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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.

Despite sharing the same histologic classification, individual tumors in multi metastatic patients may present with different characteristics and varying sensitivities to anticancer therapies. In this study, we investigate the utility of radiomic biomarkers for prediction of lesion-specific treatment resistance in multi metastatic leiomyosarcoma patients. Using a dataset of n=202 lung metastases (LM) from n=80 patients with 1648 pre-treatment computed tomography (CT) radiomics features and LM progression determined from follow-up CT, we developed a radiomic model to predict the progression of each lesion. Repeat experiments assessed the relative predictive performance across LM volume groups. Lesion-specific radiomic models indicate up to a 4.5-fold increase in predictive capacity compared with a no-skill classifier, with an area under the precision-recall curve of 0.70 for the most precise model (FDR = 0.05). Precision varied by administered drug and LM volume. The effect of LM volume was controlled by removing radiomic features at a volume-correlation coefficient threshold of 0.20. Predicting lesion-specific responses using radiomic features represents a novel strategy by which to assess treatment response that acknowledges biological diversity within metastatic subclones, which could facilitate management strategies involving selective ablation of resistant clones in the setting of systemic therapy., 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 Elsevier Ltd. All rights reserved.)

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.)

Competing Interests: Conflict of interest Nothing to Disclose

Background: Leiomyosarcoma is a tumor that can develop in any organ that contains smooth muscles. Although leiomyosarcoma is common, its epididymal localization is quite rare., Case Presentation: A 79-year-old male Chinese Han patient presented with mild pain in the right groin and scrotum for 3 years concomitant with right scrotal swelling. Ultrasonography and magnetic resonance imaging of the scrotum showed a irregular and heterogeneous mass that was extratesticular. Right high orchiectomy was performed, and pathological examination of the resected specimen confirmed the diagnosis of leiomyosarcoma of the epididymis with surgical margins clear of tumor., Conclusion: Epididymal leiomyosarcoma is rare and difficult to diagnose preoperatively. The final diagnosis of epididymal leiomyosarcoma requires histologic examination. Resection must be extensive and complete. The effect of chemotherapy and radiation on the epididymal leiomyosarcoma remains unclear. Recurrence is common, so follow-up is necessary., (© 2024. The Author(s).)

Introduction: Primary vascular leiomyosarcomas are incredibly rare and have a poor prognosis. The purpose of this study was to analyze the surgical outcomes of patients with primary inferior vena cava (IVC) leiomyosarcoma., Methods: We performed a retrospective review of IVC leiomyosarcoma resections performed at a single tertiary care hospital from 2014 to 2023. A total of 13 cases were analyzed, including 10 women and 3 men. The presenting symptoms, tumor characteristics, operative management, postoperative complications, and survival rates were assessed for each patient., Results: The median patient age was 59 years (quartile [Q]1, 52 years; Q3, 68 years). The median tumor size was 7.0 cm (Q1, 6 cm; Q3, 12 cm). The median mitotic rate was 6 per 10 high-power fields (Q1, 2.5; Q3, 15.5). All 13 patients underwent grossly negative tumor resection, with 9 (69%) having microscopically negative margins (R0). No patient had lymph node involvement. The IVCs were managed with ligation in four patients for tumors already occluding the IVC and bovine pericardial patch angioplasty in seven patients or primary repair in two patients for patent IVCs. Concomitant right nephrectomy was performed in seven patients. Left renal vein ligation was performed in three patients, but no left nephrectomies were performed. Significant postoperative complications included one patient with lower extremity compartment syndrome, two patients with severe leg swelling, and one patient with arm swelling. The 30-day mortality rate was zero. Using the Kaplan-Meier product limit method, disease-specific survival was estimated to be 93%., Conclusions: Surgical resection is a feasible and effective oncologic treatment option for patients with IVC leiomyosarcoma. The IVC can be safely managed by ligation, primary repair, or patch angioplasty, depending on the prior patency of the IVC., Competing Interests: Disclosures None., (Published by Elsevier Inc.)

