Case Of Leiomyosarcoma Research Articles

Leiomyosarcoma of the inferior vena cava: case report

Introduction and Importance: Leiomyosarcomas of the inferior vena cava are exceedingly rare tumors, presenting diagnostic dilemmas due to their nonspecific clinical features. This case report aims to shed light on the challenges associated with diagnosing and managing such tumors, emphasizing the importance of a multidisciplinary approach. This article uses SCARE2023 criteria as a framework to sort out a case report in order[1]. Case Presentation: We present a case of leiomyosarcoma of the inferior vena cava in a patient presenting with abdominal pain. Initial clinical signs, supported by computed tomography (CT) and magnetic resonance imaging (MRI), led to the suspicion of the tumor. Histological analysis confirmed the diagnosis, prompting surgical intervention. Clinical Discussion: The diagnosis of tumors within the inferior vena cava is often challenging due to their nonspecific presentation. Imaging modalities such as CT and MRI play an important role in describing the extent of the tumor and guiding surgical planning. Histological analysis remains supreme for definitive diagnosis. Surgical resection, aimed at complete excision, is the keystone of treatment to minimize the risk of recurrence. Conclusion: Leiomyosarcomas of the inferior vena cava pose diagnostic and therapeutic challenges due to their rarity and nonspecific clinical presentation. A comprehensive approach integrating clinical assessment, imaging, and histological analysis is essential for accurate diagnosis and optimal management. Multidisciplinary collaboration is key for navigating the complexities associated with these rare vascular neoplasms, ensuring positive patient outcomes.

Ultrasonographic Performance and Clinical Analysis of Ultrasound in the Diagnosis of Uterine Fibroids

Objective: To assess ultrasound’s ultrasonographic performance and clinical value in diagnosing uterine fibroids. Methods: 60 patients with suspected uterine fibroids admitted to the hospital between March 2021 and March 2024 were selected for this study. Abdominal B-mode ultrasound was used as the gold standard to detect pathological findings, and the results were compared and analyzed. Results: 54 patients were diagnosed after the gold standard, and the results of abdominal B-mode ultrasound were positive predictive value: 96.29%; negative predictive value: 66.67%; accuracy: 93.33%; sensitivity: 96.29%; specificity: 66.67%. Among 60 patients examined by B-mode ultrasound, there were a total of 54 patients who were diagnosed by the gold standard, and 19 cases of uterine leiomyosarcoma were confirmed by the detection of uterine leiomyosarcoma by the gold standard, and B-mode ultrasound (94.73%); 21 cases of cervical leiomyoma were confirmed by gold standard test and 20 cases of cervical leiomyoma were diagnosed by ultrasound (95.24%); 14 cases of mixed leiomyoma were confirmed by gold standard test and 14 cases of mixed leiomyoma were diagnosed by ultrasound (100.00%). Conclusion: Using abdominal ultrasound to examine patients with uterine fibroids can significantly improve the efficiency of diagnosis and treatment and provide a strong scientific basis for subsequent treatment decisions.

Rare case of leiomyosarcoma of the urinary bladder in ayoung man

Leiomyosarcomas are rare, highly aggressive tumors of the urinary bladder. With approximately 200 cases reported in the literature, there is limited data on the prognosis and treatment of these neoplasms. Curative treatment approaches are primarily characterized by radical surgery, especially radical cystectomy. However, this procedure is associated with significant impairments in the quality of life for patients. This circumstance forms the basis for considering acurative treatment approach with partial cystectomy for a19-year-old man with leiomyosarcoma of the urinary bladder.

A case of leiomyosarcoma of the kidney

A case of leiomyosarcoma of the kidney

Exceptional Location of a Pleomorphic Leiosarcoma: Clinical Case

Pleomorphic leiomyosarcoma is a rare entity that is part of the pleomorphic sarcomas with a complex genome with a high metastatic capacity and a usually poor prognosis. The most common location remains the lower extremities but other sites have been reported in the literature, particularly in the chest wall. Leiomyosarcoma (LMS) is an exceptionally rare tumor in the chest wall. Classical LMS may exhibit focal areas of pleomorphic cells; however, the coexistence of a large pleomorphic component allows the tumor to be designated as pleomorphic leiomyosarcoma (PLMS). The first cases of pleomorphic leiomyosarcoma were reported in the literature by Brooks in 1985. The pleomorphic component of these tumors is often heterologous with either cartilaginous differentiation; bony or rhabdomyosarcomatous. Since pleomorphic myogenic sarcomas, including high-grade leiomyosarcomas and rhabdomyosarcomas, have a poorer prognosis hence the importance of an accurate and valid histological diagnosis. The thoracic location of a leiomyosarcoma remains exceptional or even extremely rare and management requires multidisciplinary consultation involving the surgeon; the oncologist and the radiotherapist with additional molecular study to properly detect genetic anomalies in order to consider possible targeted therapy.

