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.

Validating the diagnostic accuracy of an MRI-based scoring system for differentiating benign uterine leiomyomas from leiomyosarcomas.

OBJECTIVE: Uterine leiomyomas are the most common benign uterine tumors. They are difficult to distinguish from their malignant counterparts-smooth muscle tumors of unknown malignant potential (STUMP) and leiomyosarcoma. The purpose of this study is to propose and validate the diagnostic accuracy of the MRI-based Oman-Canada Scoring System of Myometrial Masses (OCSSMM) to differentiate uterine leiomyomas from STUMP/leiomyosarcomas. METHODS: This is a retrospective study performed at two tertiary care centers. All patients with a pathology-proven uterine mass who underwent pre-operative pelvic MRI between January 2010 and January 2020 were included. Using a 1.5T MRI machine, sequences included were axial/coronal/sagittal T2 and T1 weighted imaging, axial diffusion weighted and apparent diffusion coefficient map, and axial or sagittal dynamic contrast-enhanced sequences. A scoring system was designed based on previously published worrisome MRI features for uterine leiomyosarcoma. Each feature was allocated a score from 0 to 2 according to the strength of association with malignancy. Subsequently, the MR images were blindly and independently reviewed by a fellowship-trained radiologist and a clinical fellow/senior resident. Each uterine mass was scored according to their imaging features. The scores were divided into five categories according to the sum of scores. Category III and above was considered positive for leiomyosarcoma/STUMP. Sensitivity, specificity, and positive and negative predictive values were calculated. RESULTS: A total of 244 women were included (age range 20-74 years, mean 40). Of these, 218 patients had benign leiomyoma, 13 had STUMP, and 13 had leiomyosarcoma. The sensitivity and specificity of the scoring system were 92.3% and 64.7%, respectively. The negative predictive value was 98.6%. No leiomyosarcoma was missed using this scoring system. The presence of non-cystic T2 hyperintensity or diffusion restriction in a uterine mass were the most sensitive signs of a leiomyosarcoma/STUMP. CONCLUSION: The proposed multi-parametric MRI scoring system may be useful in differentiating benign uterine leiomyomas from leiomyosarcomas/STUMP.

Smooth muscle tumours of the uterus: MR imaging malignant predictive features-a 12-year analysis in a referral hospital in Portugal.

PURPOSE: To evaluate the magnetic resonance imaging (MRI) features that may help distinguish leiomyosarcomas from atypical leiomyomas (those presenting hyperintensity on T2-W images equal or superior to 50% compared to the myometrium). MATERIALS AND METHODS: The authors conducted a retrospective single-centre study that included a total of 57 women diagnosed with smooth muscle tumour of the uterus, who were evaluated with pelvic MRI, between January 2009 and March 2020. All cases had a histologically proven diagnosis (31 Atypical Leiomyomas-ALM; 26 Leiomyosarcomas-LMS). The MRI features evaluated in this study included: age at presentation, dimension, contours, intra-tumoral haemorrhagic areas, T2-WI heterogeneity, T2-WI dark areas, flow voids, cyst areas, necrosis, restriction on diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) values, signal intensity and heterogeneity after contrast administration in T1-WI, presence and location of unenhanced areas. The association between the MRI characteristics and the histological subtype was evaluated using Chi-Square and ANOVA tests. RESULTS: The MRI parameters that showed a statistically significance correlation with malignant histology and thus most strongly associated with LMS were found to be: irregular contours (p < 0.001), intra-tumoral haemorrhagic areas (p = 0.028), T2-WI dark areas (p = 0.016), high signal intensity after contrast administration (p = 0.005), necrosis (p = 0.001), central location for unenhanced areas (p = 0.026), and ADC value lower than 0.88 × 10-3 mm2/s (p = 0.002). CONCLUSION: With our work, we demonstrate the presence of seven MRI features that are statistically significant in differentiating between LMS and ALM.

Breast lump as the initial presentation of metastatic uterine leiomyosarcoma: a case report and comprehensive literature review.

