Leiomyosarcoma

No abstract available

Myxosarcomas elicit a B-cell and Treg-rich immune response; PWTs stimulate a T-cell-rich and Treg-poor reaction. The immune system response may contribute to the more aggressive behavior of myxosarcomas and the more indolent course of PWTs.

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.

Regarding responses, 5 patients had partial response (including 2 patients that were pretreated with trastuzumab), 1 patient had stable disease at 12 weeks and 4 patients had disease progression at initial assessment. All patients but one that derived clinical benefit had HER2 3 + expression.DiscussionIn the real-world setting, T-DXd showed activity in a cohort of heavily pre-treated patients with HER2-expressing gynecological malignancies.

P2, N=20, Active, not recruiting, Memorial Sloan Kettering Cancer Center | Trial completion date: Nov 2024 --> Nov 2025 | Trial primary completion date: Nov 2024 --> Nov 2025

Of the 8 patients with available follow-up, two died of disease, 3 are currently alive with disease, and 3 have no evidence of disease. The correct recognition of tumors with the KAT6B/A::KANSL1 fusion is essential because despite the bland morphological features of most cases, these tumors have a propensity for aggressive behavior.

P2, N=57, Active, not recruiting, Dana-Farber Cancer Institute | Trial primary completion date: Aug 2025 --> Jul 2024

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.

P2, N=198, Not yet recruiting, UNICANCER | Initiation date: Oct 2024 --> Feb 2025

Our findings provide a novel mechanism by which TRPV4 directly activates Ca2+/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.

P1/2, N=30, Active, not recruiting, National Taiwan University Hospital | Trial completion date: Jun 2023 --> Dec 2024

This case presenting a distinctive pattern of dedifferentiation is, to our knowledge, the first documented case of low-grade leiomyosarcoma occurring in parosteal osteosarcoma. As it enhances the clinical understanding by broadening the range of dedifferentiated components identified in parosteal osteosarcoma and highlights the diagnostic difficulties associated with these uncommon bone tumors.

To our knowledge, we report the first case of a soft tissue tumor with a PLAG1 fusion gene in an adult. In our case, we detected a new H3-3B::PLAG1 fusion in a soft tissue tumor, which originally appeared as nodular fasciitis.

Few clinical studies investigated the putative link between the activation of immunogenic cell death (ICD) and the oncological outcome. Recent data, published in a Phase 1b trial, demonstrated that an ICD-associated surge in the plasma concentration of high-mobility group box 1 (HMGB1) indicates favorable prognosis in patients with advanced leiomyosarcomas treated with the combination of doxorubicin, dacarbazine and nivolumab.

P1/2, N=26, Active, not recruiting, Viracta Therapeutics, Inc. | N=130 --> 26

Laboratory measures and utilizing NGS assay when needed, could be implemented when encountering such problematic "low-level" MDM2 amplification specimens to avoid misdiagnosis and misuse of targeted therapy. Future studies are needed to better characterize and investigate such findings.

P1/2, N=62, Active, not recruiting, Massachusetts General Hospital | Recruiting --> Active, not recruiting | Trial primary completion date: Jul 2024 --> Jul 2025

MutPTEN vs wtPTEN was associated with worse OS in advanced STS. If confirmed, our findings could be helpful for prognostic stratification in clinical practice and for further understanding the molecular mechanisms of STS.

P2, N=40, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2024 --> Dec 2025 | Trial primary completion date: Dec 2024 --> Dec 2025

P=N/A, N=19, Completed, Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University

P2, N=29, Recruiting, Northwestern University | Not yet recruiting --> Recruiting

The patient described herein had a TFE-positive uterine malignant PEComa with lung metastasis and responded well to the mTOR inhibitor, everolimus. Close follow-up in the last 3 years showed remission without recurrence or progression.

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.

Across cohorts, only SDH-mutant GIST patients experienced prolonged disease control. Despite evidence of target engagement, patients enrolled to all other cohorts had short PFS, suggesting rapid adaptation to ATR inhibitor monotherapy. Among these patients, those with tumors expressing SLFN11 during berzosertib exposure derived the most clinical benefit.