Objective: Uterine leiomyomas are the most common benign uterine tumors. They are difficult to distinguish from their malignant counterparts-smooth muscle tumors of unknown malignant potential (STUMP) and leiomyosarcoma. The purpose of this study is to propose and validate the diagnostic accuracy of the MRI-based Oman-Canada Scoring System of Myometrial Masses (OCSSMM) to differentiate uterine leiomyomas from STUMP/leiomyosarcomas., Methods: This is a retrospective study performed at two tertiary care centers. All patients with a pathology-proven uterine mass who underwent pre-operative pelvic MRI between January 2010 and January 2020 were included. Using a 1.5T MRI machine, sequences included were axial/coronal/sagittal T2 and T1 weighted imaging, axial diffusion weighted and apparent diffusion coefficient map, and axial or sagittal dynamic contrast-enhanced sequences. A scoring system was designed based on previously published worrisome MRI features for uterine leiomyosarcoma. Each feature was allocated a score from 0 to 2 according to the strength of association with malignancy. Subsequently, the MR images were blindly and independently reviewed by a fellowship-trained radiologist and a clinical fellow/senior resident. Each uterine mass was scored according to their imaging features. The scores were divided into five categories according to the sum of scores. Category III and above was considered positive for leiomyosarcoma/STUMP. Sensitivity, specificity, and positive and negative predictive values were calculated., Results: A total of 244 women were included (age range 20-74 years, mean 40). Of these, 218 patients had benign leiomyoma, 13 had STUMP, and 13 had leiomyosarcoma. The sensitivity and specificity of the scoring system were 92.3% and 64.7%, respectively. The negative predictive value was 98.6%. No leiomyosarcoma was missed using this scoring system. The presence of non-cystic T2 hyperintensity or diffusion restriction in a uterine mass were the most sensitive signs of a leiomyosarcoma/STUMP., Conclusion: The proposed multi-parametric MRI scoring system may be useful in differentiating benign uterine leiomyomas from leiomyosarcomas/STUMP., Competing Interests: Competing interests: None declared., (© IGCS and ESGO 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Leiomyosarcomas of large vessels are rare. It is a malignant tumour and the vast majority of these tumours arose from the inferior vena cava. We report a rare case of portal vein leiomyosarcoma, in a 56-years-old female patient admitted for chronic abdominal pain with abdominal mass in the right hypochondrium all evolving in a context of deterioration in general condition. We performed an abdominopelvic CT scan and then a MRI with contrast agent which objectified a large tissue mass containing areas of necrosis at the level of the duodeno-pancreatic compartment communicating at a large angle with the portal trunk over its entire length from the hepatic hilum to the spleno-mesenteric confluence responsible for a portal cavernoma downstream. This is associated with multiple secondary nodular tissue hepatic lesions. We also noted a respect for the fatty border separating the mass of the duodenal tract and the head of the pancreas, and also the absence of dilation of the pancreatic ducts making a pancreatic origin unlikely. To eliminate a duodenal origin of the mass we performed an upper digestive endoscopy which came back without any abnormality. An ultrasound-guided trans parietal biopsy of a secondary hepatic lesion was done and the pathological result of which speaks of a secondary hepatic lesion of a leiomyosarcoma., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

