Safety and efficacy of percutaneous image-guided ablation for soft tissue sarcoma metastases to the liver.

PURPOSE: To evaluate outcomes following percutaneous image-guided ablation of soft tissue sarcoma metastases to the liver. MATERIALS AND METHODS: A single-institution retrospective analysis of patients with a diagnosis of metastatic soft tissue sarcoma who underwent percutaneous image-guided ablation of hepatic metastases between January 2011 and December 2021 was performed. Patients with less than 60 days of follow-up after ablation were excluded. The primary outcome was local tumor progression-free survival (LPFS). Secondary outcomes included overall survival, liver-specific progression-free survival. and chemotherapy-free survival. RESULTS: Fifty-five patients who underwent percutaneous ablation for 84 metastatic liver lesions were included. The most common histopathological subtypes were leiomyosarcoma (23/55), followed by gastrointestinal stromal tumor (22/55). The median treated liver lesions was 2 (range, 1-8), whereas the median size of metastases were 1.8 cm (0.3-8.7 cm). Complete response at 2 months was achieved in 90.5% of the treated lesions. LPFS was 83% at 1 year and 80% at 2 years. Liver-specific progression-free survival was 66% at 1 year and 40% at 2 years. The overall survival at 1 and 2 years was 98% and 94%. The chemotherapy-free holiday from the start of ablation was 71.2% at 12 months. The complication rate was 3.6% (2/55); one of the complications was Common Terminology Criteria for Adverse Events grade 3 or higher. LPFS subgroup analysis for leiomyosarcoma versus gastrointestinal stromal tumor suggests histology-agnostic outcomes (2 years, 89% vs 82%, p = .35). CONCLUSION: Percutaneous image-guided liver ablation of soft tissue sarcoma metastases is safe and efficacious.

En Bloc Inferior Vena Cava (IVC) Resection Without Reconstruction With Right Hepatectomy and Right Nephrectomy for a Large IVC Leiomyosarcoma.

BACKGROUND: Radical resection remains the only potential cure in the management of inferior vena cava (IVC) leiomyosarcomas with multivisceral resections often needed (Borghi et al. in J Cardiovasc Surg (Torino) 63:649-663, 2022). This video describes the technical nuances of surgical resection of a large retrohepatic IVC leiomyosarcoma. PATIENT AND METHODS: Computed tomography of a 60-year-old woman revealed a 12 × 12 × 9.5 cm mass in the right suprarenal region infiltrating the IVC with intraluminal extension up to the hepatic venous confluence. The mass involved the right hepatic vein with infiltration of segment 7 of the liver and splaying of the right portal vein. Robust lumbar venous drainage from the infratumoral IVC was seen. En bloc IVC resection without reconstruction along with a right hepatectomy and right nephrectomy was performed via a right thoracoabdominal approach. RESULTS: After a Catell-Braasch maneuver, the surgery can be broadly divided into four major steps: (1) Right retroperitoneal mobilization of the tumor and right kidney with infratumoral IVC control, (2) mobilization of the right liver with suprahepatic IVC control, (3) division of the right portal structures with right hepatectomy, and (4) en bloc resection of the IVC tumor. Reconstruction of the IVC was not performed owing to the presence of venous collaterals (Langenbecks et al. in Arch Surg 407:1209-1216, 2022). Final histopathology showed a high-grade leiomyosarcoma with histologic organ invasion in the liver and right kidney with resected margins free of the tumor (R0). CONCLUSIONS: Meticulous preoperative planning and expertise in liver resection and retroperitoneal surgeries facilitates such radical yet safe multivisceral resection for a large retrohepatic IVC leiomyosarcoma without the need for a cardiopulmonary bypass.

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.

Successful clinical approach to the metastatic uterine leiomyosarcoma to the epicardium-a case report.

