A case report of hereditary leiomyomatosis and renal cell carcinoma (HLRCC).

Leiomyomatosis and renal cell carcinoma (HLRCC) are rare autosomal dominant cancer syndromes characterized by cutaneous leiomyoma, uterine leiomyoma, and renal cell carcinoma (RCC). RCC in HLRCC is an aggressive metastatic tumor that develops at a young age. Here, we report the case of a patient with HLRCC who was diagnosed after the spontaneous rupture of a renal tumor. The patient underwent cytoreductive surgery, followed by combination therapy with the immune checkpoint inhibitor (ICI) nivolumab and cabozantinib, a tyrosine kinase inhibitor (TKI); however, no improvements were achieved.

Modules From the End-of-Life Nursing Education Consortium: Japan Core Curriculum Necessary for Second- to Fourth-Year Nurses as Assessed by Advanced Practice Registered Nurses.

AIM: This study aimed to identify the modules of the End-of-Life Nursing Education Consortium-Japan Core Curriculum (ELNEC-J), which are particularly necessary for second- to fourth-year nurses. METHODOLOGY:  This cross-sectional study recruited certified nurse specialists in cancer nursing (CNSCNs) endorsed by the Japanese Nursing Association enrolled in Advanced Practice Registered Nurses (APRNs) in Japan. We asked individuals who were active members of the volunteer association of CNSCNs in the Tokai region to participate via email, and we collected data using Google Forms. The participants were asked about their background, including APRN experience and current position. Furthermore, we asked them to select three necessary modules for second- to fourth-year nurses' education from the 10 modules of the ELNEC-J. RESULTS:  The study recruited a total of 19 (89%) APRNs (response rate: 100%). Out of them, 14 (73.6%) had more than six years of clinical experience in APRNs, and 12 (63.1%) held managerial positions. Regarding the 10 modules of the ELNEC-J, the responses for the necessary modules were as follows: nursing care at end-of-life 13 (68.4%), pain management 12 (63.2%), symptom management 10 (52.6%), communication 10 (52.6%), and ethical issues in palliative care nursing five (31.6%). CONCLUSION: According to the perspective of APRNs responsible for palliative care education for incumbent nurses, nursing care at the end of life, pain management, symptom management, and communication are required for second- to fourth-year nurse education.

Intraoperative cone-beam computed tomography-guided curettage for osteoid osteoma.

Recently, cone-beam computed tomography (CBCT)-guided surgeries have been developed for bone and soft tissue tumors. The present study aimed to evaluate the efficacy of CBCT-guided curettage for osteoid osteoma. Our study population included 13 patients who underwent primary curettage for osteoid osteoma using intraoperative CBCT in a hybrid operating room between April 2019 and November 2022. We collected the following data: sex, age, follow-up period, symptom onset to time of surgery, tumor size and location, length of skin incision, operating time, radiation dose, recurrence, postoperative complications, and visual analog scale for pain during the last follow-up. There were 10 male and 3 female patients, and the mean age was 25.0 years (range, 9-49 years). The mean follow-up period was 10.6 months (range, 0.4-24.0 months). The locations of the tumors were the proximal femur in 6 patients, the acetabular region in 2 patients, and the ilium, tibial shaft, calcaneus, cuboid, and talus in 1 patient each. The mean time of symptoms onset to surgery was 18.7 months (range, 2.3-69.9 months). The mean maximum diameter of the tumor was 5.9 mm (range, 3.5-10.0 mm). The mean length of the skin incision was 2.2 cm (range, 1.5-3.5 cm). The mean operating time was 96.9 minutes (range, 64-157 minutes). The mean dose of radiation was 193.2 mGy (range, 16.3-484.0 mGy). No recurrences, postoperative complications, and reoperation were observed in this study. All the patients reported 0 mm on the visual analogue scale for pain on the last follow-up. CBCT-guided curettage for osteoid osteoma was minimally invasive and reliable. This procedure can be effective for the treatment of lesions found in deep locations such as the pelvic bone and proximal femur or an invisible lesion that cannot be detected by regular fluoroscopy.

p16 Overexpression in Sinonasal Squamous Cell Carcinoma: Association with Human Papillomavirus and Prediction of Survival Outcomes.