We present a novel case of a malignant transformation of an extremity soft tissue angioleiomyoma to leiomyosarcoma in a man in his late 70s who presented with a painful and increasing lump on his anterior tibia. Initial imaging and biopsy showed a benign angioleiomyoma which was excised for symptomatic reasons. An analysis of the resulting specimen revealed a 50×42×15 mm smooth muscle neoplasm consistent with angioleiomyoma with a 22×11 mm entirely intralesional nodular component in keeping with a grade 1 leiomyosarcoma. The malignant constituent of the lesion was entirely encased in benign angioleiomyoma negating the need for further surgery. Systemic staging investigation revealed no evidence of metastatic disease spread final staging as per the eighth edition of the American Joint Committee on Cancer (AJCC) Staging T1N0M0 R0 Stage 1 a., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Abstract: Leiomyosarcoma is an aggressive subtype of soft tissue sarcoma with frequent metastatic relapse after curative surgery. Herein, we report the 68 Ga-FAPI PET/CT findings in a 45-year-old woman with leiomyosarcoma. 68 Ga-FAPI-04 PET/CT detected increased FAPI uptake in abdominal leiomyosarcoma and liver metastases. The positive findings of 68 Ga-FAPI in this case highlighted that 68 Ga-FAPI may have potential value in the evaluation of leiomyosarcoma., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To assess the predictive ability of conventional MRI features and MRI texture features in differentiating uterine leiomyoma (LM) from uterine leiomyosarcoma (LMS)., Methods: This single-center, IRB-approved, HIPAA-compliant retrospective study included 108 patients (69 LM, 39 LMS) who had pathology, preoperative MRI, and clinical data available at our tertiary academic institution. Two radiologists independently evaluated 14 features on preoperative MRI. Texture features based on 3D segmentation were extracted from T2W-weighted MRI (T2WI) using commercially available texture software (TexRAD™, Feedback Medical Ltd., Great Britain). MRI conventional features, and clinical and MRI texture features were compared between LM and LMS groups. Dataset was randomly divided into training (86 cases) and testing (22 cases) cohorts (8:2 ratio); training cohort was further subdivided into training and validation sets using ten-fold cross-validation. Optimal radiomics model was selected out of 90 different machine learning pipelines and five models containing different combinations of MRI, clinical, and radiomics variables., Results: 12/14 MRI conventional features and 2/2 clinical features were significantly different between LM and LMS groups. MRI conventional features had moderate to excellent inter-reader agreement for all but two features. Models combining MRI conventional and clinical features (AUC 0.956) and MRI conventional, clinical, and radiomics features (AUC 0.989) had better performance compared to models containing MRI conventional features alone (AUC 0.846 and 0.890) or radiomics features alone (0.929)., Conclusion: While multiple MRI and clinical features differed between LM and LMS groups, the model combining MRI, clinical, and radiomic features had the best predictive ability but was only marginally better than a model utilizing conventional MRI and clinical data alone., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Combining conventional radiomics models with deep learning features can result in superior performance in predicting the prognosis of patients with tumors; however, this approach has never been evaluated for the prediction of metachronous distant metastasis (MDM) among patients with retroperitoneal leiomyosarcoma (RLS). Thus, the purpose of this study was to develop and validate a preoperative contrast-enhanced computed tomography (CECT)-based deep learning radiomics model for predicting the occurrence of MDM in patients with RLS undergoing complete surgical resection., Methods: A total of 179 patients who had undergone surgery for the treatment of histologically confirmed RLS were retrospectively recruited from two tertiary sarcoma centers. Semantic segmentation features derived from a convolutional neural network deep learning model as well as conventional hand-crafted radiomics features were extracted from preoperative three-phase CECT images to quantify the sarcoma phenotypes. A conventional radiomics signature (RS) and a deep learning radiomics signature (DLRS) that incorporated hand-crafted radiomics and deep learning features were developed to predict the risk of MDM. Additionally, a deep learning radiomics nomogram (DLRN) was established to evaluate the incremental prognostic significance of the DLRS in combination with clinico-radiological predictors., Results: The comparison of the area under the curve (AUC) values in the external validation set, as determined by the DeLong test, demonstrated that the integrated DLRN, DLRS, and RS models all exhibited superior predictive performance compared with that of the clinical model (AUC 0.786 [95% confidence interval 0.649-0.923] vs. 0.822 [0.692-0.952] vs. 0.733 [0.573-0.892] vs. 0.511 [0.359-0.662]; both P < 0.05). The decision curve analyses graphically indicated that utilizing the DLRN for risk stratification provided greater net benefits than those achieved using the DLRS, RS and clinical models. Good alignment with the calibration curve indicated that the DLRN also exhibited good performance., Conclusions: The novel CECT-based DLRN developed in this study demonstrated promising performance in the preoperative prediction of the risk of MDM following curative resection in patients with RLS. The DLRN, which outperformed the other three models, could provide valuable information for predicting surgical efficacy and tailoring individualized treatment plans in this patient population., Trial Registration: Not applicable., (© 2024. The Author(s).)

Purpose: To evaluate the magnetic resonance imaging (MRI) features that may help distinguish leiomyosarcomas from atypical leiomyomas (those presenting hyperintensity on T2-W images equal or superior to 50% compared to the myometrium)., Materials and Methods: The authors conducted a retrospective single-centre study that included a total of 57 women diagnosed with smooth muscle tumour of the uterus, who were evaluated with pelvic MRI, between January 2009 and March 2020. All cases had a histologically proven diagnosis (31 Atypical Leiomyomas-ALM; 26 Leiomyosarcomas-LMS). The MRI features evaluated in this study included: age at presentation, dimension, contours, intra-tumoral haemorrhagic areas, T2-WI heterogeneity, T2-WI dark areas, flow voids, cyst areas, necrosis, restriction on diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) values, signal intensity and heterogeneity after contrast administration in T1-WI, presence and location of unenhanced areas. The association between the MRI characteristics and the histological subtype was evaluated using Chi-Square and ANOVA tests., Results: The MRI parameters that showed a statistically significance correlation with malignant histology and thus most strongly associated with LMS were found to be: irregular contours (p < 0.001), intra-tumoral haemorrhagic areas (p = 0.028), T2-WI dark areas (p = 0.016), high signal intensity after contrast administration (p = 0.005), necrosis (p = 0.001), central location for unenhanced areas (p = 0.026), and ADC value lower than 0.88 × 10 -3 mm 2 /s (p = 0.002)., Conclusion: With our work, we demonstrate the presence of seven MRI features that are statistically significant in differentiating between LMS and ALM., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