BACKGROUND: Uterine leiomyosarcoma is a rare and aggressive tumour with a poor prognosis. Its metastases to the heart are even rarer, especially to the epicardium. The majority of reported cardiac metastases of uterine leiomyosarcoma were in the cardiac chambers or intramyocardial. Surgical resection of the uterine leiomyosarcoma in the early stages is the only definitive treatment for this disease. However, in the cases of cardiac metastasis, surgery is recommended only in emergencies and patients with expected beneficial outcomes. CASE PRESENTATION: Our patient was a 49-year-old female referred to the Department of Cardiac Surgery for scheduled surgery of pericardial neoplasia. The patient underwent a hysterectomy and adnexectomy three years prior owing to the uterine leiomyosarcoma. A regular follow-up magnetic resonance imaging of the abdomen and pelvis discovered neoplasia in the diaphragmic portion of the pericardium. No other signs of primary disease relapse or metastases were found. The patient was asymptomatic. The multidisciplinary team concluded that the patient is a candidate for surgery. Surgery included diastolic cardiac arrest achievement and resection of the tumour. Macroscopically, a parietal layer of the pericardium was completely free from the tumour that invaded only the apical myocardium of the left ventricle. Completed histopathology confirmed the diagnosis of leiomyosarcoma of the uterine origin. Three months after surgery, the patient received adjuvant chemotherapy with doxorubicin and dacarbazine. One year after surgery, there are no signs of new metastases. CONCLUSIONS: Strict surveillance of patients with uterine leiomyosarcoma after successful treatment of the early stage of the disease is of utmost importance to reveal metastatic disease to the heart in a timely manner and to treat it with beneficial outcomes. Surgery with adjuvant chemotherapy might be a good approach in patients with a beneficial prognosis. From a surgical point of view, it is challenging to assess the appropriate width of the resection edges to be radical enough and, at the same time, sufficiently conservative to ensure the satisfactory postoperative function of the remaining myocardium and avoid repetitive tumour growth. Therefore, intraoperative histopathology should always be performed.

Giving birth after fertility-sparing treatment for primary leiomyosarcoma of the fallopian tube.

Primary leiomyosarcoma of the fallopian tube (PLFT) is an extremely rare gynecological malignancy that has only been described in case reports. Fertility-sparing treatment for PLFT has not been reported previously. A 24-year-old nulligravida woman was diagnosed with stage IC1 PLFT in the right fallopian tube after experiencing right lower quadrant pain for 2 weeks. She underwent laparoscopic right salpingectomy to preserve fertility followed by adjuvant chemotherapy with gemcitabine/docetaxel. She subsequently became pregnant spontaneously, delivering a term baby 27 months after treatment. This appears to be the only report of the use of fertility-preserving treatment for PLFT. The success of the treatment provides valuable information on the preservation of fertility in young women with PLFT.

Findings of an Extraluminal Leiomyosarcoma of the Urinary Bladder in a Dog.

A 9 yr old male miniature poodle presented with acute diarrhea, vomiting, and a distended abdomen. A large and firm mass was palpated in the caudal abdomen. Radiography showed a large soft-tissue mass in the mid ventral abdomen. The mass was mildly contrast-enhancing and in contact with the right cranial aspect of the bladder on computed tomography. The mass was heterogeneous with minimal blood flow on Doppler examination. Surgery confirmed its origin of the urinary bladder, and it was diagnosed leiomyosarcoma on pathology. This is the first report of extraluminal leiomyosarcoma of the bladder wall with imaging characteristics using various modalities.

[A rare malignant tumor of the renal vein]. / Une tumeur maligne rare de la veine rénale.

The renal vein is an exceptional location for leiomyosarcoma, an aggressive malignant tumor of smooth-muscle origin with a poor prognosis. We report the case of a 55-year-old female patient who consulted for left flank pain that had been present for 6 months. A CT scan revealed a 9.4cm left retroperitoneal mass in contact with the psoas muscle, left kidney, stomach, spleen, left colon and extending up to the pancreas, raising the suspicion of a tumour originating in the retroperitoneal tissues. A biopsy revealed a smooth-muscle cell tumour with a degree of malignancy difficult to define. The patient underwent a monobloc left compartmentectomy, which led to the diagnosis of leiomyosarcoma of the left renal vein. A review of the literature on these rare tumours in this location is presented.

