Epithelioid leiomyosarcoma arising from the ocular region in a dog.

A 16-year-old male mixed-breed dog presented with a mass with hemorrhage at the right conjunctiva. Five months after the initial visit, the right eye protruded and had a firm and irregular mass measuring approximately 1.00 cm in diameter with conjunctival hemorrhage. Microscopically, the mass was comprised polygonal or round tumor cells with distinct cell borders arranged in a nested and diffuse pattern. The tumor cells had round-to-oval fine hyperchromatic nuclei containing distinct multiple nucleoli and abundant eosinophilic or pale cytoplasm. Multiple giant cells were frequently observed. The mitotic index was 12.60/high power field. Extensive necrosis, hemorrhage and part of the cord-like and papillary epithelioid cells were observed in the intra-tumor tissue. Immunohistochemically, the tumor cells were positive for vimentin and α-smooth muscle actin and negative for cytokeratin, desmin and PNL2. On the other hand, the cord-like and papillary epithelioid cells were positive for vimentin, S100 and neuron-specific enolase. The tumor was diagnosed as an epithelioid leiomyosarcoma. This case considered to have occurred in the ocular region, although the ocular structure was destroyed.

Uterine Leiomyosarcoma in a 22-Year-Old Young Woman: A Case Report.

Leiomyosarcoma is one of the rarest types of gynecological cancer. It is a relatively rare condition that affects young women. The most frequent symptom of this disease is vaginal bleeding. The primary treatment for localized disease is still surgical intervention. It is widely recognized that leiomyosarcoma has a poor prognosis, with reduced survival rates and a high likelihood of early recurrence. This report presents a case of uterine leiomyosarcoma in a 22-year-old female patient. Following a total hysterectomy and bilateral salpingo-oophorectomy, the diagnosis of leiomyosarcoma was confirmed through a histopathological examination of the surgical specimen.

Rare case of giant ischiorectal leiomyosarcoma.

INTRODUCTION: Leiomyosarcoma is a rare type of soft tissue sarcoma especially giant ones (size >5 cm). It is usually arises from the uterus and retroperitoneum. Ischiorectal space is not a common site for primary leomyosarcoma. There are only few case reports about Ischiorectal leiomyosarcoma. PRESENTATION OF CASE: We present a case of 25 yrs. old female patient, who presented with a swelling on left buttock. It measures 9 by 9.5 by 18 cm on MRI. Complete tumor excision done and immunohistochemistry tests confirmed the diagnosis of leiomyosarcoma. DISCUSSION: Due to its rarity, unusual site and large size leiomyosarcoma poses a great challenge for a surgeon both to diagnose and manage it. Early diagnosis and management improves prognosis and decreases recurrence rate because sarcomas have high recurrence rate. It is highly recommended to have multidisciplinary approach. CONCLUSION: The case report highlights on both diagnostic and therapeutic challenges associated with giant leiomyosarcoma. There are no strict guidelines to follow when managing this type of cases but rather approach has to be individualized. Since there are only few case reports on this specific pathology we advocate for surgeons to share their experience on similar cases.

A rare case of leiomyosarcoma with a pleomorphic component of the sigmoid colon.

A 66-year-old man presented to our institution with a positive fecal occult blood test and lower abdominal pain. Although a tumor was found in the sigmoid colon, biopsy and imaging studies failed to enable the diagnosis of the cancer, and the patient underwent surgery for treatment and diagnosis. The tumor had two distinct areas with differing features shown both histopathologically and on imaging; it was thus diagnosed as a leiomyosarcoma of the sigmoid colon with a pleomorphic component. Here, we describe a rare case of leiomyosarcoma of the sigmoid colon with a pleomorphic component. There are no reports of leiomyosarcoma with pleomorphic components arising in the colon in the literature; thus, the recurrence and metastatic characteristics are unknown. Therefore, accumulating cases in the literature may provide valuable insights into diagnosing and treating these rare tumors.

Comprehensive Review of Uterine Leiomyosarcoma: Pathogenesis, Diagnosis, Prognosis, and Targeted Therapy.