An elderly man was referred to vascular surgery on incidental discovery of a left retroperitoneal mass ultimately found to be of left renal vein (LRV) origin. He initially presented with recurring lower back pain. CT of the abdomen/pelvis showed a 6.0×5.5 cm lobulated retroperitoneal mass anterior to the infrarenal aorta. Resection of the mass necessitated a multidisciplinary team consisting of medical oncologists, radiation oncologists, urologists and vascular surgeons. In efforts to obtain an R0 margin, en-bloc resection of the LRV from its confluence with the inferior vena cava (IVC) was necessary. A primary repair of the IVC was performed with preservation of the left kidney. The patient's back pain has since resolved after the surgery. A literature search found IVC reconstructions to be safe and effective in the removal of vascular leiomyosarcomas., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate a wide range of clinical and ultrasound characteristics of different uterine smooth muscle tumors to identify features capable of discriminating between these types., Methods: This was a retrospective, multicenter study that included 285 patients diagnosed with uterine smooth muscle tumors (50 leiomyosarcomas, 35 smooth muscle tumors of uncertain malignant potential, and 200 leiomyomas). The patients were divided into three groups based on the histological type of their tumors, and the groups were compared according to the variables collected., Results: Leiomyosarcomas were more common in older and post-menopausal women. Compared with leiomyomas, smooth muscle tumors of uncertain malignant potential and leiomyosarcomas had similar ultrasound features such as absence of normal myometrium, multilocular appearance, hyper-echogenicity in case of uniform echogenicity, absence of posterior shadows, echogenic areas, and hyperechoic rim. Leiomyosarcomas were larger, had more cystic areas, and were associated with a higher prevalence of pelvic free fluid. Smooth muscle tumors of uncertain malignant potential were characterized by a higher frequency of International Federation of Gynecology and Obstetrics (FIGO) type 6-7, the absence of internal shadows, and, in the case of cystic area, the presence of a regular internal wall. Tumor outline varied among the three histological types. A color score of 1 was typical of leiomyoma, a color score 2 was mainly observed in leiomyomas and smooth muscle tumors of uncertain malignant potential, a color score 3 did not differ among the tumors, while a color of score 4 was related to leiomyosarcomas. When combining color scores 3 and 4, leiomyosarcomas and smooth muscle tumors of uncertain malignant potential showed a high percentage of both circumferential and intra-lesional vascularization. A cooked appearance was not statistically different among the tumors., Conclusions: Based on our findings, specific ultrasonographic features as well as age and menopausal status are associated with different uterine smooth muscle tumor types. Integration of these data can help the pre-operative assessment of these lesions for proper management., Competing Interests: Competing interests: None declared., (© IGCS and ESGO 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

We present a 58-year-old female patient who underwent resection of a leiomyosarcoma arising from the right ovarian vein. She was referred to our hospital because of lower abdominal pain that had been present for 1 month prior to the visit. Ultrasound examination revealed a well-defined, smooth, lobulated, highly vascular mass(57 mm)adjacent to the distal portion of the duodenum. Contrast-enhanced computed tomography revealed the contrast enhancement mass (60 mm)located surround the right ovarian vein. In abdominal magnetic resonance image examination, the mass exhibited isointense signal on T1-weighted images, high signal on T2-weighted images, and restricted diffusion on diffusion- weighted images. We suspected primary leiomyosarcoma of the ovarian vein and proceeded with surgical intervention. On intraoperative findings, the mass was in contact with the duodenum and the inferior vena cava but dissection was easily achieved. We excised the mass together with the right ovarian vein. Pathological findings showed the mass was composed of proliferating spindle-shaped cells arranged in bundles. Some areas showed polygonal nuclear atypia and abnormal mitotic figures. Additional immunostaining showed positive for α-SMA, caldesmon, calponin, and negative for desmin, CD34, CKA1/AE3, S100. Based on the intraoperative findings, we diagnosed it as leiomyosarcoma arising of the right ovarian vein.

Background: Uterine leiomyosarcoma is a rare and aggressive tumour with a poor prognosis. Its metastases to the heart are even rarer, especially to the epicardium. The majority of reported cardiac metastases of uterine leiomyosarcoma were in the cardiac chambers or intramyocardial. Surgical resection of the uterine leiomyosarcoma in the early stages is the only definitive treatment for this disease. However, in the cases of cardiac metastasis, surgery is recommended only in emergencies and patients with expected beneficial outcomes., Case Presentation: Our patient was a 49-year-old female referred to the Department of Cardiac Surgery for scheduled surgery of pericardial neoplasia. The patient underwent a hysterectomy and adnexectomy three years prior owing to the uterine leiomyosarcoma. A regular follow-up magnetic resonance imaging of the abdomen and pelvis discovered neoplasia in the diaphragmic portion of the pericardium. No other signs of primary disease relapse or metastases were found. The patient was asymptomatic. The multidisciplinary team concluded that the patient is a candidate for surgery. Surgery included diastolic cardiac arrest achievement and resection of the tumour. Macroscopically, a parietal layer of the pericardium was completely free from the tumour that invaded only the apical myocardium of the left ventricle. Completed histopathology confirmed the diagnosis of leiomyosarcoma of the uterine origin. Three months after surgery, the patient received adjuvant chemotherapy with doxorubicin and dacarbazine. One year after surgery, there are no signs of new metastases., Conclusions: Strict surveillance of patients with uterine leiomyosarcoma after successful treatment of the early stage of the disease is of utmost importance to reveal metastatic disease to the heart in a timely manner and to treat it with beneficial outcomes. Surgery with adjuvant chemotherapy might be a good approach in patients with a beneficial prognosis. From a surgical point of view, it is challenging to assess the appropriate width of the resection edges to be radical enough and, at the same time, sufficiently conservative to ensure the satisfactory postoperative function of the remaining myocardium and avoid repetitive tumour growth. Therefore, intraoperative histopathology should always be performed., (© 2024. The Author(s).)