p16 overexpression is often used as a surrogate marker for human papillomavirus (HPV) infection in oropharyngeal squamous cell carcinoma but remains an uncertain diagnostic tool for HPV-related sinonasal squamous cell carcinoma (SNSCC). Our study involved 79 consecutive SNSCC patients who were treated at a tertiary referral university hospital during 2006-2021. We retrospectively examined their clinical characteristics and conducted p16 immunohistochemistry and HPV detection. We found that 12.7% of the patients exhibited p16 overexpression, which was significantly more common in the nasal cavity and increased from 2015 onward. The HPV was a high-risk type and viral loads ranged from 4.2 to 1.6 × 106 copies/ng DNA with genome integration. Five-year overall survival (OS) and five-year relapse-free survival (RFS) rates were 74.6% and 69.9%, respectively. Our multivariate analysis showed that T category (T1-4a) and hemoglobin levels (≥13.7) were significant favorable prognostic factors for OS, while T category, performance status, and p16 overexpression were significantly associated with RFS. In patients with p16 overexpression, OS was 100% and RFS was 90%. Our findings suggest that p16 overexpression is a reliable surrogate marker for transcriptionally active HPV infection and predicts a favorable prognosis.

Prediction of Early-stage Liver Fibrosis Using FDG-PET/CT.

BACKGROUND/AIM: 18F-fluorodeoxyglucose (FDG) is known to accumulate in the liver. We investigated whether accumulation of FDG was correlated with the degree of liver fibrosis and the grade of necro-inflammatory activity. PATIENTS AND METHODS: This retrospective study included 35 patients who underwent FDG-positron emission tomography (PET)/computed tomography (CT) before liver surgery. On fusion images of CT and PET, by placing regions of interest on the lateral, anterior and posterior segments of the liver and the aorta, the standardized uptake value (SUV) mean, and SUV normalized by lean body mass (SUL) mean of the liver were measured, and the ratio SUVmean liver/SUVmean aorta was calculated. According to the New Inuyama Classification, subjects were classified into three groups based on the grade of liver-fibrosis degree, i.e., F0, F1+F2 and F3+F4, and into three groups based on the grade of necro-inflammatory activity, i.e., A0, A1 and A2. Each of the above parameters was then compared among the groups using a Tukey test. RESULTS: Average SULmean liver values of the F0, F1+F2 and F3+F4 groups were 1.573±0.211, 1.845±0.220 and 1.716±0.119, respectively. The SULmean liver of the F1+F2 group was significantly higher than that of the F0 group (p=0.0296). No significant difference was observed for the other two parameters. None of the parameters exhibited significant difference among the A0, A1, and A2 groups. CONCLUSION: FDG accumulation in the liver may be increased in the early stage of liver fibrosis. SULmean liver could be used to determine the necessity for therapeutic intervention in chronic liver disease.

Laparoscopic resection of a metastatic myxoid liposarcoma in the mesentery of the small intestine: a case report.

BACKGROUND: Myxoid liposarcoma (MLS), with its risk factors, tends to spread to the lungs and extraperitoneally, with intraperitoneal metastases occurring rarely. We present an unusual case of a myxoid liposarcoma that metastasized to the abdominal organs. CASE PRESENTATION: A 60-year-old female patient was referred to our hospital for the evaluation of a right upper limb tumor that had been growing for 7 years. The patient refused surgery, and during follow-up, tumor hemorrhage resulted in hemorrhagic shock. The patient's right upper limb was immediately amputated. MLS was diagnosed histopathologically. Subsequently, the patient underwent adjuvant chemotherapy. Computed tomography (CT) revealed a right buttock mass, a pelvic mass, and left cardiophrenic angle lymph nodes 3 years after the initial surgery. Contrast-enhanced abdominal CT revealed a relatively low-density, lobulated pelvic tumor. Contrast-enhanced pelvic magnetic resonance imaging (MRI) revealed a low-intensity, lobulated mass on T1-weighted images and a high-intensity mass on T2-weighted images. The pelvic mass showed no significant fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET)-CT. On clinical examination, gynecological malignancies were ruled out as the origin of the pelvic lesions. After resection of the right buttock mass, pelvic mass, and left cardiophrenic angle lymph nodes, the patient underwent laparoscopic surgery for a preoperative diagnosis of small intestinal mesenteric metastasis of MLS. A tumor was found in the mesentery of the small intestine and removed with a margin of 5 cm on both the proximal and distal sides. The specimen measured 10 × 8 × 5 cm and contained a multifocal mass. The tumor was found in the mesentery of the small intestine, with no mucosal or submucosal invasion. The patient was diagnosed with MLS with small mesenteric intestinal metastases. On postoperative day 8, the patient was discharged after an uneventful postoperative course. Twelve months after the surgery, there was no evidence of local or distant recurrence. CONCLUSIONS: Small intestinal mesenteric metastases of MLSs are rare. Moreover, there are few reports on laparoscopic resection. In this case, the laparoscopic approach was useful in detecting the tumor location and determining the range of resection.