Uterine leiomyosarcoma (uLMS) is a rare but aggressive cancer with a high metastatic potential and an unfavorable prognosis. A 54-year-old woman with a history of uterine fibroids clinically presented with a painless, palpable left breast mass measuring 20 mm. A core biopsy of the breast mass demonstrated a cellular spindle cell neoplasm (a potentially malignant smooth muscle neoplasm; B4). A wide local breast-mass excision was performed, revealing grade-2 leiomyosarcoma. A re-review of the uterine fibroids revealed that the largest one (200 × 130 mm), initially diagnosed as symplastic leiomyoma, was morphologically identical to the breast lesion. Additional diagnostic work-up revealed multiple liver and pulmonary metastases with a suspected metastatic sclerotic lesion in the L3 projection. The patient was subsequently treated with chemotherapy protocol for metastatic uLMS. The latest follow-up in September 2023 confirmed stable disease. This case highlights the importance of considering unusual metastatic patterns when evaluating breast masses, particularly in patients with a history of non-specific uterine conditions. Comprehensive diagnostic work-up, including imaging and histopathologic examinations, is crucial for an accurate diagnosis of uLMS and appropriate treatment selection. Further studies are needed to better understand the underlying mechanisms and optimal management strategies for metastatic uLMS.

[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.

The role of multiparametric MRI in differentiating uterine leiomyosarcoma from benign degenerative leiomyoma and leiomyoma variants: a retrospective analysis.

AIM: To assess qualitative and quantitative magnetic resonance imaging (MRI) factors that can help distinguish leiomyosarcoma (LMS) from benign degenerative leiomyoma (BDL) and leiomyoma variants (LV) and assess the interobserver agreement for the proposed quantitative factors. MATERIALS AND METHODS: Retrospective analysis of all histopathology proven cases of LV, BDL, and LMS with a preoperative MRI was performed. Twenty-seven cases were included (five LMS, three LV, and 19 BDL) with each case independently read by a pair of radiologists. Lesion size, margins, presence or absence of degeneration, necrosis, and haemorrhage were assessed on MRI along with quantitative factors such as mean T2-weighted (W) and T1W signal intensity, T1W signal heterogeneity, diffusion-weighted imaging (DWI), and apparent diffusion coefficient (ADC) ratios as well as dynamic contrast enhancement (DCE) characteristics along with the presence or absence of lymphadenopathy and extra-uterine and peritoneal spread. Mean and standard deviation for quantitative variables and frequency with percentages for qualitative variables were assessed. RESULTS: Infiltrative margins were seen exclusively in the LMS group (n=1), with the remaining LMS cases showing lobulate or rounded smooth margins similar to BDL or LV. A high T2W signal <25% was seen exclusively in the BDL group (n=8). The presence of concomitant necrosis and haemorrhage was seen exclusively in the LMS group (n=2). Quantitative MRI had good inter-reader correlation but was not significantly different between the LMS, BDL, and LV groups. CONCLUSION: LMS, BDL, and LV may have overlapping features on multiparametric MRI making differentiation difficult.

Clinical features of uterine sarcomas presenting mainly with uterine masses: a retrospective study.

BACKGROUND: Uterine sarcomas are uncommon mesenchymal tumors of the uterus. The clinical problem is that the features of uterine sarcomas can sometimes mimic uterine fibroids. This study aims to investigate the clinical characteristics of patients with uterine sarcomas who were preoperative presenting mainly with uterine masses. METHODS: A retrospective analysis of patients who underwent gynecological surgery for uterine sarcomas at the Obstetrics & Gynecology Hospital of Fudan University, between January 2016 and December 2021. RESULTS: Over the 5-year period, 277 patients were final diagnosed of uterine sarcomas. A total of 162 patients were preoperatively diagnosed as uterine fibroids for surgical treatment, the majority of whom were diagnosed of uterine leiomyosarcoma (uLMS) (49/162) and low-grade endometrial stromal sarcoma (LG-ESS) (100/162). Ninety people underwent total hysterectomy and bilateral salpingo-oophorectomy (TH + BSO), while 72 underwent myomectomy followed by supplemental TH + BSO. The group with direct hysterectomy had a higher average age than the group with prior myomectomy (47.20 ± 8.94 vs. 40.86 ± 5.88, p < 0.001). Among patients preoperatively diagnosed as uterine fibroids, patients with uLMS had a higher proportion of previous myomectomy (26.53% vs. 5.00%, p < 0.001), a larger uterine mass diameter on ultrasound (8.38 ± 3.39 cm vs. 6.41 ± 1.92 cm, p < 0.001), and richer hypervascularity (34.69% vs. 18%, p = 0.024) compared with LG-ESS. CONCLUSIONS: Analysis of our data showed that a large proportion of uterine sarcomas, especially uLMS and LG-ESS, present mainly with uterine masses. Ultrasound features including a large uterine mass diameter and rich hypervascularity, and with a history of myomectomy may alert clinicians in suspicion of uLMS when compared with LG-ESS.

