent kinase-4 has been developed, and clinical trials for dedifferentiated liposarcoma are currently ongoing. This could change the treatment strategy drastically compared with other soft tissue sarcomas. Hence, a correct diagnosis of dedifferentiated liposarcoma is required., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Kawasaki, Tashima, Onohara, Hirano, Yamato, Shirotake, Torigoe, Yazawa, Hirasaki, Wako, Fujimaki and Ichikawa.)

Introduction and Importance: While the treatment approach for sarcomas seems straightforward and well-defined, we often encounter several diagnostic or therapeutic challenges in clinical practice. This article presents a case of retroperitoneal leiomyosarcoma in a patient with chronic kidney disease, highlighting the complexity of managing such cases., Case Report: A 63-year-old woman with a history of chronic kidney disease presented with a progressively enlarging mass in her right flank. A CT scan revealed a retroperitoneal mass affecting the muscular layer of the right lumbar ureter, without invading the kidney. Percutaneous biopsies concluded to a smooth muscle tumor with positive anti-caldesmone and anti-desmin antibodies. A 99 m Tc DMSA renal revealed significant impairment of the right kidney function while the left kidney function remained normal. The surgical procedure involved removing the mass, as well as the right kidney and ureter. Histopathological examination confirmed the diagnosis of leiomyosarcoma. No locoregional recurrence or metastases were noted after a 13-month follow-up., Discussion: Leiomyosarcomas are malignant tumors that can affect various organs, with retroperitoneum being the second most common location. A CT scan and abdominal MRI are standard imaging technique to evaluate this disease. Percutaneous biopsy and pathology are performed to confirm the nature of the tumor, especially when neoadjuvant treatment is necessary for metastatic tumors. Compartmental resection with clear margins is the only potential curative treatment. Even after R0 resection the risk of recurrence varies between 20 and 75 %., Conclusion: Managing retroperitoneal leiomyosarcoma, especially in patients with comorbidities like chronic kidney disease, necessitates a multidisciplinary approach., Competing Interests: Declaration of competing interest All authors declare they have no conflict of interest., (Copyright © 2024. Published by Elsevier Ltd.)

Radiation-induced sarcomas (RIS) are iatrogenic malignancies that arise following high-dose radiotherapy, posing a significant clinical challenge due to their poor prognosis and resistance to conventional treatments. The incidence of RIS is increasing with advancements in radiotherapy techniques. This report presents a case of a 71-year-old male diagnosed with stage III rectal adenocarcinoma treated with neoadjuvant chemoradiotherapy and curative surgery. Three years postoperatively, he developed a low-grade radiation-induced leiomyosarcoma in the perianal region. Histopathological examination confirmed a spindle cell neoplasm with notable immunohistochemical markers. RIS often presents as aggressive high-grade tumors resistant to radiotherapy and chemotherapy, necessitating surgical resection as the primary treatment. This case underscores the importance of long-term surveillance post-radiotherapy and highlights the need for innovative therapeutic strategies, including immunotherapy. Despite being rare, RIS poses a significant risk following cancer treatment, making early detection through vigilant monitoring and advancements in therapeutic approaches crucial for improving patient outcomes., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Chen, Cai, Li, Ren, Xu, Pang and Dai.)

Leiomyosarcoma is a soft-tissue sarcoma that accounts for less than 1% of all malignant tumors. Furthermore, leiomyosarcoma accounts for 6% of all soft tissue sarcomas, and leiomyosarcoma of azygos vein origin is extremely rare. In this report, we describe a case of leiomyosarcoma derived from an azygos vein that was completely resected. A 78-year-old male patient was incidentally found to have a mediastinal mass during a computed tomography (CT) scan performed for the evaluation of prostate cancer. Bronchoscopic needle biopsy revealed the mass to be a leiomyosarcoma. Preoperative contrast-enhanced CT demonstrated a filling defect in the azygos vein, suggesting the tumor's origin from this structure. Video-assisted thoracoscopic surgery (VATS) was performed to resect the mediastinal tumor. The tumor was found to have minimal adhesions to surrounding tissues and no evidence of local invasion, although findings suggested it originated from the azygos vein. Consequently, en bloc resection of the azygos vein was performed. Postoperative histopathological examination confirmed the diagnosis of leiomyosarcoma originating from the azygos vein, and complete resection was performed., (© 2024 The Author(s). Thoracic Cancer published by John Wiley & Sons Australia, Ltd.)