Leiomyosarcoma (LMS) has been challenging to diagnose because of limitations in clinical and radiographic predictors, as well as the lack of reliable serum or urinary biomarkers. Most uterine masses consist of benign leiomyoma (LM). However, it is currently a significant challenge in gynecology practice to differentiate LMS from LM. This inability poses grave consequences for patients, leading to a high number of unnecessary hysterectomies, infertility, and other major morbidities and possible mortalities. This study aimed to evaluate the use of Survivin-Sodium iodide symporter (Ad-Sur-NIS) as a reporter gene biomarker to differentiate malignant LMS from benign LM by using an F18-NaBF 4 PET/CT scan. The PET/CT scan images showed a significantly increased radiotracer uptake and a decreased radiotracer decay attributable to the higher abundance of Ad-Sur-NIS in the LMS tumors compared to LM ( p < 0.05). An excellent safety profile was observed, with no pathological or metabolic differences detected in Ad-Sur-NIS-treated animal versus the vehicle control. Ad-Sur-NIS as a PET scan reporter is a promising imaging biomarker that can differentiate uterine LMS from LM using F18-NaBF 4 as a radiotracer. As a new diagnostic method, the F18 NaBF 4 PET/CT scan can provide a much-needed tool in clinical practices to effectively triage women with suspicious uterine masses and avoid unnecessary invasive interventions.

The preoperative evaluation of myometrial tumors is essential to avoid delayed treatment and to establish the appropriate surgical approach. Specifically, the differential diagnosis of leiomyosarcoma (LMS) is particularly challenging due to the overlapping of clinical, laboratory and ultrasound features between fibroids and LMS. In this work, we present a human-interpretable machine learning (ML) pipeline to support the preoperative differential diagnosis of LMS from leiomyomas, based on both clinical data and gynecological ultrasound assessment of 68 patients (8 with LMS diagnosis). The pipeline provides the following novel contributions: (i) end-users have been involved both in the definition of the ML tasks and in the evaluation of the overall approach; (ii) clinical specialists get a full understanding of both the decision-making mechanisms of the ML algorithms and the impact of the features on each automatic decision. Moreover, the proposed pipeline addresses some of the problems concerning both the imbalance of the two classes by analyzing and selecting the best combination of the synthetic oversampling strategy of the minority class and the classification algorithm among different choices, and the explainability of the features at global and local levels. The results show very high performance of the best strategy (AUC = 0.99, F1 = 0.87) and the strong and stable impact of two ultrasound-based features (i.e., tumor borders and consistency of the lesions). Furthermore, the SHAP algorithm was exploited to quantify the impact of the features at the local level and a specific module was developed to provide a template-based natural language (NL) translation of the explanations for enhancing their interpretability and fostering the use of ML in the clinical setting., 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 © 2023 Elsevier B.V. All rights reserved.)

Competing Interests: Conflict of interest: None declared.

Endovascular stent-graft therapy is a commonly performed procedure for aortic lesions worldwide and complications unique to stent grafts, such as postoperative endoleaks, are well known. However, as this treatment modality becomes more popular, physicians should carefully monitor for other unexpected complications, which may not always be related to the graft. This study presents a case of leiomyosarcoma of the aorta that developed during follow-up for a type II endoleak (T2EL) after thoracic endovascular aortic repair. The presence of the T2EL hindered the diagnosis of the sarcoma at an early stage. These findings suggest that an apparent aneurysm that grows suddenly during follow-up after stent grafting should raise the index of suspicion for a neoplasm as well as an endoleak.