[A Case of Resection of Leiomyosarcoma Suspected to Originate from the Right Ovarian Vein].

We present a 58-year-old female patient who underwent resection of a leiomyosarcoma arising from the right ovarian vein. She was referred to our hospital because of lower abdominal pain that had been present for 1 month prior to the visit. Ultrasound examination revealed a well-defined, smooth, lobulated, highly vascular mass(57 mm)adjacent to the distal portion of the duodenum. Contrast-enhanced computed tomography revealed the contrast enhancement mass (60 mm)located surround the right ovarian vein. In abdominal magnetic resonance image examination, the mass exhibited isointense signal on T1-weighted images, high signal on T2-weighted images, and restricted diffusion on diffusion- weighted images. We suspected primary leiomyosarcoma of the ovarian vein and proceeded with surgical intervention. On intraoperative findings, the mass was in contact with the duodenum and the inferior vena cava but dissection was easily achieved. We excised the mass together with the right ovarian vein. Pathological findings showed the mass was composed of proliferating spindle-shaped cells arranged in bundles. Some areas showed polygonal nuclear atypia and abnormal mitotic figures. Additional immunostaining showed positive for α-SMA, caldesmon, calponin, and negative for desmin, CD34, CKA1/AE3, S100. Based on the intraoperative findings, we diagnosed it as leiomyosarcoma arising of the right ovarian vein.

[Clinical observation of 16 years of use of a domestic voice prosthesis without replacement after complete removal of the larynx]. / Klinicheskoe nablyudenie 16-letnei ekspluatatsii otechestvennogo golosovogo proteza bez zameny posle polnogo udaleniya gortani.

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.

Pulmonary leiomyosarcoma arising in pulmonary hamartoma: an exceptional occurrence in a rare tumor.

A solitary peripheral lung nodule was found in the left lung of a 52-year-old man. It was located in the lower lobe and measured 18.5 cm of major axis on chest computed tomography. A tru-cut core biopsy was obtained and a proliferation of bland, monomorphic, spindle cells in interlacing fascicles was observed. Accordingly, a surgical resection of the neoplasm was subsequently carried out. Macroscopically, the tumor appeared as a well-circumscribed nodule with a firm and whitish cut surface. Histologically, the neoplasm was predominantly composed of bland and monomorphic spindle cells, with a predominantly fascicular growth pattern, in which many tubular and cleft-like spaces of entrapped normal respiratory epithelium were involved. Myxoid change, stromal hyalinization and scattered bizarre mononucleated and multinucleated cells were also observed. Based on clinico-morphological, immunophenotypical and molecular features, we made a diagnosis of malignant transformation of pulmonary adenoleiomyomatous hamartoma into pulmonary leiomyosarcoma. As far as we know, this is the first described case of this exceptionally rare occurrence in an already rare neoplasm.

[A Patient with Small Intestinal Leiomyosarcoma and Intussusception Who Underwent Single Port Surgery].

The patient was a 33-year-old female. She was referred to our hospital from a previous clinic with abdominal pain and vomiting. Contrast-enhanced abdominal CT revealed intussusception, leading to a diagnosis of intussusception with a small intestinal tumor as an advanced part. Intestinal obstruction symptoms were observed, and emergency surgery was performed on the same day. As a technique, single port surgery was selected, and laparoscopic reduction was attempted. However, it was ineffective. The site of intussusception was induced outside the body through a small laparotomy wound, and reduction was performed using Hutchinson's procedure. As an adjacent lymph node was markedly swollen, the mesentery involving this lymph node was dissected in a fan shape, and the tumor was extirpated. The tumor measured 40 mm in long diameter, being a hemicircular, protruding lesion. Histologically, disarray of short spindle tumor cells was observed. Immunostaining showed Kit- and DOG1-negtive reactions and partially α-SMA and desmin-positive reactions, suggesting leiomyosarcoma. With the establishment of an immunostaining-test-based classification, leiomyosarcoma is currently rare. In this study, we report a patient in whom single port surgery for intussusception related to small intestinal leiomyosarcoma was successful.