A mass was removed surgically from the right orbit of a 1-d-old Holstein calf. Grossly, the mass filled the rostral part of an enlarged orbit and compressed the globe toward the caudal pole of the orbit. The brown, 6-cm tumor had central yellow and brown areas, and a smooth, glistening cut surface. Microscopically, the neoplasm was highly cellular and composed of spindle cells arranged in irregular, broad, interlacing streams and bundles, forming a herringbone pattern and supported by a sparse collagenous matrix. Neoplastic cells infiltrated surrounding soft tissues and compressed the globe. The neoplastic cells had positive immunolabeling for α-smooth muscle actin, desmin, and vimentin, and negative immunolabeling for factor VIII, myoglobin, cytokeratin, and skeletal muscle actin. Histopathology and immunohistochemistry results confirmed a diagnosis of leiomyosarcoma. To our knowledge, congenital periocular leiomyosarcoma has not been reported in cattle previously. This rare tumor could be included as a differential diagnosis in newborn calves with periocular masses., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest associated to the research, authorship, and/or publication of this article.

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare and aggressive mesenchymal tumor, with less than 400 reported cases to date. Complete resection of the tumor with clear margins is the only proven curative treatment, providing survival benefits. Nonetheless, leiomyosarcomas in the middle segment or those extending up to it within the inferior vena cava (IVC) frequently necessitate renal reimplantation or nephrectomy, with rates varying between 56% and 75%. In this case report, we present a 65-year-old female with lower segment IVC leiomyosarcoma with middle segment extension, successfully resected and reconstructed while avoiding associated renal reimplantation or nephrectomy morbidity., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Smooth Muscle Tumor of Uncertain Malignant Potential (STUMP) is a poorly studied neoplasm that does not fulfill the definition of either leiomyoma or leiomyosarcoma. STUMP symptoms are indistinguishable from those of benign lesions; it has no specific biochemical markers or ultrasound presentations. The management of this type of tumor is particularly challenging due to significant heterogeneity in its behavior and the lack of clear guidelines; moreover, the lesion may recur after excision. Case Report : We report on a case of a 42-year-old patient diagnosed with a STUMP. The preliminary diagnosis was a submucous leiomyoma, which was removed hysteroscopically due to menorrhagia resulting in anemia. The histopathological examination of the resected myoma pointed to the diagnosis of STUMP. The hysterectomy was performed as the patient had completed her reproductive plans. There were no complications. The patient is currently recurrence-free after a 9-month follow-up. Discussion and Conclusions : The care of a patient diagnosed with STUMP requires a personalized approach and the cooperation of various medical disciplines, including molecular diagnostics, imaging techniques, and minimally invasive surgery. Management of STUMP must consider the patient's plans for childbearing. All cases of tumors with "uncertain malignant potential" are a challenge in the context of patient-physician communication.

Synovial sarcoma is a rare mesenchymal tumor, and its occurrence as a primary renal tumor is exceedingly rare. We are presenting a case of renal synovial sarcoma with lung involvement in a 47-year-old female patient who initially presented with typical renal symptoms, including blood in urine and left flank pain. Imaging revealed a large renal mass with extension into the renal vein and metastatic nodules in the lungs. Histopathological examination and genetic analysis confirmed monophasic synovial sarcoma with SYT-SSX2 translocation. Treatment included radical nephrectomy followed by systemic chemotherapy with doxorubicin and ifosfamide. Despite the initial response, the disease progressed, leading to fatal complications. This case highlights the diagnostic challenges, limited treatment options, and poor prognosis associated with primary renal synovial sarcoma., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Abbas et al.)

A 13-year-old Maltese dog with an abdominal mass underwent 18F-FDG PET/computed tomography (CT) for tumor localization and metastatic evaluation. PET/CT scans revealed a gastric mass near the esophagogastric junction and demonstrated mean and maximum standardized uptake values (SUVs) of 4.596 and 6.234, respectively, for the abdominal mass. Subsequent surgery incorporated ICG for NIR fluorescence-guided imaging, aiding in precise tumor localization and margin assessment. The excised mass was identified as a low-grade leiomyosarcoma on histopathology. The dog underwent PET/CT imaging six months postoperatively following the excision of the mass, which confirmed the absence of recurrence or residual lesions during follow-up. NIR fluorescence imaging using ICG demonstrated efficacy in real-time tumor visualization and margin assessment, a technique not previously reported in veterinary literature. The PET/CT findings complemented the diagnosis and provided valuable insights into metastasis. The absence of recurrence or complications in postoperative follow-up underscores the potential of these imaging modalities in enhancing surgical precision and improving prognosis in canine gastric tumors.

