Vena cava leiomyosarcoma surgery results in a retrospective cohort of 41 patients from two centers.

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.

Pleomorphic leiomyosarcoma of the maxilla with metastasis to the colon: A case report.

BACKGROUND: Pleomorphic leiomyosarcomas make up around 8.6% of all leiomyosarcomas. They behave aggressively and often have poor prognoses. They can affect the gastrointestinal tract and retroperitoneum. To date, pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients. CASE SUMMARY: The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver. His most recent positron emission tomography-computed tomography (PET-CT) scan showed uptake in the ascending and transverse colons. A colonoscopy revealed a 5.0 cm × 3.5 cm × 3.0 cm pedunculated polyp in the ascending colon. The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet. Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma. CONCLUSION: Uptake(s) on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.

Combined Treatment of Uterine Leiomyosarcoma with Gamma Secretase Inhibitor MK-0752 and Chemotherapeutic Agents Decreases Cellular Invasion and Increases Apoptosis.

Due to limited effective therapeutics for uterine leiomyosarcoma (uLMS), the impact of the gamma secretase inhibitor (GSI) MK-0752 with common chemotherapeutics was explored in uLMS. MTT assays were performed on two human uLMS cell lines, SK-UT-1B and SK-LMS-1, using MK-0752, docetaxel, doxorubicin, and gemcitabine, individually and in combination, to determine cell viability after treatment. Synergistic combinations were used in transwell invasion assays, cell cycle flow cytometry, proliferation assays, and RNA sequencing. In SK-UT-1B, MK-0752 was synergistic with doxorubicin and gemcitabine plus docetaxel. In SK-LMS-1, MK-0752 was synergistic with all individual agents and with the combination of gemcitabine plus docetaxel. MK-0752, gemcitabine, and docetaxel decreased invasion in SK-UT-1B 2.1-fold* and in SK-LMS-1 1.7-fold*. In SK-LMS-1, invasion decreased 1.2-fold* after treatment with MK-0752 and docetaxel and 2.2-fold* after treatment with MK-0752 and doxorubicin. Cell cycle analysis demonstrated increases in the apoptotic sub-G1 population with MK-0752 alone in SK-UT-1B (1.4-fold*) and SK-LMS-1 (2.7-fold**), along with increases with all combinations in both cell lines. The combination treatments had limited effects on proliferation, while MK-0752 alone decreased proliferation in SK-LMS-1 (0.63-fold**). Both MK-0752 alone and in combination altered gene expression and KEGG pathways. In conclusion, the combinations of MK-0752 with either doxorubicin, docetaxel, or gemcitabine plus docetaxel are potential novel therapeutic approaches for uLMS. (* p < 0.05, ** p < 0.01).

Development and Validation of a Nomogram to Predict the Risk of Special Uterine Leiomyoma Pathological Types or Leiomyosarcoma in Postmenopausal Women: A Retrospective Study.

Purpose: The aim of this study was to investigate the risk factors of postmenopausal special uterine leiomyoma pathological types or leiomyosarcoma and to develop a nomogram for clinical risk assessment, ultimately to reduce unnecessary surgical interventions and corresponding economic expenses. Methods: A total of 707 patients with complete information were enrolled from 1 August 2012 to 1 August 2022. Univariate and multivariate logistic regression models were used to analyse the association between variables and special uterine leiomyoma pathological types or leiomyosarcoma in postmenopausal patients. A nomogram for special uterine leiomyoma pathological types or leiomyosarcoma in postmenopausal patients was developed and validated by bootstrap resampling. The calibration curve was used to assess the accuracy of the model and receiver operating characteristic (ROC) curve and decision curve analysis (DCA) were compared with the clinical experience model. Results: The increasing trend after menopause, the diameter of the largest uterine fibroid, serum carcinoembryonic antigen 125 concentration, Serum neutrophil to lymphocyte ratio, and Serum phosphorus ion concentration were independent risk factors for special uterine leiomyoma pathological types or leiomyosarcoma in postmenopausal patients. We developed a user-friendly nomogram which showed good diagnostic performance (AUC=0.724). The model was consistent and the calibration curve of our cohort was close to the ideal diagonal line. DCA indicated that the model has potential value for clinical application. Furthermore, our model was superior to the previous clinical experience model in terms of ROC and DCA. Conclusion: We have developed a prediction nomogram for special uterine leiomyoma pathological types or leiomyosarcoma in postmenopausal patients. This nomogram could serve as an important warning signal and evaluation method for special uterine leiomyoma pathological types or leiomyosarcoma in postmenopausal patients.

