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).
CONCLUSIONS: 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.

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.

Leiomyosarcomas (LMS) arise from smooth muscle cells with more predilection to the uterus, abdomen, retroperitoneum, and blood vessels. LMS of vagina is very rare and usually presents in the early stage as an asymptomatic mobile mass with no clinical features of malignancy and gives the appearance of a benign lesion which can easily be mistaken for a Bartholin\'s cyst or a vaginal fibroid. The chances of metastasis in LMS are high with poor survival rates. Histopathology confirms the diagnosis and treatment still remains controversial due to less data on this rare malignancy. Even though there is no evidence that leiomyoma can transform into LMS, benign-looking vaginal fibroids need to be resected to avoid misdiagnosis of LMS. We present a case of vaginal LMS which was mistaken to be Bartholin\'s cyst due to the lack of knowledge of this aggressive tumor and further management.

Uterine leiomyosarcoma (uLMS) is a type of malignant soft-tissue tumor, which is developed from myometrium in the female reproductive system. This disease is difficult to be distinguished from benign uterine leiomyoma in the early stages, but it progresses aggressively and relentlessly. Hence, uLMS has a dismal prognosis and high rates of both misdiagnosis and missed diagnosis. Unfortunately, current studies of uLMS pathogenesis and disease biology are inadequate. uLMS disease models are also very limited, hindering the development of effective therapeutics. In this review, we focus on the pathological molecular biology of uLMS, and systematically review the molecular genetic features, epigenetic variants, experimental models, and clinical research progress of uLMS. We further discuss the development direction and potential needs of uLMS in the fields of tumor evolution, tumor microenvironment, and tumor therapy, with the aim of providing a better understanding of the pathobiological mechanism of uLMS and providing a reference for the development of potential diagnostic and therapeutic strategies.
子宫平滑肌肉瘤(uterine leiomyosarcoma，uLMS)是一种发生在女性生殖系统子宫肌层的恶性软组织肿瘤，漏诊误诊率高、侵袭性强、预后差。uLMS的发生机制尚未明确，疾病生物学研究相对滞后，实验模型和治疗手段也较为有限。本文重点关注了uLMS的病理分子生物学，系统梳理了uLMS的分子遗传学特征、表观遗传学变异、实验模型以及临床研究进展，同时还探讨了uLMS在肿瘤演进、肿瘤微环境、肿瘤治疗等生物学研究领域的发展方向和潜在需求，以期更好地理解uLMS的病理生物学机制并为开发潜在诊疗策略提供参考。.

Lumps are commonly found in the femoral triangle. Femoral hernias and lymphadenopathy are included in the differential diagnosis. One of the rare possibilities of femoral triangle swellings is leiomyosarcoma. Leiomyosarcoma originating from the walls of blood vessels is very rare, and only a few cases are reported. We present a case of a 50-year-old male patient complaining of swelling over the left thigh. Ultrasonography showed a highly vascular soft tissue tumour in the anteromedial compartment of the thigh. Magnetic resonance imaging (MRI) was done later. It showed a well-defined, heterogeneously enhancing solid cystic lesion along the femoral vein with intravenous extension, and the femoral artery was seen encasing along its length. A surgical exploration of the lesion suggested a mass originating from the femoral vein, obstructing the vein itself. The mass was excised, and the defect in the vein was repaired. Histopathological examination revealed the mass to be leiomyosarcoma of vascular origin.

BACKGROUND: This is a multicentre, single-arm, phase II study aimed at further exploring the activity of trabectedin as second-/further-line treatment in retroperitoneal leiomyosarcoma (LMS) and well-differentiated/dedifferentiated liposarcoma (LPS).
METHODS: The primary endpoint was the growth modulation index (GMI) defined as the ratio between PFS under trabectedin (PFS) and during previous chemotherapy treatment: time to progression (TTP-1). Secondary endpoints were objective response rate (ORR) and PFS. As per protocol, patients were considered responders if the GMI was >1.33, non-responders if <0.75 and neither if 0.76-1.32.
RESULTS: Overall 91 patients were assessable for the primary endpoint (32 patients with LMS and 59 patients with LPS): the median number of cycles received was 6.0 (Q1-Q3 3.0-12.0), and the main reason for treatment discontinuation was disease progression in 72% of patients. The median PFS was 6.0 months, while the median TTP1 was 7.5 months (8.1 and 6.4 months for LMS and LPS, respectively). Thirty-three patients [52%, 95% confidence interval (CI) 36% to 58%, P = 0.674, odds of response 1.1] had a GMI >1.33 (LMS 46%, 95% CI 26% to 67%, odds of response 0.85; LPS 56%, 95% CI 40% to 72%, odds of response 1.3). Overall, in LPS we observed 15/47 patients with a GMI <0.5 and 15/47 patients with a GMI >2. Among LMS patients, 9/26 had a GMI <0.5 and 10/26 had a GMI >2. Overall, ORR (complete response + partial response) was 16% (24% for LMS and 12% for LPS).
CONCLUSIONS: While the primary endpoint of the study was not met, we noticed a subgroup of patients with a markedly discrepant TTP with trabectedin in comparison to previous therapy (GMI <0.5 or >2, the latter including some patients with a long TTP with trabectedin). A mismatch between PFS and overall survival was observed, possibly due to the natural history of the two different histologies and the availability of further lines in LMS.