Gastric Leiomyosarcoma in Post-Gastrointestinal Stromal Tumor Era: Revisit.

Primary gastric leiomyosarcoma is an exceptionally rare disease. This review covers 41 post-gastrointestinal stromal tumor (GIST) era gastric leiomyosarcoma cases that are supported by immunohistochemistry markers. Other spindle cell lesions are also excluded through histological and immunohistochemistry evaluations. The patients range from 3 to 82 years old, with an average age of 54.6 years. The male-to-female ratio is 1.4:1, from diverse geographic areas. Patients may experience abdominal symptoms, and tumor sizes vary between 1cm and 22cm. Morphologically, tumors originate from the muscularis propria or the muscularis mucosae, well-circumscribed with spindle cells arranged in fascicule. Tumoral cells exhibit positivity for smooth muscle markers while being negative for GIST markers and others. The mitotic index ranges from 2 to 500/50 high power field. Ki-67 index varies from 15% to 70%. Management typically involves gastrectomy and other appropriate treatments, with tumor recurrence being uncommon. 56% of patients are alive, with 5 patients dying from this disease. Statistical analyses conducted on post-GIST era cases reveal that a mitotic index of ≥100/50 high power field, tumor recurrence, metastasis, or positive lymph nodes significantly correlate with prognosis.

A rare case of Leiomyosarcoma of pancreatic head

A rare case of Leiomyosarcoma of pancreatic head

A Case of Leiomyosarcoma of Vaginal Vault in a Post-hysterectomy Patient

A Case of Leiomyosarcoma of Vaginal Vault in a Post-hysterectomy Patient

Loss of the DNA Repair Gene RNase H2 Identifies a Unique Subset of DDR-Deficient Leiomyosarcomas.

Targeting the DNA damage response (DDR) pathway is an emerging therapeutic approach for leiomyosarcoma (LMS), and loss of RNase H2, a DDR pathway member, is a potentially actionable alteration for DDR-targeted treatments. Therefore, we designed a protein- and genomic-based RNase H2 screening assay to determine its prevalence and prognostic significance. Using a selective RNase H2 antibody on a pan-tumor microarray (TMA), RNase H2 loss was more common in LMS (11.5%, 9/78) than across all tumors (3.8%, 32/843). In a separate LMS cohort, RNase H2 deficiency was confirmed in uterine LMS (U-LMS, 21%, 23/108) and soft-tissue LMS (ST-LMS; 30%, 39/102). In the TCGA database, RNASEH2B homozygous deletions (HomDels) were found in 6% (5/80) of LMS cases, with a higher proportion in U-LMS (15%; 4/27) compared with ST-LMS (2%; 1/53). Using the SNiPDx targeted-NGS sequencing assay to detect biallelic loss of function in select DDR-related genes, we found RNASEH2B HomDels in 54% (19/35) of U-LMS cases with RNase H2 loss by IHC, and 7% (3/43) HomDels in RNase H2 intact cases. No RNASEH2B HomDels were detected in ST-LMS. In U-LMS patient cohort (n = 109), no significant overall survival difference was seen in patients with RNase H2 loss versus intact, or RNASEH2B HomDel (n = 12) versus Non-HomDel (n = 37). The overall diagnostic accuracy, sensitivity, and specificity of RNase H2 IHC for detecting RNA-SEH2B HomDels in U-LMS was 76%, 93%, and 71%, respectively, and it is being developed for future predictive biomarker driven clinical trials targeting DDR in U-LMS.