2­D08 mediates notable anticancer effects through multiple cellular pathways in uterine leiomyosarcoma cells.

2',3',4'­trihydroxyflavone (2­D08), a SUMO E2 inhibitor, has several biological functions, including anticancer activity, but its effects on uterine leiomyosarcoma (Ut­LMS) are unknown. The anticancer activity of 2­D08 was explored in an in vitro model using SK­LMS­1 and SK­UT­1B cells (human Ut­LMS cells). Treatment with 2­D08 inhibited cell viability in a dose­ and time­dependent manner and significantly inhibited the colony­forming ability of Ut­LMS cells. In SK­UT­1B cells treated with 2­D08, flow cytometric analysis revealed a slight increase in apoptotic rates, while cell cycle progression remained unaffected. Western blotting revealed elevated levels of RIP1, indicating induction of necrosis, but LC3B levels remained unchanged, suggesting no effect on autophagy. A lactate dehydrogenase (LDH) assay confirmed increased LDH release, further supporting the induction of apoptosis and necrosis by 2­D08 in SK­UT­1B cells. 2­D08­induced production of reactive oxygen species and apoptosis progression were observed in SK­LMS­1 cells. Using Ki67 staining and bromodeoxyuridine assays, it was found that 2­D08 suppressed proliferation in SK­LMS­1 cells, while treatment for 48 h led to cell­cycle arrest. 2­D08 upregulated p21 protein expression in SK­LMS­1 cells and promoted apoptosis through caspase­3. Evaluation of α­SM­actin, calponin 1 and TAGLN expression indicated that 2­D08 did not directly initiate smooth muscle phenotypic switching in SK­LMS­1 cells. Transcriptome analysis on 2­D08­treated SK­LMS­1 cells identified significant differences in gene expression and suggested that 2­D08 modulates cell­cycle­ and apoptosis­related pathways. The analysis identified several differentially expressed genes and significant enrichment for biological processes related to DNA replication and molecular functions associated with the apoptotic process. It was concluded that 2­D08 exerts antitumor effects in Ut­LMS cells by modulating multiple signaling pathways and that 2­D08 may be a promising candidate for the treatment of human Ut­LMS. The present study expanded and developed knowledge regarding Ut­LMS management and indicated that 2­D08 represents a notable finding in the exploration of fresh treatment options for such cancerous tumors.

Case report: Spontaneous rupture of leiomyosarcoma uteri 8 months after primary laparoscopic surgery of STUMP.

Introduction: Leiomyosarcoma (LMS), together with smooth muscle tumors of uncertain malignant potential (STUMP) and benign leiomyomas, belongs to a heterogeneous group of uterine neoplasms. According to the World Health Organization, tumors originating from uterine smooth muscle fibers are the second most frequent tumors. It is challenging to distinguish between STUMP and LMS because of an overlap of symptoms, lack of a precise definition, and unequivocal information obtained using imaging diagnostic methods. Following myomectomy or hysterectomy with laparoscopic or laparotomy surgery and a definitive histological diagnosis of STUMP, the course of treatment is determined by the need to preserve fertility. In 2014, the U.S. Food and Drug Administration published an alert that unprotected laparoscopic morcellation is correlated with a 3-fold higher likelihood of dissemination of malignant cells and disease progression. Unprotected morcellation was independently associated with a higher risk of disease recurrence after demolition or conservative surgery, with a relative risk of 2.94. Conclusion: Hematoperitoneum resulting from the spontaneous rupture of a uterine tumor is a rare gynecological emergency, with very few cases reported in the last decade.

Case series of the inferior vena cava primary leiomyosarcoma treatment.