Background: We hypothesized that abdominal venous leiomyosarcoma (AV-LMS) disproportionately originates in veins of the sex-hormone drainage pathway (SHDP). Our purpose was to classify the anatomical origin of AV-LMS in a large cohort using imaging and explore prognostic implications., Methods: A retrospective review of imaging of all patients presenting with abdominal non-uterine LMS at a single tertiary oncology center was performed. Inclusion criteria were a biopsy-proven LMS of non-uterine abdominal/pelvic origin with pretreatment enhanced computed tomography (CT)/magnetic resonance imaging (MRI). Patients with uterine LMS or prior radiation were excluded. LMS site of origin was assigned by one expert radiologist and indeterminate sites were reviewed with a second external expert radiologist. Locations of inferior vena cava (IVC) tumors were subclassified based on a modification of prior literature. SHDP was defined as originating from ovarian/testicular vein, distal left renal vein, adrenal vein or mid-IVC (IIA)., Results: One hundred fifty-five (155) patients were included (92/152 (61%) female) with distant metastases found at presentation in 23/155 (14.8%). Most common organs of origins were veins (84/152, 55.3%), gastrointestinal (24, 15.8%), genital (11, 7.2%) and paratesticular/spermatic cord (11, 7.2%). For venous LMS, the adrenal (both sexes), mid-IVC (IVC IIA, females) and ovarian veins had the highest relative predilection for abdominal non-uterine LMS. Eighty-four (84/152, 55.3%) of tumors were SHDP. On multivariable analysis, both size and SHDP were significant predictors of distant metastases at presentation (P = 0.01), while sex, age, organ system/site and grade were not., Conclusions: For both sexes, tumors arising from SHDP constitute the majority of AV-LMS and may impart a significantly lower risk of metastatic disease at presentation. Among veins, the adrenal veins had the highest predilection for LMS., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright 2024, Tarique et al.)

Sarcomas of thyroid glands represent a distinctive subset of rare and perplexing anomaly that present a challenges in the field of thyroid pathology. Thyroid sarcomas, primary or secondary, are exceptionally rare with only a handful of case reports documented so far. The challenges lie in the fact that certain primary thyroid malignancies of epithelial origin may exhibit spindle cell morphology, making them difficult to differentiate from thyroid sarcomas. Despite the morphological similarities, discerning between these entities is crucial due to their distinct treatment and management implications. This report documents a series of unusual types of sarcoma in the thyroid gland. The aim is to explore the peculiarities of these lesions, the diagnostic challenges faced as well to study the potential implications for both clinicians and patients., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Primary ovarian leiomyosarcoma (LMS) is an extremely rare and aggressive tumor that originates in the smooth muscle tissue of the ovary. Typically affecting older women, it is diagnosed at an advanced stage and is associated with a poor prognosis, with most patients succumbing within the first year. The effectiveness of adjuvant chemotherapy and radiotherapy remains unclear due to the rarity of reported cases. In this case report, a 66-year-old woman presented with lower abdominal pain persisting for four months, accompanied by weight loss and appetite reduction. Imaging revealed a large, heterogeneous solid lesion in the left adnexa, suggesting a malignant ovarian neoplasm. Surgery was performed, and histopathological analysis confirmed the presence of a high-grade spindle cell sarcoma, consistent with ovarian LMS. Immunohistochemistry showed positive smooth muscle actin (SMA) and negative S100 and Myo D1, supporting the diagnosis. The patient completed two cycles of chemotherapy and was then lost to follow-up. This case highlights the diagnostic challenges and limited treatment options associated with primary ovarian LMS. Given the scarcity of cases, there is no established standard therapy, and the prognosis remains poor. Further research is needed to develop effective therapeutic strategies for this aggressive malignancy., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Kani et al.)

Leiomyosarcoma is an aggressive soft tissue cancer frequently seen in the female gastrointestinal and genital tracts due to the preponderance of smooth muscle. We report the case of a patient with leiomyosarcoma next to the iliac crest, discussing this rare location with data from the literature., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Tebbaa El Hassali et al.)