Aim: To evaluate the incidence of pseudoprogression in patients with metastatic or inoperable uterine leiomyosarcoma (LMS) treated with first-line single-agent doxorubicin., Methods: The Royal Marsden NHS Foundation Trust Sarcoma Unit database was searched to identify all patients with metastatic or inoperable LMS treated with first-line doxorubicin from January 2006 to January 2022. Patients with available computed tomography scans performed at baseline and during doxorubicin therapy were included. Response evaluation criteria in solid tumours v1.1 and Choi criteria were applied. Any increase in the sum of the longest diameter that decreased on the subsequent scan was labelled as pseudoprogression., Results: The total number of patients evaluated was 52. In total, 19% (n = 10) of patients treated with doxorubicin showed pseudoprogression. However, pseudoprogression at the time of the second scan was not associated with time to doxorubicin failure. Choi criteria identified 30% (n = 3) of pseudoprogressors as responding., Conclusion: Despite the use of doxorubicin as first-line therapy for soft-tissue sarcomas for over 40 years, pseudoprogression has not been described. This retrospective study shows that pseudoprogression occurs in 19% of patients with metastatic/inoperable uterine LMS treated with first-line doxorubicin. Choi criteria were not consistently able to differentiate pseudoprogression from true progression. It is imperative that oncologists and radiologists are aware of this as symptomatically stable/improving patients may benefit from continued treatment despite initial radiological growth in tumour size., 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 © 2023 Elsevier Ltd. All rights reserved.)

The purpose of this study was to distinguish leiomyosarcomas/smooth muscle tumors of uncertain malignant potential (STUMP) from leiomyomas with high signal intensity (SI) on T2-weighted imaging (T2WI) using quantitative MR texture analysis combined with patient characteristics and visual assessment. Thirty-one leiomyomas, 2 STUMPs, and 6 leiomyosarcomas showing high SI on T2WI were included. First, we searched for differences in patient characteristics and visual assessment between leiomyomas and leiomyosarcomas/STUMPs. We also compared the MR texture on T2WI and the apparent diffusion coefficient (ADC) to identify differences between leiomyomas and leiomyosarcomas/STUMPs. In the univariate analysis, significant differences between leiomyomas and leiomyosarcomas/STUMPs were observed in age, menopausal status, margin, hemorrhage, long diameter, T2-variance, T2-volume, ADC-variance, ADC-entropy, ADC-uniformity, ADC-90th and 95th percentile values, and ADC-volume (P < .05, respectively). There were significantly more postmenopausal patients with leiomyosarcomas/STUMPs than with leiomyomas, and leiomyosarcomas/STUMPs had more irregular margins, more frequent presence of hemorrhage and exhibited larger tumor diameters, T2-volume, T2-variance, ADC-volume, ADC-variance, ADC-entropy, and higher ADC-90th and 95th percentile values but lower ADC-uniformity. Multivariate analyses revealed that the independent differentiators were menopausal status, hemorrhage and ADC-entropy (P < .05, respectively). The area under the curve obtained by combining the 3 items was 0.980. The best cutoff value for ADC-entropy was 9.625 (sensitivity: 100%, specificity: 58%). The combination of menopausal status, hemorrhage, and ADC-entropy can help accurately distinguish leiomyosarcomas/STUMPs from leiomyomas with high SI on T2WI; however, external validation in a larger population is required because of the small sample size of our study., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Abstract: A 49-year-old woman was referred to our hospital due to suspected lung metastases for 1 month. She had a history of thyroid micropapillary carcinoma and uterine leiomyomas. An 18 F-FDG PET/CT scan, which was performed to search the source of the presumed metastasis of the disease, showed multiple lung metastases and 2 18 F-FDG-avid foci in the thyroid and colon. Biopsy of lung and resection of colon lesions were then performed, and the disease was finally identified to be rare primary colon leiomyosarcoma with multiple lung metastases., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Primary CNS leiomyosarcomas are rare, dural-based intracranial or intravertebral tumors seen in immunocompromised patients and are associated with latent EBV infection. They may mimic a meningioma or schwannoma on imaging but their clinical presentation progresses much more rapidly. Often times, these tumors are hard to distinguish from secondary, metastatic leiomyosarcoma., Case Presentation: A 30-year-old female with congenital HIV presented to clinic with shoulder pain, paresthesias of the right upper extremity and gait instability. She was noted to have a contrast enhancing dural-based spinal canal lesion measuring 1.5 cm at the C1 vertebral level on MRI. Surgery was proposed but patient deferred. She represented to our Emergency Department 1 month later with right-sided hemiparesis and difficulty with ambulation. On repeat MRI, the lesion had grown to 2.6 cm. She was taken to the OR emergently for gross total tumor resection. The histopathology demonstrated a primary CNS leiomyosarcoma. MRI scan of the brain revealed an extra-axial right frontal lobe lesion measuring 1.8 cm which was also treated with subtotal surgical resection followed by proton beam radiotherapy., Discussion: Primary CNS leiomyosarcomas should be considered in young immunocompromised patients presenting with dural-based spinal cord tumors. Histopathological studies including EBV testing can definitively make the diagnosis. These tumors have an aggressive nature and need to be treated with complete surgical resection to prevent severe neurological deterioration and adjuvant therapy to prevent recurrence., (© 2023. The Author(s), under exclusive licence to International Spinal Cord Society.)