Tumors of the inferior vena cava (IVC) are rare and usually malignant and they can be primary and secondary. The most common primary tumor of the IVC is primary leiomyosarcoma. The first case of primary IVC leiomyosarcoma has been described in 1871 [1].The total number of 218 cases has collected until 1996 [2]. After that, three large single center series of these tumors emerged [3-5]. Present a series of five cases of these tumors. All the patients underwent a wide complete resection of tumors and the reconstruction with Dacron grafts. One patient died 19 months after the surgery, while the remaining ones survived without a local and system disease relapse. Although a surgical resection combined with the chemotherapy is often not curative, it can achieve a significant long-term survival. For this reason, we recommend the aggressive surgical management using the modern vascular surgical and oncology techniques.

Retroperitoneal leiomyosarcoma mimicking an ovarian tumor diagnosed using a negative ovarian pedicle sign.

Retroperitoneal leiomyosarcoma (RPLMS) is rare and usually presents as a large abdominal mass with poor clinical symptoms. Radiological findings of an RPLMS arising in the pelvis of a woman resemble those of adnexal tumors. Herein, we present a case of RPLMS mimicking an adnexal tumor which was differentiated from having an ovarian origin as the right ovarian vein was passing through the tumor but there was no direct vascular connection with the tumor. Therefore, it is important to identify the ovarian vein to distinguish between these tumors.

Utility of ADC Values for Differentiating Uterine Sarcomas From Leiomyomas: Systematic Review and Meta-Analysis.

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 and 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 (MEDLINE, EMBASE, and Cochrane databases) for studies distinguishing uterine sarcomas from leiomyomas using MRI, including ADC, with pathologic tissue confirmation or imaging follow-up as the reference standard. Data extraction and QUADAS-2 quality assessment were performed. Sensitivity and specificity were pooled using hierarchic models, including bivariate and hierarchic summary ROC models. Metaregression was used to assess the impact of various factors on heterogeneity. Evidence synthesis: Twenty-one studies met study inclusion criteria. Pooled sensitivity and specificity were 89% (95% CI, 82-94%) and 86% (95% CI, 78-92%), respectively. Area under the summary ROC curve was 94% (95% CI, 92-96%). Context of ADC interpretation (i.e., standalone vs 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 as compared with standalone ADC assessment. ADC cutoff values ranged (0.87-1.29 × 10-3 mm2/s) but were not associated with statistically different performance (p = .37). Pooled mean ADC values in sarcomas and leiomyomas were 0.904 × 10-3 mm2/s and 1.287 × 10-3 mm2/s, respectively. Conclusion: As part of mpMRI evaluation of uterine masses, mass ADC value less than 0.904 × 10-3 mm2/s may be a useful test-positive threshold for uterine sarcoma, consistent with 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.

A Rare Case of Leiomyosarcoma Arising From the Stomach: A Case Report and Review of the Literature.

Leiomyosarcomas (LMSs) account for 10-20% of all soft-tissue sarcomas (STSs). Soft-tissue sarcomas, and more specifically LMS, typically originate from the uterus, extremity, retroperitoneal, or lower intraabdominal gastrointestinal organs. Due to the rarity and variability in presentation, it is difficult to describe identifiable risk factors, determine etiology, predict disease progression, and prognosticate these types of neoplasms. We present the case of a 77-year-old woman presenting to the emergency department with shortness of breath. After being diagnosed and treated for mild exacerbation of congestive heart failure, she was incidentally found to be anemic. Further workup, including an esophagogastroduodenoscopy, revealed a bleeding gastric mass, which was biopsied. Histopathology and immunohistochemistry confirmed the mass to be primary gastric LMS. Due to its rarity, an interdisciplinary approach involving clinical, histopathologic, and immunohistochemical data is necessary to successfully identify and diagnose gastrointestinal LMS. This case report aims to contribute to the paucity of information available in the literature regarding gastric LMS so that it may be better understood.

Current landscape of primary small bowel leiomyosarcoma: cases report and a decade of insights.