CT features of malignant tubular genital tract tumors in seven goats.

Neoplasia of the tubular genital tract in goats, while rarely described, is most commonly reported as uterine adenocarcinoma, leiomyoma, or leiomyosarcoma. In this retrospective, single-center, case series, medical records were searched for goats with a computed tomography (CT) diagnosis of tubular genital mass and a definitive histologic (surgical biopsy or necropsy) diagnosis of malignant neoplasia. Data recorded from CT images were presence of peritoneal/retroperitoneal fluid, urinary tract obstruction, abdominal lymphadenomegaly, additional abdominal nodules/masses, and pulmonary nodules. For masses, maximum cross-sectional area, contrast enhancement, and uterine luminal fluid accumulation were also recorded. Seven goats met the inclusion criteria (leiomyosarcoma n = 5, adenocarcinoma n = 2). Both goats with adenocarcinoma had upper urinary tract obstruction, moderate to severe regional lymphadenopathy, peritoneal fluid, and peritoneal or hepatic nodules/masses; one goat with adenocarcinoma was discharged and subsequently euthanized, and the other had palliative mass debulking and was lost to follow up. Goats with leiomyosarcoma had infrequent, mild peritoneal fluid and mild sublumbar lymphadenopathy. Of the goats with leiomyosarcoma, two were euthanized at or near the time of CT imaging, two were euthanized at the time of surgery due to perceived mass non-resectability, and one had mass regression approximately four months post ovariohysterectomy but was subsequently lost to follow up. Five goats had pulmonary nodules, three of which had pathologic confirmation (pulmonary metastasis in a single patient with adenocarcinoma, and lungworm granulomas in two goats with leiomyosarcoma). Severe sublumbar lymphadenopathy and obstructive uropathy were sequelae in the two caprine patients with genital adenocarcinoma, and in none with leiomyosarcoma.

Primary gastric leiomyosarcoma presenting as a giant polyp.

We present the case of a 35-year-old woman with previous hereditary retinoblastoma treated with radiotherapy, admitted due to severe iron deficiency anemia. Upper endoscopy and endoscopic ultrasound revealed a 5-cm polypoid lesion in the fundus arising from muscularis mucosa. Histological findings favored a sarcoma with muscular differentiation. After exclusion of metastatic disease, the patient underwent surgery and diagnosis of primary gastric leiomyosarcoma was confirmed. We report a case of double rarity of gastric leiomyosarcoma, as she presented with severe anaemia from a polypoid lesion of the gastric fundus.

A Rare Case of Pulmonary Leiomyosarcoma.

Leiomyosarcoma commonly occurs in the abdomen, retroperitoneum, large blood vessels, and uterus[1]. Cardiac leiomyosarcoma is a rare and highly aggressive sarcoma. We reported a case of a 63-year-old male with pulmonary artery leiomyosarcoma. Transthoracic echocardiography showed a large 4.4×2.3 cm hypoechoic mass in the right ventricular outflow tract and pulmonary artery. Computed tomography pulmonary angiography showed a filling defect in a similar location. The initial impression was PE, but a tumor was not ruled out. An emergency surgery was performed due to progressively worse chest distress and dyspnea. A yellow mass that had adhered to the ventricular septum and pulmonary artery wall was detected to be compressing the pulmonary valve. Immunohistochemistry confirmed tumor cells positive staining for Desmin and smooth muscle actin and negative staining for S-100, CD34, myogenin, or myoglobin, and KI67(+)80%, indicating leiomyosarcoma. Pulmonary leiomyosarcoma showed a side-inserted heart chamber filling defect in CTA and should be excised when the patient suddenly deteriorated.

[Primary Leiomyosarcoma of the Epididymis : A Case Report].

A 63-year-old man presented with right scrotal swelling. A physical examination revealed a painless, palpable mass in the right scrotum. The mass was well defined and lobulated. Subsequently, a diagnosis of right epididymal tumor was made, and right high orchiectomy was performed. Hematoxylin-eosin and immunostaining revealed leiomyosarcoma of the epididymis. When a diagnosis of epididymal malignant tumor is made, the standard treatment is radical orchiectomy.