Subcutaneous leiomyosarcoma in a cynomolgus macaque (Macaca fascicularis).

Leiomyosarcoma, a malignant tumour originating from smooth muscle cells, has rarely been documented in non-human primates. In this case study, a 7-year-old female cynomolgus macaque (Macaca fascicularis) presented with a rapidly growing mass overlying the left elbow joint. Radiographs indicated the presence of a soft tissue neoplasm without any associated bone involvement. The mass was surgically resected. Histological and immunohistochemical analyses revealed spindle-shaped cells with eosinophilic cytoplasm that resembled smooth muscle cells, exhibiting positive immunoreactions for vimentin, desmin and smooth muscle actin and a negative reaction for pan-cytokeratin. This is the first reported case of subcutaneous leiomyosarcoma in a cynomolgus macaque and provides important insights into the incidence and characteristics of this condition in this species.

Deep learning radiomics-based prediction model of metachronous distant metastasis following curative resection for retroperitoneal leiomyosarcoma: a bicentric study.

BACKGROUND: Combining conventional radiomics models with deep learning features can result in superior performance in predicting the prognosis of patients with tumors; however, this approach has never been evaluated for the prediction of metachronous distant metastasis (MDM) among patients with retroperitoneal leiomyosarcoma (RLS). Thus, the purpose of this study was to develop and validate a preoperative contrast-enhanced computed tomography (CECT)-based deep learning radiomics model for predicting the occurrence of MDM in patients with RLS undergoing complete surgical resection. METHODS: A total of 179 patients who had undergone surgery for the treatment of histologically confirmed RLS were retrospectively recruited from two tertiary sarcoma centers. Semantic segmentation features derived from a convolutional neural network deep learning model as well as conventional hand-crafted radiomics features were extracted from preoperative three-phase CECT images to quantify the sarcoma phenotypes. A conventional radiomics signature (RS) and a deep learning radiomics signature (DLRS) that incorporated hand-crafted radiomics and deep learning features were developed to predict the risk of MDM. Additionally, a deep learning radiomics nomogram (DLRN) was established to evaluate the incremental prognostic significance of the DLRS in combination with clinico-radiological predictors. RESULTS: The comparison of the area under the curve (AUC) values in the external validation set, as determined by the DeLong test, demonstrated that the integrated DLRN, DLRS, and RS models all exhibited superior predictive performance compared with that of the clinical model (AUC 0.786 [95% confidence interval 0.649-0.923] vs. 0.822 [0.692-0.952] vs. 0.733 [0.573-0.892] vs. 0.511 [0.359-0.662]; both P < 0.05). The decision curve analyses graphically indicated that utilizing the DLRN for risk stratification provided greater net benefits than those achieved using the DLRS, RS and clinical models. Good alignment with the calibration curve indicated that the DLRN also exhibited good performance. CONCLUSIONS: The novel CECT-based DLRN developed in this study demonstrated promising performance in the preoperative prediction of the risk of MDM following curative resection in patients with RLS. The DLRN, which outperformed the other three models, could provide valuable information for predicting surgical efficacy and tailoring individualized treatment plans in this patient population. TRIAL REGISTRATION: Not applicable.

Primary left ventricular leiomyosarcoma: a case report.

Cardiac leiomyosarcomas are a rare subset of the already infrequent, primary malignant cardiac neoplasia spectrum. The most common site for a primary leiomyosarcoma of the ventricle is on the right with fewer than five globally reported cases in the left ventricle. Most present with non-specific symptoms but attention is usually sought after the appearance of compressive symptoms or arrhythmias. We present a case of a left ventricular leiomyosarcoma in a 50-year old female patient that had a delayed diagnosis and its subsequent surgical resection and oncological management with docetaxel and gemcitabine. This case highlights the need for a high index of suspicion for cardiac masses especially if there are competing chronic diseases with similar symptomatology. Given the rare presentation of left ventricular leiomyosarcomas, case reports may provide valuable information that is otherwise unavailable.