The incidence of leiomyosarcoma (LMS) is about 4-5/100,000 individuals per year. LMSs occurring in the small bowel are even rarer, and their preoperative diagnosis is very difficult. We described two patients with pathologically confirmed small bowel LMS and analyzed their clinical and medical imaging features. Similar cases reported in English in Pubmed database over the past decade were reviewed and summarized. These tumors were categorized by the growth direction and relationship with the intestinal lumen into three types: intraluminal (n = 10), intermural (n = 3), and extraluminal (n = 7). Notably, among the three types of LMS, the intramural leiomyosarcoma stands out as a noteworthy subtype. Emerging evidence suggests that smaller tumor size (< 5 cm) and the intraluminal type may serve as favorable prognostic indicators, while the extraluminal type is associated with relatively poor prognosis. Furthermore, the integration of imaging features with CA125 and LDH biomarkers holds promise for potential diagnostic value in LMS.

Primary Tumor Resection in Leiomyosarcoma Patients With Synchronous Isolated Lung Metastases: A National Cancer Database Study.

INTRODUCTION: Up to half of patients with leiomyosarcoma (LMS) present with distant metastases, most commonly in the lungs. Despite guidelines around managing metachronous oligometastatic disease, limited evidence exists for synchronous isolated lung metastases (SILMs). Our histology-specific study describes management patterns and outcomes for patients with LMS and SILM across disease sites. METHODS: We used the National Cancer Database to analyze patients with LMS of the retroperitoneum, extremity, trunk/chest/abdominal wall, and pelvis with SILM. Patients with extra-pulmonary metastases were excluded. We identified factors associated with primary tumor resection and receipt of metastasectomy. Outcomes included median, 1-year, and 5-year overall survival (OS) across treatment approaches using log-rank tests, Kaplan-Meier curves, and Cox proportional hazard models. RESULTS: We identified 629 LMS patients with SILM from 2004 to 2017. Patients were more likely to have resection of their primary tumor or lung metastases if treated at an academic center compared to a community cancer center. Five year OS for patients undergoing both primary tumor resection and metastasectomy was 20.9% versus 9.2% for primary tumor resection alone, and 2.6% for nonsurgical patients. Median OS for all-comers was 15.5 mo. Community treatment site, comorbidity score, and larger primary tumors were associated with worse survival. Chemotherapy, primary resection, and curative intent surgery predicted improved survival on multivariate Cox regression. CONCLUSIONS: An aggressive surgical approach to primary LMS with SILM was undertaken for select patients in our population and found to be associated with improved OS. This approach should be considered for suitable patients at high-volume centers.

PLAG1-Rearranged Uterine Sarcomas: A Study of 11 Cases Showing a Wide Phenotypical Spectrum Not Limited to Myxoid Leiomyosarcoma-like Morphology.

PLAG1 gene fusions were recently identified in a subset of uterine myxoid leiomyosarcomas (M-LMS). However, we have encountered cases of PLAG1-rearranged uterine sarcomas (PLAG1-US) lacking M-LMS-like morphology and/or any expression of smooth muscle markers. To better characterize their clinicopathological features, we performed a multi-institutional search which yielded 11 cases. The patients ranged in age from 34-72 years (mean: 57). All tumors arose in the uterine corpus, ranging in size from 6.5-32 cm (mean: 15). The most common stage at presentation was pT1b (n=6), three cases had stage pT1 (unspecified) and one case each presented in stage pT2a and pT3b. Most were treated only by hysterectomy with adnexectomy. The follow-up (range: 7-71 months; median: 39 months) was available for 7 patients. Three cases (7-21 months of follow-up) had no evidence of disease. Three out of 4 remaining patients died of disease within 55-71 months, while the last developed peritoneal spread and was transferred for palliative care at 39 months. Morphologically, the tumors showed a high inter- and intratumoral heterogeneity. M-LMS-like and epithelioid LMS-like morphology was present in 3 and 5 primary tumors, respectively, the rest mostly presented as non-descript ovoid/spindle cell sarcomas. Unusual morphological findings included prominently hyalinized stroma (n=3), adipocytic differentiation with areas mimicking myxoid liposarcoma (n=2), osteosarcomatous differentiation (n=1) and undifferentiated pleomorphic sarcoma-like areas (n=1). The mitotic activity ranged from 3-24 mitoses/10 high-power fields (mean: 9), 3/10 cases showed necrosis. In 3/11 cases, no expression of SMA, h-caldesmon or desmin was noted, whereas 5/5 cases expressed PLAG1. By RNA-sequencing, the following fusion partners were identified: PUM1, CHCHD7 (each n=2), C15orf29, CD44, MYOCD, FRMD6, PTK2 and TRPS1 (each n=1). One case only showed PLAG1 gene break by FISH. Our study documents a much broader morphological spectrum of PLAG1-US than previously reported, encompassing but not limited to M-LMS-like morphology with occasional heterologous (particularly adipocytic) differentiation. Since it is currently difficult to precisely define their line of differentiation, for the time being, we suggest using a descriptive name PLAG1-rearranged uterine sarcoma.