Background: Leiomyosarcoma (LMS) is a rare neoplasm that arises from tissues of embryonic mesodermal origin. Primary tissues of origin can include smooth muscle cells of the abdominopelvic viscera, blood vessels, or arrector pili muscles. LMS is known to metastasize to the lungs, with few reported cases of spread to the central nervous system., Observations: A 66-year-old male with cutaneous LMS of the left forearm with metastases to the lungs and kidney that had been treated with chemoradiation presented with worsening headaches. Magnetic resonance imaging revealed a sellar lesion. An endocrine workup was unremarkable. Imaging over 6 months revealed rapid interval growth. Positron emission tomography demonstrated moderate uptake. Given the rapid growth, the patient was offered an endoscopic endonasal approach for resection. Pathology confirmed LMS., Lessons: To the authors' knowledge, this is the first documented case of LMS metastasis to the sella. Pituitary carcinoma or metastases to the sellar region should be in the differential among patients with sellar region tumors with a rapid growth rate, bony erosion, or findings of lesions in the upper cervical lymph nodes or soft tissue. Tumors that show significant interval growth should raise suspicion for nonadenomatous lesions, and surgical intervention should be considered even in the absence of endocrinological dysfunction or cranial neuropathies. https://thejns.org/doi/10.3171/CASE2435.

Uterine lipoleiomyosarcomas (L-LMS) are rare malignant tumors with only few cases descripted in literature. As well, liposarcomas (LPS) arising from uterine corpus are extremely rare since fat tissue is commonly poor or absent in uterus. We report a case of L-LMS of the uterine corpus with an associated component of pleomorphic LPS. As for other female pelvic malignancies, these neoplasms are more common in postmenopausal women and clinical findings are nonspecific. Most frequent signs and symptoms are metrorrhagia and abdominal pain often associated with pelvic distension/mass. We describe diagnostic and therapeutic steps of our patient from acceptation to conclusive diagnosis. In this case, contrast-enhanced computed tomography (CECT) depicted pivotal diagnostic findings and showed a clear distinction between different tumor components. Therefore, our goal with this essay is to stress the role of CECT imaging for diagnosis of these rare neoplasms. We also perform a review of current literature about liposarcomas of the uterine corpus., (© 2024 Published by Elsevier Inc. on behalf of University of Washington.)

Primary ovarian sarcoma is a rare malignancy, with primary ovarian leiomyosarcoma being even rarer because of the lack of smooth muscle in the ovaries. We herein report a case of primary ovarian leiomyosarcoma in a woman in her late 50s who presented with a 6-month history of abdominal pain. Imaging revealed a pelvic mass. The patient underwent surgery and was diagnosed with ovarian leiomyosarcoma. One month postoperatively, she began gemcitabine and docetaxel chemotherapy and continued this treatment for 6 months. Eight months postoperatively, however, recurrence was detected in the pelvic cavity. This case is reported with the aim of raising awareness about this rare disease., Competing Interests: Declaration of conflicting interestThe authors declare that they have no conflict of interest regarding the publication of this research.

Uterine leiomyosarcoma (LMS) represents a rare yet highly aggressive tumor, comprising approximately 1% of uterine malignancies. First-line regimens involving doxorubicin or gemcitabine and docetaxel demonstrate modest response rates. Notably, the combination of doxorubicin plus trabectedin has emerged as a preferred first-line option following the LMS-04 study, showing superior progression-free survival compared to doxorubicin alone. Second-line therapy for recurrent LMS poses greater challenges, with single-agent treatments exhibiting limited efficacy. Herein, we present a case of a 65-year-old woman with stage 1B uterine leiomyosarcoma, previously treated with surgical resection and adjuvant gemcitabine/docetaxel, due to surgical morcellation. Despite initially achieving disease-free status, she experienced a first recurrence 5 years later, treated with surgery and radiation, and a second recurrence 4 years after, necessitating second-line therapy with doxorubicin and trabectedin. The patient exhibited a remarkable response to this regimen, achieving partial response after 6 cycles of doxorubicin and trabectedin chemotherapy. She maintained stable disease over 13 cycles of maintenance trabectedin and 6 months off treatment, for a total of 16 months of progression-free survival. This case underscores the potential efficacy of combination chemotherapy with doxorubicin and trabectedin as a second-line treatment option for recurrent uterine leiomyosarcoma., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors. Published by Elsevier Inc.)