Background: Uterine sarcomas are uncommon mesenchymal tumors of the uterus. The clinical problem is that the features of uterine sarcomas can sometimes mimic uterine fibroids. This study aims to investigate the clinical characteristics of patients with uterine sarcomas who were preoperative presenting mainly with uterine masses., Methods: A retrospective analysis of patients who underwent gynecological surgery for uterine sarcomas at the Obstetrics & Gynecology Hospital of Fudan University, between January 2016 and December 2021., Results: Over the 5-year period, 277 patients were final diagnosed of uterine sarcomas. A total of 162 patients were preoperatively diagnosed as uterine fibroids for surgical treatment, the majority of whom were diagnosed of uterine leiomyosarcoma (uLMS) (49/162) and low-grade endometrial stromal sarcoma (LG-ESS) (100/162). Ninety people underwent total hysterectomy and bilateral salpingo-oophorectomy (TH + BSO), while 72 underwent myomectomy followed by supplemental TH + BSO. The group with direct hysterectomy had a higher average age than the group with prior myomectomy (47.20 ± 8.94 vs. 40.86 ± 5.88, p < 0.001). Among patients preoperatively diagnosed as uterine fibroids, patients with uLMS had a higher proportion of previous myomectomy (26.53% vs. 5.00%, p < 0.001), a larger uterine mass diameter on ultrasound (8.38 ± 3.39 cm vs. 6.41 ± 1.92 cm, p < 0.001), and richer hypervascularity (34.69% vs. 18%, p = 0.024) compared with LG-ESS., Conclusions: Analysis of our data showed that a large proportion of uterine sarcomas, especially uLMS and LG-ESS, present mainly with uterine masses. Ultrasound features including a large uterine mass diameter and rich hypervascularity, and with a history of myomectomy may alert clinicians in suspicion of uLMS when compared with LG-ESS., (© 2023. The Author(s).)

Purpose: Reconstruction of the esophagus with sternohyoid muscle after enucleation of the cervical esophageal leiomyosarcoma (ELS) was rarely reported., Methods: A case of 55-year-old female with a large leiomyosarcoma in the cervical esophagus was reported. The tumor was enucleated, and the defect of the esophagus was patched with left sternohyoid muscle flap., Results: The patient recovered uneventfully after surgery. She has not had any discomfort with swallowing since surgery, and nowadays, there is not any recurrence and metastasis being detected., Conclusion: It is minimal invasive and simple to enucleate the cervical ELS and patch the defect of esophagus with sternohyoid muscle flap. For some selected patients, this method may be a promising surgical procedure to achieve both good swallowing function and satisfying prognosis.

We present the case of a 35-year-old woman with previous hereditary retinoblastoma treated with radiotherapy, admitted due to severe iron deficiency anemia. Upper endoscopy and endoscopic ultrasound revealed a 5-cm polypoid lesion in the fundus arising from muscularis mucosa. Histological findings favored a sarcoma with muscular differentiation. After exclusion of metastatic disease, the patient underwent surgery and diagnosis of primary gastric leiomyosarcoma was confirmed. We report a case of double rarity of gastric leiomyosarcoma, as she presented with severe anaemia from a polypoid lesion of the gastric fundus.

Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose.

A 63-year-old man presented with right scrotal swelling. A physical examination revealed a painless, palpable mass in the right scrotum. The mass was well defined and lobulated. Subsequently, a diagnosis of right epididymal tumor was made, and right high orchiectomy was performed. Hematoxylin-eosin and immunostaining revealed leiomyosarcoma of the epididymis. When a diagnosis of epididymal malignant tumor is made, the standard treatment is radical orchiectomy.

A 55-year-old male revealed with a 5 cm-diameter mass in the lower abdomen on ultrasonography incidentally. Computed tomography showed a mass of 7 cm in size on the left side of the bladder. A malignant tumor was suspected, and surgically excised for purpose of diagnosis and treatment. Pathological examination confirmed retroperitoneal leiomyosarcoma, and the resection margins were negative. Follow-up computed tomography scan was performed every 3 months. Repeated resections were performed for twice recurrences within a year after surgery. A year after the first surgery, lung metastasis was detected and chemotherapy was started. Although retroperitoneal leiomyosarcoma is considered to have a poor prognosis, the present case had relatively good prognosis. This may be due to early detection and repeated surgical resection.