Abstract 4808: Small intestinal leiomyosarcoma in a young adult

Abstract Objectives: • Outline the epidemiology of leiomyosarcoma•Identify the immunohistochemistry of a leiomyosarcoma Introduction: Primary small intestinal malignancies are rare malignancies as compared with other malignancies of the gastrointestinal tract. Here, we present a case of a jejunal leiomyosarcoma in a young adult. Case Presentation: A 23-year-old Caucasian male with GERD presented with five months of abdominal pain, intermittent nausea with non-bloody emesis, and a 20-pound weight loss. Presenting vital signs were within normal limits. Physical exam revealed conjunctival pallor with epigastric tenderness to palpation. Serology demonstrated iron deficiency anemia, hemoccult positive stool, negative CEA, CA19-9, and AFP tumor markers. HIV antigen antibody and EBV-PCR were non-reactive. CT of the abdomen and pelvis revealed markedly thickened heterogenous jejunal loop in the left hemiabdomen with associated mesenteric adenopathy and small volume pelvic ascites. EGD with push enteroscopy revealed a single, malignant appearing and ulcerated mass in the proximal jejunum. PET-CT showed a hypermetabolic, multilobulated, ill-defined mass involving multiple loops of jejunum. He underwent exploratory laparotomy with resection of a proximal jejunal mass (10x8 x7 cm), mesenteric lymphadenectomy, and a jejunojejunostomy creation. Pathology demonstrated leiomyosarcoma (pT2aN0MoG3), and he was initiated on cyclophosphamide and doxorubicin. Discussion: Primary small intestinal malignancies are rare malignancies with an incidence of less than 5% of all GI malignancies. 56 cases of GI leiomyosarcoma in adults were identified between 2000-2012 with a peak incidence between 55-59 years of age (Aggarrwal 2012). Leiomyosarcomas are soft tissue sarcomas arising from bones, tendons, blood vessels, or muscles with a spectrum of disease ranging from low-grade cutaneous lesions to aggressive lesions of internal organs with significant potential for metastasis (Pipe 2002). Diagnosis is based on immunohistochemical positivity for SMA, desmin, and h-caldesmon, and negativity for CD117, CD34, and DOG1.1, notably lacking KIT and PDGFRA mutations (Aggarwal 2012). Treatment involves surgical resection and a cost benefit analysis of adjuvant chemotherapy with (Reynolds, 2014). This case of a 23 year old, without significant risk factors, diagnosed with a jejunal leiomyosarcoma presents as one of the youngest cases ever to be reported and emphasizes the importance of recognizing the cancer in a broader range of the adult population. Citation Format: Alyssa A. Guo, Blake H. Bentley, Abigail L. Ellington, Haiyan Lu, William C. Lippert. Small intestinal leiomyosarcoma in a young adult [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 4808.

A Complex Case: Solid Unilateral Ovarian Mucinous Carcinoma Masquerading as Leiomyosarcoma in Postmenopausal Women

Introduction:: Ovarian cancers are the third most common gynecological and 8th most common cancer in women, associated with high mortality rates globally. Case Presentation:: A 60-year-old postmenopausal woman presented to the Gynecological outpatient department with complaints of pain in the abdomen for 2-3 months and loss of appetite with weight loss for 1-2 months. She was diagnosed as a case of uterine leiomyosarcoma or left adnexal mass on ultrasound and MRI. Intra-operatively, she was diagnosed with a unilateral left ovarian malignant mass, predominantly solid in consistency with metastasis to the left fallopian tube, uterus, cervical stroma, and omentum. The right ovary and tube were healthy. On histopathological examination, the final diagnosis of primary mucinous ovarian carcinoma was made. The patient tolerated staging laparotomy (total abdominal hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy) well and was started on six cycles of adjuvant chemotherapy with Paclitaxel and Carboplatin injections along with Bevacizumab. Conclusion:: Primary mucinous ovarian carcinomas are rare and distinct subtypes of epithelial ovarian carcinomas. Due to their strong resemblance to other ovarian tumors and leiomyosarcoma, their diagnosis becomes challenging. Hence, it is crucial to consider leiomyosarcomas in the differential diagnosis of ovarian masses, especially in postmenopausal women.

Uterine Leiomyosarcoma Associated With Perivascular Epithelioid Cell Tumor: A Phenomenon of Differentiation/Dedifferentiation and Evidence Suggesting Cell-of-Origin.