[A Case of Retroperitoneal Leiomyosarcoma Treated with Repeated Surgical Resection].

A 55-year-old male revealed with a 5 cm-diameter mass in the lower abdomen on ultrasonography incidentally. Computed tomography showed a mass of 7 cm in size on the left side of the bladder. A malignant tumor was suspected, and surgically excised for purpose of diagnosis and treatment. Pathological examination confirmed retroperitoneal leiomyosarcoma, and the resection margins were negative. Follow-up computed tomography scan was performed every 3 months. Repeated resections were performed for twice recurrences within a year after surgery. A year after the first surgery, lung metastasis was detected and chemotherapy was started. Although retroperitoneal leiomyosarcoma is considered to have a poor prognosis, the present case had relatively good prognosis. This may be due to early detection and repeated surgical resection.

Ultrasound features of highly vascularized uterine myomas (uterine smooth muscle tumors) and correlation with histopathology.

OBJECTIVE: To correlate the ultrasound appearance of highly vascularized uterine myomas with their histopathological diagnosis. METHODS: This was a prospective observational study of patients with a preoperative ultrasound diagnosis of a highly vascularized uterine myoma (color score of 3 or 4, according to the Morphological Uterus Sonographic Assessment (MUSA) criteria), characterized by circumferential and intralesional vascular pattern, who underwent myomectomy or hysterectomy. For each patient, ultrasound characteristics were recorded at baseline, including the number of lesions, the size, echogenicity and border regularity of the lesion, presence of cystic areas and shadowing within the myoma, and visualization of the endometrium. Ultrasound features were correlated with the definitive histological diagnosis. Ultrasound features were then compared between malignant and benign lesions. RESULTS: We included 70 patients with highly vascularized uterine myomas on power/color Doppler. Their mean age was 46.5 ± 11.4 years and 13 (18.6%) were postmenopausal. At histological examination, 65 (92.9%) uterine myomas were benign lesions, comprising 32 typical leiomyomas, 29 leiomyoma variants and four adenomyomas. The remaining five (7.1%) uterine myomas were malignant masses, comprising two uterine sarcomas, one leiomyosarcoma, one neuroendocrine tumor and one uterine smooth muscle tumor of uncertain malignant potential (STUMP). The mean age of patients with a malignant lesion was significantly higher than the age of those with a benign lesion (64.8 ± 16.0 vs 42.4 ± 5.1; P < 0.001). Four out of five patients with a malignant lesion were over 45 years old. Ultrasound demonstrated cystic areas within the lesion in 10/32 (31.3%) typical leiomyomas, 16/29 (55.2%) leiomyoma variants, all four adenomyomas and in the cases of STUMP and leiomyosarcoma. Lesion borders were regular in 64/65 (98.5%) benign lesions and 2/5 (40%) malignant lesions (P < 0.05). No significant differences were observed between benign and malignant lesions with respect to echogenicity, presence of shadowing and size. The endometrium was visible in 55/65 women with benign lesions and in 2/5 with malignant lesions (P = 0.03). CONCLUSIONS: Our results showed that ultrasound features of uterine myomas, such as circumferential and intralesional vascularity, cystic areas and lesion borders, are important parameters for differential diagnosis, especially when combined with the patient's age. Such features could be useful to differentiate typical myomas from benign variants and malignant lesions in a preoperative setting and to select patients that may benefit from conservative management rather than surgery. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Can Posttransarterial Chemoembolization Volumetric Oil Deposition on Computed Tomography Predict Treatment Response on Magnetic Resonance Imaging in Leiomyosarcoma Metastases to the Liver?

OBJECTIVE: To predict early tumor response to transarterial chemoembolization (TACE) based on volumetric oil deposition on posttreatment computed tomography (CT) in patients with leiomyosarcoma liver metastases. METHODS: This retrospective lesion-by-lesion based study included 32 lesions. The volumetric percent enhancing tumor on pre-TACE and 1-month post-TACE venous phase magnetic resonance imaging (MRI), and the percent oil deposition on CT 1 day after TACE were calculated. The predicted post-TACE enhanced percentage was computed by subtracting percent oil deposition from baseline percent enhanced. RESULTS: Mean percentage of viable tumor on pre-TACE MRI was 90.6% ± 9.3%. Mean oil deposition was calculated as 51.4% ± 26.2%. Mean percentage of measured residual tumor enhancement 1 month after TACE was 58.3% ± 27%, which correlates with predicted enhancement percentage of 43.9% ± 25.1% (r = 0.72, P < 0.001). A threshold of 35.5% for enhancement reduction was determined to predict tumor response with an accuracy of 78.1%. CONCLUSION: Volumetric oil deposition on CT can predict residual enhancement on post-TACE MRI.