Background: Leiomyosarcoma of the vena cava (LMS-VC) is a rare entity with poor oncological outcomes and a lack of histological staging prognostic factors., Methods: Outcomes of consecutive patients operated on LMS-VC between March 2003 and May 2022, in two specialized sarcoma centers were reported., Result: Forty-one patients were identified. Median size of LMS-VC was 9 cm with 68% of complete obstruction. After surgery, severe complication rate was 30%. No postoperative mortality was reported. Microscopic complete excision was obtained for 71% of patients, R1 for 27% and one patient presented an R2 resection. Grade 3 was found in 24%. After a median follow-up of 70 months, 3 years disease-free survival (DFS) and 5 years DFS were 34% and 17%, and 3 years overall survival (OS) and 5 years OS were 74% and 50%. Distant metastasis concerned 54% of recurrences, local 7% and local and distant 5%. Multivariate analysis showed that FNCLCC grade (p < 0.001) and perioperative chemotherapy (p = 0.026) were significant factors for DFS. In multivariate analysis, FNCLCC grade was a significant factor for OS (p = 0.004)., Discussion: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor., (© 2024 The Author(s). Journal of Surgical Oncology published by Wiley Periodicals LLC.)

BACKGROUND. Uterine sarcomas are rare; however, they display imaging features that overlap those of leiomyomas. The potential for undetected uterine sarcomas is clinically relevant because minimally invasive treatment of leiomyomas may lead to cancer dissemination. ADC values have shown potential for differentiating benign from malignant uterine masses. OBJECTIVE. The purpose of this study was to perform a systematic review of the diagnostic performance of ADC values in differentiating uterine sarcomas from leiomyomas. EVIDENCE ACQUISITION. We searched three electronic databases (the MEDLINE, Embase, and Cochrane databases) for studies distinguishing uterine sarcomas from leiomyomas using MRI, including ADC values, with pathologic tissue confirmation or imaging follow-up used as the reference standard. Data extraction and QUADAS-2 quality assessment were performed. Sensitivity and specificity were pooled using hierarchical models, including bivariate and hierarchical summary ROC models. Metaregression was used to assess the impact of various factors on heterogeneity. EVIDENCE SYNTHESIS. Twenty-one studies met the study inclusion criteria. Pooled sensitivity and specificity were 89% (95% CI, 82-94%) and 86% (95% CI, 78-92%), respectively. The area under the summary ROC curve was 0.94 (95% CI, 0.92-0.96). The context of the ADC interpretation (i.e., used as a stand-alone assessment vs integrated as part of multiparametric MRI [mpMRI]) was the only factor found to account significantly for heterogeneity ( p = .01). Higher specificity (95% [95% CI, 92-99%] vs 82% [95% CI, 75-89%]) and similar sensitivity (94% [95% CI, 89-99%] vs 88% [95% CI, 82-93%]) were observed when ADC was evaluated among mpMRI features rather than as a stand-alone ADC assessment. ADC cutoff values ranged from 0.87 to 1.29 × 10 -3 mm 2 /s but were not associated with statistically different performance ( p = .37). Pooled mean ADC values for sarcomas and leiomyomas were 0.904 × 10 -3 mm 2 /s and 1.287 × 10 -3 mm 2 /s, respectively. CONCLUSION. As part of mpMRI evaluation of uterine masses, a mass ADC value of less than 0.904 × 10 -3 mm 2 /s may be a useful test-positive threshold for uterine sarcoma, consistent with the findings of a prior expert consensus statement. Institutional protocols may influence locally selected ADC values. CLINICAL IMPACT. Using ADC as part of mpMRI assessment improves detection of uterine sarcoma, which could influence candidate selection for minimally invasive treatments. TRIAL REGISTRATION. Prospective Register of Systematic Reviews CRD42024499383.