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor thought to originate from perivascular epithelioid cells (PECs). The normal counterpart to PEC, however, has not been identified in any human organ, and the debate as to whether PEComa is related to smooth muscle tumors has persisted for many years. The current series characterizes 4 cases of uterine leiomyosarcoma (LMS) coexisting with PEComas. All cases exhibited an abrupt transition from the LMS to PEComa components. The LMS component displayed typical spindled morphology and fascicular growth pattern and was diffusely positive for desmin and smooth muscle myosin heavy chain, completely negative for HMB-45 and Melan A, and either negative or had focal/weak expression of cathepsin K and GPNMB. In contrast, the PEComa tumor cells in case 1 contained glycogen or lipid-distended cytoplasm with a foamy appearance (low grade), and in cases 2, 3, and 4, they displayed a similar morphology characterized by epithelioid cells with eosinophilic and granular cytoplasm and high-grade nuclear atypia. Different from the LMS component, the epithelioid PEComa cells in all cases were focally positive for HMB-45, and diffusely immunoreactive for cathepsin K and GPNMB. Melan A was focally positive in cases 1 and 3. Loss of fumarate hydratase expression (case 1) and RB1 expression (cases 2, 3, 4) was identified in both LMS and PEComa components, indicating that they are clonally related. In addition, both components showed an identical TP53 p.R196* somatic mutation and complete loss of p53 and ATRX expression in case 2 and complete loss of p53 expression in case 3. We hypothesize that LMSs containing smooth muscle progenitor cells may give rise to divergent, lineage-specific PEComatous lesions through differentiation or dedifferentiation. While we do not dispute the recognition of PEComas as a distinct entity, we advocate the hypothesis that modified smooth muscle cells represent the origin of a subset of PEComas, and our case series provides evidence to suggest this theory.

Pathological Study of Rare Case of Duodenal Leiomyosarcoma in a Labrador Dog

The leiomyosarcoma is a tumor of mesenchymal origin of smooth, malignant, slow-growing muscles. The present study reports a case of duodenal leiomyosarcoma in Labrador dog. A 6 year old black female Labrador dog with history off feed, vomition and melena brought for necropsy examination. Gross lesions revealed tennis ball size tumorus mass at the initial portion of duodenum resulting into the obstruction of intestinal lumen. Microscopically, duodenum with tumours mass revealed smooth muscle bundles arranged in all the directions having elongated blunt ended nuclei along with numerous blood vessels and mitotic figure which is indicative of leiomyosarcoma.

Development and external validation of a machine learning model for prediction of survival in extremity leiomyosarcoma

PurposeMachine learning (ML) models have been used to predict cancer survival in several sarcoma subtypes. However, none have investigated extremity leiomyosarcoma (LMS). ML is a powerful tool that has the potential to better prognosticate extremity LMS. MethodsThe Surveillance, Epidemiology, and End Results (SEER) database was queried for cases of histologic extremity LMS (n = 634). Patient, tumor, and treatment characteristics were recorded, and ML models were developed to predict 1-, 3-, and 5-year survival. The best performing ML model was externally validated using an institutional cohort of extremity LMS patients (n = 46). ResultsAll ML models performed best at the 1-year time point and worst at the 5-year time point. On internal validation within the SEER cohort, the best models had c-statistics of 0.75–0.76 at the 5-year time point. The Random Forest (RF) model was the best performing model and used for external validation. This model also performed best at 1-year and worst at 5-year on external validation with c-statistics of 0.90 and 0.87, respectively. The RF model was well calibrated on external validation. This model has been made publicly available at https://rachar.shinyapps.io/lms_app/ ConclusionsML models had excellent performance for survival prediction of extremity LMS. Future studies incorporating a larger institutional cohort may be needed to further validate the ML model for LMS prognostication.

A Case of Gastric Leiomyosarcoma Completely Resected by Endoscopic Submucosal Dissection after a Precise Preoperative Diagnosis.

A 68-year-old woman was diagnosed with leiomyosarcoma (LMS) based on preoperative biopsy of the gastric body. As tumor invasion confined to the submucosa with no breaking of the submucosal layer was confirmed on endoscopic ultrasonography (EUS), the patient underwent endoscopic submucosal dissection (ESD) for gastric LMS, resulting in complete tumor resection. No apparent recurrence was observed in the 2.5 years after treatment. This is an extremely rare case of gastric LMS that underwent ESD after a precise preoperative diagnosis, with no signs of recurrence after treatment. ESD may be an acceptable option for gastric LMS when EUS findings allow this treatment method.