Uterine leiomyosarcoma (uLMS) is the most common subtype of uterine sarcomas. They have a poor prognosis with high rates of recurrence and metastasis. The five-year survival for uLMS patients is between 25 and 76%, with survival rates approaching 10-15% for patients with metastatic disease at the initial diagnosis. Accumulating evidence suggests that several biological pathways are involved in uLMS pathogenesis. Notably, drugs that block abnormal functions of these pathways remarkably improve survival in uLMS patients. However, due to chemotherapy resistance, there remains a need for novel drugs that can target these pathways effectively. In this review article, we provide an overview of the recent progress in ascertaining the biological functions and regulatory mechanisms in uLMS from the perspective of aberrant biological pathways, including DNA repair, immune checkpoint blockade, protein kinase and intracellular signaling pathways, and the hedgehog pathway. We review the emerging role of epigenetics and epitranscriptome in the pathogenesis of uLMS. In addition, we discuss serum markers, artificial intelligence (AI) combined with machine learning, shear wave elastography, current management and medical treatment options, and ongoing clinical trials for patients with uLMS. Comprehensive, integrated, and deeper insights into the pathobiology and underlying molecular mechanisms of uLMS will help develop novel strategies to treat patients with this aggressive tumor.

Diagnostic and therapy of an early-stage primary leiomyosarcoma of the sigmoid colon: A case report.

Key Clinical Message: Primary leiomyosarcoma of the colon is a very rare tumor entity. Because of unspecific findings, diagnostic can be challenging. Most cases are diagnosed in advanced stages with poor overall survival. Unclear histological findings of smooth muscle cell tissue in colon biopsies together with a tumor of the colon wall in computed tomography (CT) imaging should lead to the differential diagnosis of primary colonic leiomyosarcoma and further diagnostic procedures. Abstract: Primary colonic leiomyosarcoma is an extremely rare tumor entity arising from smooth muscle cells in the colon wall. Only 0.1% of all colorectal malignancies are leiomyosarcomas. Most patients are diagnosed in advanced disease stages. The overall survival rates are low, and recurrence rates are high. Only few data regarding the outcome in localized early disease stages are available. We report the case of an early-stage primary leiomyosarcoma of the sigmoid colon treated with surgical resection. We present the case of a 53-year-old male patient who underwent a colonoscopy due to intermittent rectal bleeding. Colonoscopy revealed an intraluminal polypoid growing tumor in the sigmoid colon. A biopsy was performed with inconclusive histological results. A CT scan revealed a process in the sigmoid colon with wall thickening; there was no evidence of metastatic lesions. After laparoscopic oncological resection of the sigmoid colon, histological examination surprisingly revealed a localized high-grade leiomyosarcoma. Primary leiomyosarcoma of the colon is a rare tumor entity and diagnostic can be challenging. Only a few patients with colonic leiomyosarcoma diagnosed in localized early disease stages and treated with complete surgical resection have been reported in the literature. These patients seem to have a better prognosis with longer overall survival. Because of unspecific diagnostic findings and the lack of symptoms in early disease stages, interdisciplinary collaborations between gastroenterologists, radiologists, pathologists, and surgeons are crucial for early diagnosis and treatment.

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.

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.

Myxoid leiomyosarcoma of the oviduct and uterus in a Cockatiel (Nymphicus hollandicus).

An 11-year-old female cinnamon cockatiel (Nymphicus hollandicus) was presented with a coelomic distention. Dystocia was suspected, given its previous history of a calcium-deficient diet and multiple instances of nonobstructive dystocia. Exploratory coeliotomy revealed a large intraluminal mass extending through the magnum to the uterus (shell gland). Metastasis and multiorgan involvement were not seen. Histopathologically, malignant and invasive fascicles of spindle cells were associated with abundant myxoid matrix and hypocellular areas. Multinucleation, bizarre cells and atypical mitotic figures were prominent. Masson's trichrome staining verified the muscular origin, and the myxoid matrix was demonstrated utilizing Alcian blue. The neoplastic cells exhibited alpha-smooth muscle actin and desmin immunoreactivity and were negative for vimentin. Thus, the patient was diagnosed with oviductal and uterine myxoid leiomyosarcoma (LMS). The patient survived 34 days post-surgery before death associated with suspected enteritis. Myxoid LMS is an extremely rare neoplasm in animals. To our knowledge, myxoid LMS has not been reported previously in pet birds.

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.

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.

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.

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.

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).

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.

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.

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.

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.