Transposable elements (TEs) are of interest as immunomodulators for cancer therapies. TEs can fold into dsRNAs that trigger the interferon response. Here, we investigated the effect of different HDAC inhibitors (HDACIs) on the expression of TEs in leiomyosarcoma cells. Our data show that endogenous retroviruses (ERVs), especially ERV1 elements, are upregulated after treatment with HDAC1/2/3-specific inhibitors. Surprisingly, the interferon response was not activated. We observed an increase in A-to-I editing of upregulated ERV1. This could have an impact on the stability of dsRNAs and the activation of the interferon response. We also found that H3K27ac levels are increased in the LTR12 subfamilies, which could be regulatory elements controlling the expression of proapoptotic genes such as TNFRSF10B. In summary, we provide a detailed characterization of TEs modulation in response to HDACIs and suggest the use of HDACIs in combination with ADAR inhibitors to induce cell death and support immunotherapy in cancer., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Leiomyosarcoma (LMS) is a malignancy with smooth muscle differentiation. Metastatic LMS is associated with poor prognosis and limited efficacy of systemic treatment. Novel treatment modalities are desperately needed for this entity., Case Presentation: We report the first use of pembrolizumab plus pharmacologic ascorbate in 3 patients with metastatic LMS. All cases resulted in persistent objective responses and disease control significantly better than has been reported with chemotherapy or other immunotherapeutic approaches. Three patients with metastatic LMS, one each of uterine, vascular, and soft tissue origin, were treated with pembrolizumab plus pharmacologic ascorbate. The patient with uterine LMS received combination therapy at presentation and had persistent response for 12 months, which is ongoing. The patient with metastatic LMS of the inferior vena cava received combination therapy at presentation and had persistent response for 12 months, at which time new metastases were found. The patient with soft tissue LMS had disease progression on pembrolizumab monotherapy prior to the addition of ascorbate, after which she had a 17-month response, which is ongoing. No side effects attributed to treatment were reported., Conclusion: Pembrolizumab plus pharmacologic ascorbate is a novel immunotherapeutic approach and warrants further study in LMS., Competing Interests: The authors have no conflicts of interest to declare., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Key Clinical Message: LMS of IVC needs a multidisciplinary approach. Surgical excision with free margin is the cornerstone of management. Upon case-by-case selection, adjuvant chemotherapy may play a role in better oncologic outcome., Abstract: Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare form of mesenchymal origin malignancy with less than 400 cases reported to date. Surgery is the mainstay of management but it requires vast experience in vascular and visceral surgery to attain a free tumor margin. Subsequent adjuvant treatment with chemotherapy and radiation remains as an area of gray zone. We report the case of a 61-year-old man with an 8-month history of abdominal pain. Upon physical examination, an ill-defined mass over the right side of the lower abdomen and bilateral lower extremity edema were detected. Abdominal ultrasound with Doppler revealed a right-side retroperitoneal mass invading the IVC with extensive venous thrombosis for which anticoagulation was initiated. Computed Tomography of the abdomen revealed a huge heterogeneously enhancing mass involving the whole length of the infrarenal IVC obstructing the IVC lumen with collateral veins draining through the paralumbar veins and inferior epigastric veins bilaterally. With a top differential of primary IVC LMS, a midline longitudinal laparotomy was performed with an intraoperative finding of a tumor arising from the infra-renal IVC which was excised. Gore-Tex graft was used to reconstruct the IVC. There was an injury to the right common iliac artery and it was repaired by end-to-end anastomosis. Histopathology confirmed a high-grade LMS of the IVC and surgical margin status was unknown. He was given adjuvant Chemotherapy consisting of Doxorubicin and Dacarbazine. He has been on follow-up at the Oncology side with a good performance status., Competing Interests: None declared., (© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: In retroperitoneal leiomyosarcoma (RP LMS), the predominant issue is distant metastasis (DM). We sought to determine variables associated with this outcome and disease-specific death (DSD)., Methods: Data were retrospectively collected on patients with primary RP LMS treated at a high-volume center from 2002 to 2023. For inferior vena cava (IVC)-origin tumors, the extent of macroscopic vascular invasion was re-assessed on each resection specimen and correlated with preoperative cross-sectional imaging. Crude cumulative incidences were estimated for DM and DSD and univariable and multivariable models were performed., Results: Among 157 study patients, median tumor size was 11.0 cm and 96.2% of cases were intermediate or high grade. All patients underwent complete resection, 56.7% received chemotherapy (43.9% neoadjuvant) and 14.6% received radiation therapy. Only tumor size and grade and not site of tumor origin (e.g., IVC vs. other) were associated with DM and DSD (p < 0.05). Among 64 patients with IVC-origin tumors, a novel 3-tier classification was devised based on the level of intimal disruption, which was associated with both DM (p = 0.007) and DSD (0.002)., Conclusion: In primary RP LMS, only tumor size and grade are predictive of DM and DSD. In IVC-origin tumors, the extent of macroscopic vascular invasion is also strongly predictive of these outcomes., (© 2024 Wiley Periodicals LLC.)

Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. The imaging features are nonspecific and the diagnosis is often delayed until the tumor reaches a large size, which leads often to a dismal prognosis. We report a case of a 46-year-old male patient who was complaining about abdominal pain for 2 months. The imaging revealed the presence of a large mass in the liver with adrenal and liver metastasis. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. In this case report, we review the epidemiological, clinical, and paraclinical aspects of the disease, as well as the treatment modalities., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)