UNASSIGNED: Primary leiomyosarcoma (LMS) of the colon is a rare neoplasm and constitutes less than 0.1% of all colon malignancies. These tumors are more aggressive and have poorer prognoses than other gastrointestinal tumors, including gastrointestinal stromal tumors (GIST) or adenocarcinomas. The authors herein report two cases and review the literature to highlight the epidemiology, diagnosis, treatment and prognosis of this uncommon malignancy.
UNASSIGNED: The authors reported two very rare cases of LMS of left colon, which referred to our institution with symptoms of abdominal pain. After the initial investigations, patients were diagnosed with primary colonic leiomyosarcoma that underwent laparotomy. In both cases pathological examination revealed a spindle cell tumor growing circumferentially and transmurally in the colon. Final immunohistochemistry were positive with SMA, CK and desmin without the expression of GIST markers (CD117, CD34 and DOG1) that confirmed leiomyosarcoma. One patient was diagnosed with diffused peritoneal metastasis at 6 months postoperatively and he died after 2 months of paliative care, another one is still on active surveillance.
UNASSIGNED: LMS of the colon is a really rare entity and is only presented in clinical case reports. LMS has non-specific symptoms and is commonly diagnosed when it reaches a large size. Surgery is a mainstay treatment option. Nowadays, there is no clear evidence for the effectiveness of chemotherapy and radiation therapy.
UNASSIGNED: LMS is a rare neoplasm of colon. For the time being, there is no guidelines for treatment, but surgery still plays a fundamental role.

This paper presents a unique clinical observation of 16 years of use without replacement of a domestic voice prosthesis in a patient after laryngectomy. Long-term recurrence-free survival was achieved as a result of treatment of laryngeal leiomyosarcoma.
В данной работе представлено уникальное клиническое наблюдение 16-летней эксплуатации без замены отечественного голосового протеза у больного после ларингэктомии. Достигнута длительная безрецидивная выживаемость в результате лечения лейомиосаркомы гортани.

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.

UNASSIGNED: In recent years, due to the increase in medical mal-practice complaints, the Sicilian Regional Health System has adopted procedures for the direct management of claims by each health facility with the aim of reducing the costs of insurance premiums and related taxes. Mandatory sentinel event monitoring is a crucial part of this strategy to improve patient safety and quality of care. The reported case relates to a laparoscopic myomectomy surgery performed by means of morcellation, a controversial technique. After the FDA\'s intervention in 2014, it is believed that morcellation may worsen the staging of the disease by spreading malignancies such as leiomyosarcoma into the abdomen.
UNASSIGNED: A 28-year-old woman, underwent laparoscopic surgery for uterine fibroids and an ovarian cyst removal in August 2018. Post-surgery, she was diagnosed with Leiomyoma. She returned to the hospital due to metrorrhagia and was discharged after a week. Persistent symptoms led to her readmission and subsequent exploratory laparoscopic surgery at another hospital. This resulted in a total hysterectomy and the discovery of uterine leiomyosarcoma, with FIGO STAGE IIIB staging. Despite chemotherapy, she passed away six months later.
UNASSIGNED: This case highlights medical-legal issues. Informed consent for morcellation and its risks was not obtained. The morcellation technique was used, increasing cancer spread risk. The histopathological process was inadequate, with three biopsies leading to misdiagnosis. This could be medical malpractice, making providers legally responsible for the patient\'s deteriorating condition and the anticipation of possible death.