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Background: To report the diagnosis and treatment of a rare disease of intravenous leiomyomatosis (IVL) originating from the uterus, growing in the inferior vena cava (IVC) and extending into the right atrium (RA) associated with a pelvic arteriovenous fistula (AVF). This is the first reported case of IVL in the IVC and RA with pulmonary benign metastasizing leiomyoma (PBML) secondary to a pelvic AVF despite the use of GnRH agonists in a nonmenopausal woman., Case Presentation: The patient was a 50-year-old premenopausal woman with a history of surgical resection for and antiestrogen conservative drug for pulmonary benign metastasizing leiomyoma (PBML) 5 years. The patient nevertheless developed IVL in the IVC, internal iliac vein and RA accompanied by AVF. Vaginal ultrasound combined with echocardiography and computerized tomographic venography imaging assists in the diagnosis of IVL combined with AVF, with histopathology and immunohistochemistry ultimately confirming the diagnosis. The patient ultimately was performed with a combination of hysterectomy, bilateral adnexectomy, and resection of tumors in the IVC and RA without cardiopulmonary bypass and sternotomy., Conclusion: BML may be difficult to control with incomplete removal of the uterus and ovaries even with the use of antiestrogenic medications, and medically induced AVF resulting from fibroid surgery may accelerate this process and the development of IVL., Competing Interests: Declaration of competing interest We declare that we have no financial and personal relationships with other people or organizations that can inappropriately influence our work; there is no professional or other personal interest of any nature or kind in any product, service and/or company, (Copyright © 2024 Elsevier Inc. All rights reserved.)

Uterine leiomyoma invading internal iliac vein and consequently disseminating into the right atrium is an extremely rare condition, and surgical strategy is controversial. Here, we reported a specific case with successful surgical resection through one-stage total hysterectomy, bilateral oophorectomy, and the intracardiovascular lesion. This procedure would be an optimal choice for uterine leiomyoma invading inferior vena cava and spreading to right atrium., (© 2024. The Author(s).)

Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: This case report aims to present a rare and challenging clinical scenario involving diffuse uterine leiomyomatosis (DUL), an infrequently encountered benign uterine pathology. The primary objective is to describe this unique case's clinical presentation, diagnostic challenges, and subsequent management., Methods: A detailed analysis of the patient's medical history, diagnostic evaluations, and treatment approach was conducted. A relevant literature review on DUL was also performed. This case report includes high-resolution images and figures, showcasing MRI scans, surgical procedures, and histopathology slides related to the case., Results: The case report outlines the clinical journey of a patient with DUL, an exceptionally rare uterine condition characterized by the uncontrolled proliferation of smooth muscle cells forming nodules and fascicles. This case illustrates the diagnostic complexities associated with DUL, as it mimics other uterine pathologies such as leiomyomas or adenomyosis. The patient's clinical presentation included abnormal uterine bleeding, heavy menstrual bleeding, fertility issues, and dysmenorrhea, leading to initial misdiagnoses. Surgical intervention eventually addressed the condition with corresponding images illustrating the procedures., Conclusion: This case report highlights DUL's rarity and diagnostic challenges. Clinicians must be vigilant when encountering similar clinical presentations, ensuring a comprehensive diagnostic workup to differentiate DUL from other uterine pathologies. Enhanced awareness among healthcare providers and further research into DUL's pathophysiology is essential for accurate diagnosis and timely management. The presented case underscores the need for tailored approaches to managing DUL and expanding the knowledge base surrounding this puzzling uterine disorder., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Reed's syndrome (RS) is a rare autosomal-dominant disorder characterised by multiple cutaneous and uterine leiomyomas, with a strong tendency for renal cell carcinoma (RCC) development. A woman in her 50s, who had previously undergone total abdominal hysterectomy due to multiple uterine leiomyomas, presented with painful nodules on her trunk and right arm for the past 6 years. These nodules were confirmed as leiomyomas through histopathology. Diagnosis of RS was established through clinicopathological correlation and positive family history, particularly her mother's. Early-onset uterine leiomyomas in patients with a similar family history should raise suspicion for RS, necessitating vigilant long-term follow-up. RCC detection requires mandatory renal imaging. Screening family members and providing genetic counselling are crucial., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: The aim was to explore the magnetic resonance imaging (MRI) features of stage-I intravenous leiomyomatosis (IVL)., Materials and Methods: From January 2019 to January 2023, clinical, pathological, and MRI data were collected from 19 cases confirmed by surgical pathology. Two radiologists retrospectively measured the tumor sizes, T1WIs, T2WIs, and ADC values and evaluated contrast-enhanced T1WIs, DWIs, complications and parauterine infiltrations. The number of tumor cells and the total nuclear area were measured. The percentage of tumor cell area out of the total area was used as the tumor cell density., Results: Nineteen patients with stage-I IVL aged 33 to 66 years (mean age: 46 ± 7.6 years) were included in this study. All 19 cases were located in the myometrium or parametrium, with a mean diameter of 11.2 ± 4.8 cm. Among these cases, 14 (73.6%) were associated with leiomyoma, and six (31.6%) involved the broad ligament. Isointensity was observed in the T1WIs of 12 cases (63.2%), while slight hypointensity was seen in five patients (26.3%). Isointensity was observed in the on T2WIs of four cases (21.1%), and iso- or slight hyperintensity was observed in 15 cases (78.9%). A significant difference was detected between the normalized T2WIs of IVL and myometrium (p < 0.001). A Pearson correlation test showed demonstrated a negative correlation between the ADC and tumor cell density values (r = - 0.946, p < 0.001). Tortuous vessels were present in 17 cases (89.5%) within or next to the lesions, and multiple winding cord-like filling defects were seen in 11 cases (57.9%) within the tortuous vessels on the T2WIs., Conclusion: Identifying the characteristic MRI features of stage-I IVL helped improve the diagnostic accuracy achieves for this rare tumor. Stage-I IVL often presents as a large mass accompanied by leiomyoma, and it easily invades the broad ligament. TIWI signals exhibited isointensity, and T2WI signals contained iso- or slight hyperintensity. Tortuous vessels were present within or next to the lesions, and multiple winding cord-like filling defects were observed within the tortuous vessels on the T2WIs., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: This study provides a concise overview of diagnostic and treatment strategies for intravenous leiomyomatosis (IVL), a rare disease with nonspecific clinical manifestations, based on cases from a tertiary referral hospital in China., Methods: We retrospectively analyzed 11 premenopausal patients with confirmed IVL between 2018 and 2022. Clinical data from Ultrasound, Enhanced CT, and MRI were studied, along with surgical details, postoperative pathology, and follow-up information., Results: Premenopausal patients showed no disease-specific symptoms, with 90.9% having a history of gynecological or obstetric surgery, and 72.7% having prior uterine fibroids. Cardiac involvement was evident in two cases, with echocardiography detecting abnormal floating masses from the inferior vena cava. Pelvic ultrasound indicated leiomyoma in 90.9% of cases, with ≥ 50 mm size. Surgery was the primary treatment, and lesions above the internal iliac vein resulted in significantly higher intraoperative blood loss (median 1300 ml vs. 50 ml, p = 0.005) and longer hospital stays (median 10 days vs. 4 days, p = 0.026). Three patients with lesions above the inferior vena cava required combined surgery with cardiac specialists. Recurrence occurred in 2 out of 11 patients with incomplete lesion resection., Conclusions: IVL mainly affects premenopausal women with uterine masses, primarily in the pelvic cavity (Stage I). Pelvic ultrasound aids early screening, while Enhanced CT or MR assists in diagnosing and assessing venous lesions. Complete resection is crucial to prevent recurrence. Lesions invading the internal iliac vein and above pose higher risks during surgery. A multidisciplinary team approach is essential for patients with lesions above the inferior vena cava, with simultaneous surgery as a potential treatment option., (© 2023. The Author(s).)