Leiomyosarcoma of sphenoid sinus- A rare tumor at an unusual site.

Leimyosarcoma (lms) is a malignant soft tissue tumor of smooth muscles. The tumor arises intramuscularly and in subcutaneous locations. It is unusual to encounter lms in head and neck region, even more infrequent to discover lms in nasal and paranasal sinuses. A case of 28 years old male with leiomyosarcoma originating from sphenoid sinus with intracranial extension is being presented with aim to highlight its rarity and to highlight the differential diagnosis and the need for prudent diagnosis in the work-up of the patient.

Posterior mediastinal leiomyosarcoma: Case report and literature review.

BACKGROUND: Posterior mediastinal leiomyosarcoma is an extremely rare malignant mesenchymal tumor with no special clinical symptoms, which is easily confused with some common tumors in the posterior mediastinum, affecting the accuracy of the first diagnosis by clinicians and delaying the treatment of patients. CASE SUMMARY: We report a 59-year-old woman with a space-occupying lesion in the posterior mediastinum. The patient was mistakenly diagnosed with lumbar muscle or vertebral body lesions due to chest and back pain and underwent conservative treatment, but her symptoms did not improve significantly and she gradually developed pain in both lower limbs. Chest computed tomography (CT) scan indicated the left lower lung paraspinal space and underwent standard single-aperture video-assisted thoracoscopic surgery (VATS), which was pathologically confirmed as posterior mediastinal leiomyosarcoma. CONCLUSION: Complete surgical resection of posterior mediastinal leiomyosarcoma can achieve good clinical results.

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.

Surgically treated pelvic liposarcoma and leiomyosarcoma: The effect of tumor size on cancer-specific survival.

INTRODUCTION: In soft tissue pelvic liposarcoma and leiomyosarcoma, it is unknown whether a specific tumor size cut-off may help to better predict prognosis, defined as cancer-specific survival (CSS). We tested whether different tumor size cut-offs, could improve CSS prediction. MATERIALS AND METHODS: Surgically treated non-metastatic soft tissue pelvic sarcoma patients were identified (Surveillance, Epidemiology, and End Results 2004-2019). Kaplan-Meier plots, univariable and multivariable Cox-regression models and receiver operating characteristic-derived area under the curve (AUC) estimates were used. RESULTS: Overall, 672 (65 %) liposarcoma (median tumor size 11 cm, interquartile range [IQR] 7-16) and 367 (35 %) leiomyosarcoma (median tumor size 8 cm, IQR 5-12) patients were identified. The p-value derived ideal tumor size cut-off was 17.1 cm, in liposarcoma and 7.0 cm, in leiomyosarcoma. In liposarcoma, according to p-value derived cut-off, five-year CSS rates were 92 vs 83 % (≤17.1 vs > 17.1 cm). This cut-off represented an independent predictor of CSS and improved prognostic ability from 83.8 to 86.8 % (Δ = 3 %). Similarly, among previously established cut-offs (5 vs 10 vs 15 cm), also 15 cm represented an independent predictor of CSS and improved prognostic ability from 83.8 to 87.0 % (Δ = 3.2 %). In leiomyosarcoma, according to p-value derived cut-off, five-year CSS rates were 86 vs 55 % (≤7.0 vs > 7.0 cm). This cut-off represented an independent predictor of CSS and improved prognostic ability from 68.6 to 76.5 % (Δ = 7.9 %). CONCLUSIONS: In liposarcoma, the p-value derived tumor size cut-off was 17.1 cm vs 7.0 cm, in leiomyosarcoma. In both histologic subtypes, these cut-offs exhibited the optimal statistical characteristics (univariable, multivariable and AUC analyses). In liposarcoma, the 15 cm cut-off represented a valuable alternative.