We identified three unique sarcoma cell cycle phenotypes that have prognostic significance. This performs similarly to the AJCC staging system.

P2, N=10, Active, not recruiting, Institut für Klinische Krebsforschung IKF GmbH at Krankenhaus Nordwest | Recruiting --> Active, not recruiting | Trial completion date: Jul 2024 --> Jan 2025

Surgeons should consider diverting colostomy creation for patients with risk factors such as age 70 or older, ASA-PS III/IV, and hypertension.

Our analysis of the largest number of cLMS cases to-date highlights the importance of large cohort sizes and the exploration beyond small targeted gene panels when performing molecular analyses, as it allowed a comprehensive exploration of the mutational landscape of these tumours and identification of novel candidate driver events. It also uniquely afforded the opportunity to compare the molecular phenotype of cLMS with LMS of other tissue types, such as uterine and soft tissue LMS. Given that molecular profiling has resulted in the development of novel targeted treatment approaches for uterine and soft tissue LMS, our study now allows the same opportunities to become available for patients with cLMS.

P2, N=66, Recruiting, National Cancer Institute (NCI) | Suspended --> Recruiting

As the cases are rare in nature, screening is impractical. Hence, the diagnosis of uterine leiomyosarcoma is done by histopathologic examination after surgery.

These findings deepen our understanding of disease etiology and offer promising avenues for drug repurposing. Experimental validation is needed to translate these insights into clinical applications and innovative treatments.

This scheme of doxorubicin, dacarbazine, and nivolumab is feasible and well tolerated. Clinical activity is encouraging and the prognostic impact of HMGB1 supports the relevance of ICD activation. Further clinical research is already underway with this concept in leiomyosarcoma.

P2, N=72, Active, not recruiting, City of Hope Medical Center | Trial completion date: Sep 2024 --> Dec 2024 | Trial primary completion date: Sep 2024 --> Dec 2024

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.

The consequences of NNMT overexpression, such as the activation and inactivation of oncoproteins and tumor suppressor proteins, respectively, as well as the enrichment of the cancer stem cell population, overlap with the major mechanisms responsible for poor prognosis in mesenchymal tumors. NNMT may be investigated further in the context of antitumor treatment in patients with mesenchymal malignancies.

P1/2, N=130, Active, not recruiting, Viracta Therapeutics, Inc. | Recruiting --> Active, not recruiting

P1, N=92, Recruiting, City of Hope Medical Center | Active, not recruiting --> Recruiting | Trial completion date: Sep 2024 --> Jan 2026 | Trial primary completion date: Sep 2024 --> Jan 2026

Furthermore, inhibiting BET proteins with their small, potent inhibitors (JQ1 and I-BET 762) significantly inhibited the uLMS proliferation dose-dependently via cell cycle arrest. The connections between BET proteins and crucial biological pathways provide a fundamental structure to better understand uterine diseases, particularly uLMS pathogenesis. Accordingly, targeting the vulnerable epigenome may provide an additional regulatory mechanism for uterine cancer treatment.

Sarcomas exhibit a high degree of defects in APM components, with differences among histotypes and tumoral areas. The most commonly altered APM components were HLA Class I subunit β2-microglobulin, HLA Class I subunit α (HC10), and MHC I transporting unit TAP2. The loss of APM components was prognostic of DM and OS and clinically relevant for LMS and DDLPS. This study explores sarcoma molecular mechanisms, enriching personalized therapeutic approaches.

It is about a 79-year-old female patient, who presented with a progressive enlarged right paraaortic retroperitoneal mass, thought first to be leiomyosarcoma of vena cava on imaging. Subsequently, high alpha-fetoprotein (AFP) level and biopsy allowed the diagnosis of primary extrahepatic hepatocellular carcinoma.

P2, N=29, Not yet recruiting, Northwestern University

This is a rare case of leiomyosarcoma associated with HLRCC, and our patient remains under surveillance with interval abdominal imaging and skin examination. Leiomyosarcomas are difficult to distinguish clinically from their benign counterpart; therefore, histopathological examination is paramount with a low threshold for excision.