Neoplasia of the tubular genital tract in goats, while rarely described, is most commonly reported as uterine adenocarcinoma, leiomyoma, or leiomyosarcoma. In this retrospective, single-center, case series, medical records were searched for goats with a computed tomography (CT) diagnosis of tubular genital mass and a definitive histologic (surgical biopsy or necropsy) diagnosis of malignant neoplasia. Data recorded from CT images were presence of peritoneal/retroperitoneal fluid, urinary tract obstruction, abdominal lymphadenomegaly, additional abdominal nodules/masses, and pulmonary nodules. For masses, maximum cross-sectional area, contrast enhancement, and uterine luminal fluid accumulation were also recorded. Seven goats met the inclusion criteria (leiomyosarcoma n = 5, adenocarcinoma n = 2). Both goats with adenocarcinoma had upper urinary tract obstruction, moderate to severe regional lymphadenopathy, peritoneal fluid, and peritoneal or hepatic nodules/masses; one goat with adenocarcinoma was discharged and subsequently euthanized, and the other had palliative mass debulking and was lost to follow up. Goats with leiomyosarcoma had infrequent, mild peritoneal fluid and mild sublumbar lymphadenopathy. Of the goats with leiomyosarcoma, two were euthanized at or near the time of CT imaging, two were euthanized at the time of surgery due to perceived mass non-resectability, and one had mass regression approximately four months post ovariohysterectomy but was subsequently lost to follow up. Five goats had pulmonary nodules, three of which had pathologic confirmation (pulmonary metastasis in a single patient with adenocarcinoma, and lungworm granulomas in two goats with leiomyosarcoma). Severe sublumbar lymphadenopathy and obstructive uropathy were sequelae in the two caprine patients with genital adenocarcinoma, and in none with leiomyosarcoma., (© 2022 American College of Veterinary Radiology.)

Background: Pulmonary vein thrombosis (PVT) is rarely associated with malignancies. Leiomyosarcoma, a malignant tumor originating from smooth muscles, has never been reported as the etiology of PVT., Case Presentation: In this case report, we described a 43-year-old Kurdish woman with a known case of leiomyosarcoma who presented with hemoptysis, dyspnea, and pleuritic chest pain. Chest computed tomography (CT) angiography revealed a thrombus in the left infero-posterior pulmonary vein. She was successfully treated with unfractionated heparin administered intravenously followed by orally administered warfarin. At the end of the article, we describe and compare other reports of malignancy-related PVT., Conclusions: While malignancies are not a common cause of PVT, both primary lung tumors and metastatic cancers could be associated with PVT. Delay in diagnosis may lead to serious complications and even death. Therefore, clinicians should be aware of the possibility of the development of PVT in different malignancies for appropriate diagnosis and treatment., (© 2023. The Author(s).)

Aim: To assess qualitative and quantitative magnetic resonance imaging (MRI) factors that can help distinguish leiomyosarcoma (LMS) from benign degenerative leiomyoma (BDL) and leiomyoma variants (LV) and assess the interobserver agreement for the proposed quantitative factors., Materials and Methods: Retrospective analysis of all histopathology proven cases of LV, BDL, and LMS with a preoperative MRI was performed. Twenty-seven cases were included (five LMS, three LV, and 19 BDL) with each case independently read by a pair of radiologists. Lesion size, margins, presence or absence of degeneration, necrosis, and haemorrhage were assessed on MRI along with quantitative factors such as mean T2-weighted (W) and T1W signal intensity, T1W signal heterogeneity, diffusion-weighted imaging (DWI), and apparent diffusion coefficient (ADC) ratios as well as dynamic contrast enhancement (DCE) characteristics along with the presence or absence of lymphadenopathy and extra-uterine and peritoneal spread. Mean and standard deviation for quantitative variables and frequency with percentages for qualitative variables were assessed., Results: Infiltrative margins were seen exclusively in the LMS group (n=1), with the remaining LMS cases showing lobulate or rounded smooth margins similar to BDL or LV. A high T2W signal <25% was seen exclusively in the BDL group (n=8). The presence of concomitant necrosis and haemorrhage was seen exclusively in the LMS group (n=2). Quantitative MRI had good inter-reader correlation but was not significantly different between the LMS, BDL, and LV groups., Conclusion: LMS, BDL, and LV may have overlapping features on multiparametric MRI making differentiation difficult., (Copyright © 2022 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Leiomyosarcoma commonly occurs in the abdomen, retroperitoneum, large blood vessels, and uterus[1]. Cardiac leiomyosarcoma is a rare and highly aggressive sarcoma. We reported a case of a 63-year-old male with pulmonary artery leiomyosarcoma. Transthoracic echocardiography showed a large 4.4×2.3 cm hypoechoic mass in the right ventricular outflow tract and pulmonary artery. Computed tomography pulmonary angiography showed a filling defect in a similar location. The initial impression was PE, but a tumor was not ruled out. An emergency surgery was performed due to progressively worse chest distress and dyspnea. A yellow mass that had adhered to the ventricular septum and pulmonary artery wall was detected to be compressing the pulmonary valve. Immunohistochemistry confirmed tumor cells positive staining for Desmin and smooth muscle actin and negative staining for S-100, CD34, myogenin, or myoglobin, and KI67(+)80%, indicating leiomyosarcoma. Pulmonary leiomyosarcoma showed a side-inserted heart chamber filling defect in CTA and should be excised when the patient suddenly deteriorated.