A Comprehensive Study of the Immunophenotype and its Clinicopathologic Significance in Adult T-Cell Leukemia/Lymphoma.

Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell tumor caused by human T-lymphotropic virus type 1 (HTLV-1). The typical ATLL immunophenotypes are described in the 2017 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues (positive: CD2, CD3, CD5, CD4, and CD25; negative: CD7, CD8, and cytotoxic markers; and partially positive: CD30, CCR4, and FOXP3). However, limited studies are available on the expression of these markers, and their mutual relationship remains unknown. Furthermore, the expression status of novel markers associated with T-cell lymphomas, including Th1 markers (T-bet and CXCR3), Th2 markers (GATA3 and CCR4), T follicular helper markers (BCL6, PD1, and ICOS), and T-cell receptor (TCR) markers, and their clinicopathologic significance is unclear. In this study, we performed >20 immunohistochemical stains in 117 ATLL cases to determine the comprehensive immunophenotypic profile of ATLL, which were compared on the basis of clinicopathologic factors, including morphologic variants (pleomorphic vs anaplastic), biopsy locations, treatments, Shimoyama classification-based clinical subtype, and overall survival. CD3+/CD4+/CD25+/CCR4+ was considered a typical immunophenotype of ATLL, but approximately 20% of cases did not conform to this pattern. Simultaneously, the following new findings were obtained: (1) most cases were negative for TCR-ß and TCR-δ (104 cases, 88.9%), indicating the usefulness of negative conversion of TCR expression to provide differentiation from other T-cell tumors; (2) the positivity of CD30 and CD15 and the negativity of FOXP3 and CD3 were significantly associated with anaplastic morphology; and (3) atypical cases, such as T follicular helper marker-positive (12 cases, 10.3%) and cytotoxic molecule-positive cases (3 cases, 2.6%), were identified. No single markers could predict the overall survival among patients with acute/lymphoma subtypes of ATLL. The results of this study illustrate the diversity of ATLL phenotypes. In T-cell tumors occurring in HTLV-1 carriers, the possibility of ATLL should not be eliminated even when the tumor exhibits an atypical phenotype, and the confirmation of HTLV-1 in the tissue is recommended.

Reconstruction of the lateral collateral ligament using the plantaris tendon after wide excision of soft tissue sarcoma of the knee: A case report.

An 83-year-old woman presented with a 1-year history of a growing mass on the lateral surface of the right knee. Magnetic resonance imaging revealed a large soft tissue tumor in the subcutis of the right knee. The mass in the right knee rapidly increased, due to hemorrhage from the tumor. A needle biopsy revealed that the diagnosis was synovial sarcoma. The patient underwent wide excision and lateral collateral ligament reconstruction using the plantaris tendon. The patient had a Musculoskeletal Tumor Society Score of 86% at the lateset follow-up. In conclusion, reconstruction of the lateral collateral ligament using the plantaris tendon may be useful for preserving the function of the knee joint after resection of the soft tissue due to sarcoma of the knee.

Wide excision alone for elderly patients aged > 70 years old with soft tissue sarcomas.

The purpose of the present study was to clarify clinical outcomes of elderly patients with soft tissue sarcoma who underwent surgery neither with neoadjuvant nor adjuvant chemotherapy. The median follow-up period was 46.3 (range 6.7-99.0) months. All patients underwent surgical resections. R0 margins were achieved in 24 cases (92.3%) and R1 margins in 2 cases (7.7%). The 1-, 2-, and 5-year sarcoma-specific survival (SSS) rates were 92.3%, 88.5%, and 83.8%, respectively. Multivariate analysis showed no significant risk factors for SSS. No significant relationship of histological grades and local recurrences (P = .56) or distant metastases (P = .54) was shown. In the current study, we observed a comparable survival ratio, despite no neoadjuvant or adjuvant chemotherapies performed. Tumor resections with adequate margins might, at least in part, have contributed to the decent survival ratio regardless of histological grade. Twenty-six consecutive patients aged ≥ 70 years, who underwent surgical resections of soft tissue sarcoma between January 2013 and December 2019, were included. SSS were analyzed by the Kaplan-Meier method, and the relationships between SSS and clinical parameters were evaluated by Cox proportional hazards analysis.