Femoral Artery Leiomyosarcoma: Case Report and Literature Review.

BACKGROUND: Vascular leiomyosarcoma is a rare type of malignant tumor which arises from the smooth muscle tissue of blood vessel walls; it involves veins five times more frequently than arteries. There are only a few cases published in the literature and consequently there is limited experience regarding treatment and prognosis. METHODS: A 66-year-old woman presented with left lower limb swelling and a left inguinal mass. Imaging revealed a seven-by-five cm well-circumscribed oval inguinal mass that incorporated the common femoral artery including its bifurcation and compressed the common femoral vein. Other malignancies or metastatic disease were excluded. The patient underwent en bloc resection of the tumor, including the common femoral artery and its bifurcation and arterial reconstruction, using the inverted contralateral great saphenous vein, was carried out. Histopathological examination of the mass revealed moderately-differentiated leiomyosarcoma arising from the femoral artery wall without invasion of the intima. The postoperative course was uneventful. 12 months after the procedure the patient was in good clinical conditions and a contrast enhanced CT scan showed patency of the arterial reconstruction without local recurrence or metastatic disease. RESULTS: A systematic literature search identified nine cases of femoral artery leiomyosarcoma; in the eight patients for whom follow-up data were reported, recurrent or metastatic disease developed in five and only three were alive and free of disease. CONCLUSIONS: As with any soft tissues sarcoma, complete surgical resection is the cornerstone of treatment and any directly involved adjacent structures must be sacrificed, as well as a margin of uninvolved normal tissue; consequently, a vascular reconstruction is almost always necessary.

Ultrasound Features and Diagnostic Workup of Uterine Leiomyosarcomas.

Uterine leiomyosarcoma is a rare malignancy and is difficult to diagnose preoperatively. In this case series, we retrospectively reassessed ultrasound findings of 18 leiomyosarcoma cases and proposed the diagnostic workup. We are select seven ultrasound features and found irregular tumor border in 100%, loss of normal myometrium >25% in 61.11% (12 cases), loss of typical benign leiomyoma feature >50% in 77.78%, necrosis in 85.7% (16 cases), and cystic degeneration in 83.3% (15 cases). Circumferential vascularity was absent or minimal in 66.7% of cases, whereas intralesional vascularity with minimal or moderate intralesional vascularity was seen in 12 (66.7%) cases. Diagnosis of suspected uterine leiomyosarcoma requires five out of these seven features present, four gray-scale and one color Doppler ultrasound.

[Clinicopathological characteristics and molecular alterations of primary cardiac leiomyosarcoma: report of five cases].

Objective: To investigate the clinical, pathologic and radiologic features and molecular alterations in patients with primary cardiac leiomyosarcoma (PCLMS). Methods: Five cases of PCLMS were collected in Beijing Anzhen Hospital from January 2016 to December 2020. The clinical, pathologic and radiologic data, and molecular alterations were analyzed, and the patients were followed up. Results: All five patients were female, and had no history of leiomyosarcoma in other parts of the body. The age of patients ranged from 37 to 62 years (median 47 years). The main clinical symptoms were chest pain and dyspnea, one also presented with palpitation and lower limb weakness and one with dizziness. Two tumors were located in the left atrium, two in the right atrium, and one in the right ventricle, and they maximal diameter ranged from 2.5 to 14.0 cm (mean 6.2 cm). The neoplasms presented as medium-echo masses with a broad base in the echocardiography, and as a low-density, solid mass when detected by contrast-enhanced CT. Histologically, two tumors were well-differentiated and three were moderately and poorly differentiated, and two included extensive, loose myxoid stroma. Immunohistochemical staining showed that PCLMS was positive for SMA, desmin, MDM2, and epidermal growth factor receptor. Fluorescence in situ hybridization showed ALK gene rearrangement in two cases, and COL1A1-PDGFB fusion in three cases. All cases received surgical excision and two cases received chemotherapy. Three patients died within 0-11 months (mean survival of 7.7 months) and two patients were alive. Conclusions: PCLMS is a malignant tumor with a high recurrence rate and poor prognosis. These cases may provide useful information to improve the diagnosis and management of PCLMS.