Kiosk 4Q-TA-02 - A Case of Pleomorphic Leiomyosarcoma Mimicking Thrombus

Kiosk 4Q-TA-02 - A Case of Pleomorphic Leiomyosarcoma Mimicking Thrombus

A RARE CASE OF CERVICAL LEIOMYOSARCOMA: CASE REPORT AND COMPREHENSIVE MANAGEMENT AT PHC SURABAYA HOSPITAL

Introduction: Cervical cancer is a malignant neoplasm that grows from cells originating from the cervix in the uterus. Leiomyosarcoma is a type of neck cancer that is relatively rare, data from the USA shows an incidence rate of only 0.35-0.64 per 100,000 women. Objective: This paper reports on the management of leiomyosarcoma cases at PHC Surabaya Hospital. Case Report: A 45-year-old female patient complained of continuous menstruation. An ultrasound was performed, and a biopsy was planned due to the mass found during the ultrasound. The biopsy showed cervical malignancy. A total hysterectomy, and bilateral oophorectomy, were performed and a PA examination was carried out on the uterine samples resulting from the operation the results, were leiomyosarcoma, high-grade, cervix, invasion in the stroma of the parametrium, vaginal cuff and both sides of the lymph nodes were free of tumor (pt1b3 n0mx) Conclusion: The patient was treated for 3 days at PHC Hospital after surgery and went home without complications from the surgical wound. The patient returned to control after 6 months with signs of tumor recurrence and negative metastases.

Rate of Leiomyosarcomas during Surgery for Uterine Fibroids: 8-Year Experience of a Single Center.

The aim of this study is to investigate the prevalence of occult malignant mesenchymal tumors in patients operated on for uterine fibroids in relation to the surgical approach and type of operation. A retrospective review of all patients that underwent surgery for uterine fibroids (January 2011-December 2018) at the 1st Department of Obstetrics & Gynecology at "Papageorgiou" Hospital. The surgical approach and clinicopathological characteristics were analyzed. A total of 803 patients were operated on: 603 (75.1%) with laparotomy, 187 (23.3%) laparoscopically, and 13 (1.6%) vaginally. Furthermore, 423 (52.7%) patients underwent hysterectomy and 380 (47.3%) myomectomies. Laparoscopy and myomectomy were offered to younger patients with fewer smaller uterine fibroids and were associated with statistically significant shorter hospitalization. The pathological reports revealed: 690 (86%) benign leiomyomas, 32 (4%) cellular leiomyomas, 29 (3.6%) degenerated leiomyomas, 22 (2.7%) adenomyomas, 18 (2.2%) atypical-bizarre leiomyomas, 1 (0.1%) STUMP, 5 (0.65%) endometrial stromal sarcomas, and 6 (0.75%) cases of leiomyosarcomas (LMS). All LMS were preoperatively characterized as suspicious and underwent abdominal hysterectomy. Morcellation was offered in two cases of atypical leiomyomas, with no morcellation-associated complication. Laparoscopy as a valuable surgical approach for young patients with fewer in number and smaller in size fibroids is associated with shorter hospitalization. The risk of unintended morcellation of LMS seems to be very low and can be reduced with careful preoperative work-up but not eliminated.

LARYNGEAL LEIOMYOSARCOMA-A RARE CASE REPORT

Mesenchymal neoplasms are sporadic in the larynx accounting for about 1% of all laryngeal malignancies. Leiomyosarcoma is a high-grade tumor of smooth muscle fibres which is very rarely seen in the larynx. The present case report aims to discuss a rare case of laryngeal leiomyosarcoma with special emphasis on immunohistochemical techniques in arriving at the diagnosis.

Uterine Epithelioid Leiomyosarcoma Arising Within Leiomyoma in A Postmenopausal Woman: A Case Report

Uterine leiomyosarcoma is a malignant smooth muscle tumour of the uterus. It is rare and accounting for less than 2% of cases in malignant gynaepathology. To date, only a few reported cases of leiomyosarcoma arising from leiomyoma documented in the literature. We shared an uncommon occurrence of leiomyosarcoma arising from leiomyoma. Presented herein is a case of a ‘rare epithelioid subtype’ of leiomyosarcoma arising from a leiomyoma in a postmenopausal woman. We highlighted the importance of recognizing the possibilities of this event to allow for a timely diagnosis of leiomyosarcoma and to provide insights on management of patients presented with clinically presumed fibroid.