Large adrenocortical adenoma with malignant features on imaging: A case report.

Large adrenocortical adenomas have rarely been reported. We describe a case of a 26-year-old man who underwent an adrenalectomy for a large adrenocortical adenoma (8.6 × 7.7 cm). Although the lesion had typical malignant features on imaging, histopathological examination revealed an adrenocortical adenoma. This highlights that imaging alone may not be able to distinguish adrenocortical carcinomas from adrenal masses. In most cases, a resection should be performed for early diagnosis and management of large adrenal masses with malignant features on imaging. To our knowledge, this is the first report of a large adrenocortical adenoma diagnosed with multiple imaging investigations.

Experiences of caregivers desiring to refuse life-prolonging treatment for their elderly parents at the end of life.

Purpose: This study aimed to clarify the experiences of caregivers desiring to refuse life-prolonging treatment for their elderly parents at the end of life. Methods: A semi-structured interview was performed for four family caregivers who wanted to refuse life-prolonging treatment suggested by the physicians. Results: In this study, four caregivers who refused life-prolonging treatment suggested by the physicians for their elderly parents completed semi-structured interviews. The obtained data were analyzed in relation to the theme "Experiences of caregivers who desire to refuse life-prolonging treatment for their elderly parents at the end of life." As a result, 38 subcategories and 12 categories were extracted. Conclusions: Participants in this study initially had a negative view of life-prolonging treatment. However, they agonized over the decision when they received conflicting advice from the physicians. The participants indicated that physicians' advice and attitudes complicated their decisions to reject life-prolonging treatment for their elderly parents.

The effectiveness of end-of-life care simulation in undergraduate nursing education: A randomized controlled trial.

BACKGROUND: Nursing students have limited opportunities to experience end-of-life care, so it is difficult for them to learn how to deliver it empirically. The use of simulations with standardized patients may be a way to provide realistic experience of end-of-life care for nursing students. OBJECTIVES: The aim of this study was to evaluate the effectiveness of end-of-life care simulations with standardized patients in improving the knowledge, skill performance and self-confidence of undergraduate nursing students. DESIGN: Randomized controlled study. SETTING: Japanese university nursing school. PARTICIPANTS: Thirty-eight students in the third year of the Bachelor's degree in nursing (simulation group = 20, control group = 18). METHODS: After randomization to a simulation or control group, participants in the simulation group participated in an end-of-life care simulation with standardized patients. The primary outcome of a change in the knowledge score was assessed using a knowledge questionnaire, skill performance by completing Objective Structured Clinical Evaluations, and self-confidence related to end-of-life care by self-reported questionnaires. RESULTS: The simulation group improved significantly in knowledge, skill performance in physical assessment and psychological care, and self-confidence related to end-of-life care. The results of the two-way analysis of variance showed a significant interaction between groups and time (p = 0.000). Analysis of the simple main effect showed a significant difference (p = 0.000) between groups after the end-of-life care simulation and a significant difference (p = 0.000) over time in the simulation group. There were large effects on knowledge improvement (η2 = 0.372), physical assessment (η2 = 0.619), psychological care skill performance (η2 = 0.588), and self-confidence in both physical assessment (η2 = 0.410) and psychological care (η2 = 0.722). CONCLUSIONS: End-of-life care simulation with standardized patients would be an effective strategy to train nursing students, who have limited opportunities to experience end-of-life care.

Prognostic factors in patients who received end-of-life chemotherapy for advanced cancer.