Leiomyosarcoma of the small bowel presenting as an acute small bowel obstruction.

Leiomyosarcoma is a subtype of soft-tissue sarcoma, which is a rare soft-tissue malignancy comprising < 1% of adult cancers. There are a variety of etiologies of small bowel obstruction. Infrequently, small bowel malignancies can first present as small bowel obstruction. In exceedingly rare cases, leiomyosarcomas can be the offending malignancy. A 53-year-old male presented to the emergency department with several weeks of persistent right abdominal pain, nausea, and vomiting. Computed tomography scan revealed a central necrotic mass within the right lower quadrant originating from the small bowel. The patient underwent exploratory laparotomy to relieve the obstruction and a mass was identified originating from the terminal ileum that adhered to surrounding structures. Pathological analysis determined the mass to be small bowel leiomyosarcoma. Leiomyosarcoma is definitively diagnosed after primary resection with histopathology and immunohistochemistry. As opposed to other small bowel neoplasms, surgical resection with negative margins is the only potentially curative option.

Clinical actionability of BRCA2 alterations in uterine leiomyosarcoma: a molecular tumor board case report and a cBioPortal comprehensive analysis.

BACKGROUND: Uterine leiomyosarcoma (uLMS) represents one of the most common sarcoma histotypes, demonstrating an overall dismal prognosis. Previous studies reported uLMS to carry recurrent somatic BRCA2 homozygous deletions, related to significant clinical benefits from the use of PARP inhibitors. METHODS: To investigate the prevalence in uLMS of genomic alterations (alt) in BRCA2 and other homologous recombination (HR) and DNA damage response (DDR) genes, cBioPortal was accessed and data were retrieved from studies including pan-sarcoma histologies. HR-/DDR-genes included BRCA1, BRCA2, ATM, BARD1, BRIP1, CHEK1, CHEK2, FANCA, FANCB, FANCC, FANCD2, FANCE, FANCF, FANCG, FANCI, FANCL, FANCM, NBN, PALB2, RAD51C, RAD51D, RAD50, and ATR. Only oncogenic/likely oncogenic alterations were included according to OncoKB. CLINICAL REPORT AND RESULTS: We reported a clinical case of a patient affected by a highly pretreated uLMS discussed at the European Institute of Oncology Molecular Tumor Board. A targeted next-generation sequencing panel demonstrated a somatic BRCA2 homozygous deletion (homDel). Upon access to Niraparib, a remarkable response of 15 months was observed before experiencing disease progression. In the genomic query, among 2393 cases, uLMS (n = 193) displayed 9 of all 31 BRCA2alt observed, representing the only sarcoma histotype showing an enrichment in BRCA2alt (4.66%; q < 0.001). All of 9 BRCA2alt were represented by homDel, which related to a high fraction of genome altered. CONCLUSION: uLMS displays a significant frequency of somatic BRCA2alt homDel. Considering their dismal prognosis, further investigation is warranted to test the use of PARPi in uLMS, and particularly in the setting of BRCA1/2 alterations.

[Portal leiomyosarcoma: An extremely rare location!] / Léiomyosarcome portal : une localisation extrêmement rare !

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.

HMGA1 regulates trabectedin sensitivity in advanced soft-tissue sarcoma (STS): A Spanish Group for Research on Sarcomas (GEIS) study.