[Surgical Case of the Primary Pulmonary Leiomyosarcoma:Report of a Case].

A 70-year-old man was referred for an abnormal chest shadow. Enhanced computed tomography (CT) revealed a well-circumscribed lung tumor of 53 mm in diameter in the left upper lobe with slight enhancement. Positron emission tomography-CT showed a high maximum standardized uptake value for the tumor but no metastasis in the lymph nodes or other organs. Although a definitive diagnosis could not be made by transbronchial biopsy, the tumor was highly suspected to be malignant based on the radiological findings, and a left upper lobectomy with mediastinal lymph nodes dissection was performed for definitive diagnosis and treatment. A pathological examination showed the tumor to be composed of mitotic spindle-shaped cells, which were positive for α-smooth muscle actin, desmin, and caldesmon. The MIB-1 labelling index was 60~70%. According to these pathologic findings, the tumor was identified as a leiomyosarcoma. Metastases to the skin of chest and hilar lymph nodes were noted six months after the surgery for which radiotherapy was performed.

[A Case of Retroperitoneal Leiomyosarcoma Resected Radically by Pancreaticoduodenectomy with Vascular Resection].

A 54-year-old woman visited to a doctor nearby medical clinic complaining of loss of appetite. She was diagnosed with right hydronephrosis on abdominal ultrasonography, and referred to our hospital for further examination. Contrast abdominal computed tomography(CT)revealed that a 6.2 cm tumor with a contrast-enhancing effect inside in the retroperitoneum near the lower pole of the right kidney. She was diagnosed with hydronephrosis due to infiltration of the right kidney of a retroperitoneal tumor. The tumor was suspected of invading the duodenum and inferior vena cava, but no obvious lymph node or distant metastasis was observed. Abdominal MRI revealed a tumor showed hyperintensity on T2-weighted and diffusion-weighted images. We performed pancreaticoduodenectomy with inferior vena cava resection and right nephrectomy. The pathological diagnosis was leiomyosarcoma originating from retroperitoneum and pT2, pN0, pM0, pStage ⅢA. The postoperative course was good, and she was discharged 10 days after the operation. Thoracoabdominal CT showed a tumor 4 cm at the hepatic hilum three months after surgery, and EOB-MRI showed many tumors other than the same site, so multiple liver metastases were diagnosed as recurrence. Doxorubicin has been started and is still being treated.

Resection of Retro-Hepatic Vena Cava (RHVC) En-bloc with Caudate Lobe without Vascular Exclusion for a Low Grade Leiomyosarcoma of Inferior Vena Cava.

BACKGROUND: Leiomyosarcomas (LMS) of the inferior vena cava (IVC) originate in the retrohepatic (RHVC) portion in 15% of cases.1 Due to complex anatomy and need to preserve venous outflow from the infra-diaphragmatic viscera, the operation may require total vascular exclusion, veno-venous bypass and hypothermic liver resections.2,3 In this video, virtual planning of the operation allowed a parenchyma-sparing radical resection in a patient with limited liver reserve. METHODS: A 12-cm LMS of RHVC invading the entire segment 1 (i.e., Spiegel's lobe, paracaval portion, and caudate process) was diagnosed in a man with metabolic steato-hepatitis (BMI: 34). He had no response to previous chemotherapy. Major hepatectomy was excluded considering the high risk of postoperative liver failure. 3D-reconstruction of regional anatomy allowed planning of a parenchymal-sparing, en bloc resection of tumor, RHVC, and caudate lobe while avoiding hilar and suprahepatic venous clamping. RESULTS: The operation strategy relied on the en bloc separation of caudate lobe, RHVC, and tumor from the hepatic veins confluence and the posterior segments after complete mobilization of the liver. Vessel loop-assisted hanging maneuver, encircling tumor, and RHVC with superimposed 3D-reconstructions guided the parenchymal transection, while preserving the middle hepatic vein outflow. RHVC was replaced with prosthetic material. CONCLUSIONS: Complex resection of primary tumor of the IVC en bloc with caudate lobe and RHVC can be attempted in chronic liver diseases at-risk of postoperative failure. Preservations of transhepatic flow and liver function depends on tumor size and preservation of noninvaded hepatic-veins confluence. Preoperative virtual 3D reconstruction is crucial in surgical planning.