BACKGROUND: Clinical efficacy of aggressive end-of-life (EOL) chemotherapy remains unclear. METHOD: Medical records of patients with advanced cancer between August 2011 and August 2016 were retrospectively analyzed. The primary endpoint was to identify prognostic factors at the last administration of chemotherapy. The secondary endpoint was to analyze the relationship between EOL symptoms and EOL treatment details. RESULTS: Among 300 evaluated patients, the number of patients who died within 14 and 30 days from the last administration of chemotherapy were 16 (5.3%) and 50 (16.7%), respectively. Multivariate analysis revealed that ECOG-PS (OR 3.698, p < 0.001) and GPS2 (OR 3.791, p = 0.028) were significant prognostic factors. The MST of patients with both PS 2-4 and GPS2 (+) was 38 days, while that in patients with both PS 0-1 and GPS2 (-) was 134.5 days. The prevalence rate of nausea and vomiting (25.0%) and the mean hydration volume (0.50 L/day) in patients who died within 30 days from the chemotherapy was significantly higher than others (7.4%) (0.20 L/day). CONCLUSION: ECOG-PS and GPS were significant prognostic factors for aggressive EOL chemotherapy. Information on these factors may aid clinical decision-making in terms of risk-benefit balance, particularly in patients with poor prognosis.

Fetal Sirenomelia Associated with an Abdominal Cyst Originating from a Saccular Cloaca.

A 40-year-old pregnant woman presented with a fetal abdominal cyst and oligohydramnios. Color Doppler scan revealed a single blood vessel from the fetal aorta into a single umbilical artery. Severe oligohydramnios limited ultrasonographic evaluation of the fetal lower limbs, kidneys, or bladder. The pregnancy was terminated; the fetus showed fused lower limbs, bulging abdomen, and absent external genitalia and was diagnosed with type III sirenomelia. On autopsy, no normal bladder was observed, but duodenal atresia, anorectal atresia, and right renal agenesis were found. An intra-abdominal cyst, diagnosed histologically as a saccular cloaca, occupied the abdominal cavity. Ultrasonographic diagnosis of fetal sirenomelia is difficult due to poor depiction of the lower limbs. A vitelline artery leading to a single umbilical artery and a fetal abdominal cyst occupying most of the abdominal cavity are considered fetal sirenomelia associated with large defects of the gastrointestinal and genitourinary tracts.

Lymphatic vessel involvement is predictive for lymph node metastasis and an important prognostic factor in endometrial cancer.

BACKGROUND: Lymphovascular space involvement is reported to be an important risk factor in endometrial cancer. This study was conducted to evaluate the separate prognostic effects of lymphatic invasion and venous invasion on the outcomes of patients with endometrial cancer. METHODS: From 2006 to 2013, 189 histologically confirmed endometrial cancer patients were examined. To study the venous invasion (v) of the endometrial cancer, Victoria blue-H&E staining-which positively stains the elastic fibers of vessels-was performed. Immunohistochemical staining with D2-40 was used to study the lymphatic invasion (ly) of the endometrial cancer. RESULTS: The median age of the patients was 57 (range 25-84) years. ly(+) and/or v(+) patients were significantly more likely to present an advanced cancer stage, G3 tumor, and deep myometrial invasion than ly(-)/v(-) patients. The incidence of lymph node metastasis was high in ly(+) patients, and that of ovarian metastasis was high in v(+) patients. Lymphatic vessel invasion was significantly correlated with regional lymph node metastasis. We found a significantly higher incidence of distant metastasis in ly(+) patients. Most recurrences in ly(+)/v(-) patients occurred in lymph nodes, while those in ly(+)/v(+) patients occurred mainly at distant organs. Finally, the prognosis was significantly poorer for ly(+) patients, in whom lymphatic invasion was an independent prognostic factor along with distant metastasis. CONCLUSIONS: Our study suggests that by separately evaluating lymphatic invasion and blood vessel invasion in endometrial cancer cases, useful information for predicting lymph node metastasis and recurrence sites as well as prognostic information can be obtained.

Nurses' Experiences of End-of-life Care in Long-term Care Hospitals in Japan: Balancing Improving the Quality of Life and Sustaining the Lives of Patients Dying at Hospitals.

PURPOSE: In Japan, about 80% of deaths occur in hospitals, especially long-term care beds. The purpose of this study was to clarify the nursing practices used for such older patients at the end-of-life stage in long-termcare wards via the modified grounded theory approach (M-GTA). METHODS: Data were obtained through semi-structured interviews of nineteen nurses working in cooperating long-term care wards, acute care wards, or hospice services (to allow for constant comparison between these types of wards) in western Japan in 2014. We analyzed the transcribed data using M-GTA. RESULTS: The core category that emerged from the analysis was "Balancing enhancement of patients' daily life quality and life-sustaining care in the face of uncertainty about the patients' character." Eleven categories emerged, such as Seeking older patients' character with their family, Supporting families' decision making, Rebuilding patients' daily life in the ward, and Sustaining patients' life span through medical care. CONCLUSIONS: Nurses experienced uncertainty about the care needs of older patients, the ethical problems of Enhancing the patients' QOL by using risky care, and the evaluation criteria used to judge their own nursing care after the patients' death. All nurses had the goal of ensuring a natural death for all patients. Nurses' acceptance and evaluation of their own care was critically influenced by the patient's family's responses to their care after patients' death. Further research is necessary to develop evaluation criteria and educational programs for end-of-life nursing care of older adults.