HMGA1 is a structural epigenetic chromatin factor that has been associated with tumor progression and drug resistance. Here, we reported the prognostic/predictive value of HMGA1 for trabectedin in advanced soft-tissue sarcoma (STS) and the effect of inhibiting HMGA1 or the mTOR downstream pathway in trabectedin activity. The prognostic/predictive value of HMGA1 expression was assessed in a cohort of 301 STS patients at mRNA (n = 133) and protein level (n = 272), by HTG EdgeSeq transcriptomics and immunohistochemistry, respectively. The effect of HMGA1 silencing on trabectedin activity and gene expression profiling was measured in leiomyosarcoma cells. The effect of combining mTOR inhibitors with trabectedin was assessed on cell viability in vitro studies, whereas in vivo studies tested the activity of this combination. HMGA1 mRNA and protein expression were significantly associated with worse progression-free survival of trabectedin and worse overall survival in STS. HMGA1 silencing sensitized leiomyosarcoma cells for trabectedin treatment, reducing the spheroid area and increasing cell death. The downregulation of HGMA1 significantly decreased the enrichment of some specific gene sets, including the PI3K/AKT/mTOR pathway. The inhibition of mTOR, sensitized leiomyosarcoma cultures for trabectedin treatment, increasing cell death. In in vivo studies, the combination of rapamycin with trabectedin downregulated HMGA1 expression and stabilized tumor growth of 3-methylcholantrene-induced sarcoma-like models. HMGA1 is an adverse prognostic factor for trabectedin treatment in advanced STS. HMGA1 silencing increases trabectedin efficacy, in part by modulating the mTOR signaling pathway. Trabectedin plus mTOR inhibitors are active in preclinical models of sarcoma, downregulating HMGA1 expression levels and stabilizing tumor growth.

Primary leiomyosarcoma of thyroid with pulmonary metastasis: A diagnostic odyssey.

The presented primary thyroid leiomyosarcoma (TL) case report underscores the importance of recognizing and addressing the diagnostic challenges and management complexities associated with this exceedingly rare malignancy. Given the limited effective therapeutic strategies available, timely intervention, thorough diagnostics, and vigilant follow-up are paramount in managing such intricate tumors. Further research focusing on molecular-based treatment modalities is imperative to improve patient outcomes in cases of primary TL.

Leiomyosarcoma of stomach extending to gastroesophageal junction and distal esophagus as a rare cause of dysphagia: a case report.

Introduction and importance: Gastric leiomyosarcoma is a rare malignant tumor among the primary gastric carcinomas. Among the different common presentations, dysphagia is an uncommon presentation of gastric leiomyosarcoma. Case presentation: A 29-year-old female presented with complaints of progressive dysphagia for 1 year associated with vomiting, significant weight loss, and anorexia for 6 months. On blood investigations, she had anemia, hypokalemia, prerenal acute kidney injury, and unconjugated hyperbilirubinemia. Upper gastrointestinal endoscopy and contrast-enhanced computed tomography (CECT) were initially suggestive of carcinoma of stomach. Immunohistochemistry was diagnostic of leiomyosarcoma of stomach extending to the gastroesophageal junction and distal esophagus. She underwent total gastrectomy with distal esophagectomy with lateral segmentectomy of liver (nonanatomical) with Roux-en-Y esophago-jejunal anastomosis (end-to-side and retro-colic) through thoracoabdominal approach. After 6 weeks, she received four cycles of doxorubicin therapy. Follow-up at 18 months after surgery revealed no recurrence of malignancy. Clinical discussion: Leiomyosarcoma, a rare malignant tumor arising from stomach involves commonly gastric body followed by antrum and fundus. Imaging including CECT and tissue diagnosis including immunohistochemistry (positive for α-SMA, desmin, calponin, h-caldesmon, or smoothelin) have been mainstay for definitive diagnosis. The standard treatment for leiomyosarcoma of stomach is complete surgical resection of tumor because it has malignant potential and does not respond to targeted treatment with a tyrosine kinase inhibitor. The type of surgery depends on the size and localization of the tumor. Conclusions: Early diagnosis with proper imaging, immunohistochemistry, and biopsy play important role in differentiating gastric leiomyosarcoma from gastrointestinal stromal tumor. Surgical resection is the mainstay of treatment.