Clinicopathologic Diversity of Undifferentiated Sarcoma With BCOR-CCNB3 Fusion: Analysis of 11 Cases With a Reappraisal of the Utility of Immunohistochemistry for BCOR and CCNB3.

Undifferentiated sarcoma harboring the BCOR-CCNB3 fusion is characterized by its predilection to affect skeletons of adolescent males, cellular small round/spindle cell morphology, and CCNB3 immunoreactivity. We analyzed 11 cases of BCOR-CCNB3 sarcoma, 10 of which were identified in a reverse transcription-polymerase chain reaction-based screen of 85 patient samples recorded in our database as unclassified small round or spindle cell sarcomas. BCOR rearrangements were confirmed by fluorescence in situ hybridization in 8 tumors. All patients were males aged between 6 and 31 years. In addition to 5 tumors in soft tissue and 4 in the axial or appendicular skeletons, which are typical locations, a tumor was located in the paranasal sinus and another in the lung. Microscopically, the tumors comprised proliferating atypical spindle and/or small round cells with diverse morphologic features such as small concentric whorls, myxoid stroma, a hemangiopericytomatous appearance, and/or hyalinized collagen resembling a solitary fibrous tumor, and angiomatous or slit-like spaces containing extravasated erythrocytes. Tumor cells were immunoreactive to CCNB3 (9/11), BCOR (10/10), TLE1 (6/10), bcl-2 (9/11), CD99 (8/10), CD56 (8/10), c-kit (4/10), and cyclin D1 (10/10). In an immunohistochemical analysis of an additional 412 small round or spindle cell tumors, CCNB3 was detected in 6 (1.5%) and BCOR in 18 (4.4%). Our analysis highlights the varying clinicopathologic features of this tumor, which partially overlap with other small round or spindle cell tumors, including solitary fibrous tumor and vascular tumors. Because CCNB3 and BCOR immunohistochemistry lacks adequate sensitivity and specificity, a molecular genetic approach remains essential for diagnosis.

Primary Villoglandular Mucinous Adenocarcinoma of the Vulva.

Primary villoglandular mucinous adenocarcinoma of the vulva is rare tumor. We report a case of 68-year-old woman who developed this very uncommon malignant tumor. Immunohistochemical examination of this tumor revealed positive staining for Cytokeratin 20, Mucin 2, and CDX2, although Cytokeratin 7 and Mucin 6 were negative. This positive staining indicated the tumor enteric type characters. In order to exclude the possibility of the metastasis from another site, we thoroughly evaluated clinical data and extensively investigated the whole body. However, we could not detect any other tumors. The patient was treated by tumor resection. The patient remains free of disease 5 years after surgery.

Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Multidisciplinary Approach.

Background. A rare case of low-grade endometrial stromal sarcoma (LG-ESS) extending to inferior vena cava (IVC) and cardiac chambers. Case Report. A 40-year-old woman had IVC tumor, which was incidentally detected by abdominal ultrasonography during a routine medical checkup. CT scan revealed a tumor in IVC, right iliac and ovarian veins, which was derived from the uterus and extended into the right atrium and ventricle. The operation was performed, the heart and IVC were exposed, and cardiopulmonary bypass was initiated. A right atriotomy was performed, and the intracardiac mass was removed. Then the tumor in IVC and the right internal iliac vein were removed after longitudinal venotomies in the suprarenal and infrarenal vena cava, the right common iliac vein. Next the pelvis was explored. Tumors were found originating from the posterior wall of the uterus and continuing into both the right uterine and ovarian vein. The patient underwent total hysterectomy with bilateral salpingooophorectomy. Complete tumor resection was achieved. Histopathological analysis confirmed a diagnosis of LG-ESS. She showed no evidence of disease for 2 years and 3 months. Conclusions. Our case highlights the importance of a multidisciplinary approach in treating this rare cardiovascular pathological condition through preoperative assessment to final operation.