Abstract: A 35-year-old woman with a history of laparoscopic myomectomy presented with repeated abdominal pain. Contrast-enhanced abdominal and pelvic CT showed multiple enhancing solid or mixed cystic and solid peritoneal masses, and an enhancing uterine mass. All these masses showed intense FDG uptake on FDG PET/CT. The intraperitoneal and uterine masses were surgically removed. The histological and immunohistochemical findings of the peritoneal lesions were consistent with leiomyomatosis peritonealis disseminata with fumarate hydratase deficiency, and the uterine mass was adenomyosis. This case indicates fumarate hydratase-deficient extrauterine leiomyoma can show intense FDG uptake mimicking malignancy., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To assess factors influencing preoperative diagnosis and hemorrhage during surgery with uterine intravenous leiomyomatosis., Methods: This retrospective single-institution study used univariate analysis and multivariate models to investigate potential factors contributing to preoperative diagnosis and hemorrhage during surgery associated with intravenous leiomyomatosis in 135 patients from January 2012 to April 2022. Risk factors for disease recurrence were also investigated. The SPSS statistical analysis package was used for data analysis., Results: Previous myomectomy or fibroid ablation and tumor location on color Doppler were related to preoperative diagnosis (P = 0.031 and P = 0.003, respectively). Multivariate regression analysis demonstrated that lesions extending to the broad ligament were the only factors affecting preoperative diagnosis (odds ratio [OR] 5.383, 95% confidence interval [CI] 1.49-19.47). Univariate analysis showed that previous myomectomy or fibroid ablation (P = 0.017), tumor location (P = 0.027), and parauterine involvement (P = 0.014) were associated with intraoperative hemorrhage. Parauterine involvement was an independent risk factor for increased bleeding (OR 1.36, 95% CI 1.14-3.92). Six patients (4.4%) relapsed. The present study demonstrated that age (P = 0.031) and surgical type (P < 0.001) might be associated with disease recurrence., Conclusions: Treatment emphasis should focus on lesions extending to the broad ligament. Intraoperative bleeding associated with parauterine involvement should be stopped as effectively as possible., (© 2023 International Federation of Gynecology and Obstetrics.)

Abstract: Intravenous (IV) leiomyomatosis is a rare IV disease. Our case presents 68 Ga-FAPI and 18 F-FDG PET/CT findings of IV leiomyomatosis in a 37-year-old woman. Intravenous leiomyomatosis shows only mild FDG but intense 68 Ga-FAPI activity on PET/CT studies. In this case, 68 Ga-FAPI was superior to 18 F-FDG PET/CT in detecting IV leiomyomatosis., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Intravenous leiomyomatosis is a rare benign vascular tumor. Intracardiac extension is infrequently but can lead into a threating-life situation. We report a 41-year-old woman who has undergone previous hysterectomy due to uterine myomatosis and now presents with a pelvic mass contacting the venous system through the right internal iliac vein and extending up to the right pulmonary artery. Surgical resection of the pelvic mass and intravenous tumor removal was successfully performed in a single-stage approach with cardiopulmonary bypass. Patient recovered satisfactorily, being asymptomatic at hospital discharge time. One-stage surgical approach needs a multidisciplinary surgical team and needs a longer operative time than two-stage approach. However it can be a safety treatment option for patients with good general condition. Furthermore cardiopulmonary bypass guarantees a safe procedure, avoiding renal and hepatic ischemia and potential embolizations into pulmonary circulation during mass or ilio cava venous sector manipulation.

Objective: To provide perspectives on preoperative diagnosis and conservative treatment for diffuse uterine leiomyomatosis (DUL)., Methods: The clinical characteristics, management, and outcome of the five cases diagnosed with DUL receiving surgical treatment at Peking Union Medical College Hospital between January 2010 and December 2021 were retrospectively analyzed., Results: DUL is a diagnosis based on histopathology. It is a subtype of uterine leiomyoma, characterized by innumerable, poorly circumscribed hypercellular nodules of bland smooth muscle cells with no cytologic atypia diffusely involving the myometrium. Clinical manifestations, including menorrhagia, anemia, and infertility, are similar to those of typical uterine leiomyomas, making a definitive preoperative diagnosis difficult. Magnetic resonance imaging plays an important role in the pre-treatment mapping. Conservative surgery can reduce the uterine volume and improve the contour of the uterine cavity, thereby relieving symptoms of menorrhagia and improving the chance of conception. Gonadotropin-releasing hormone (GnRH) agonist therapy is of great significance for controlling vaginal bleeding, reducing uterine volume, and delaying postoperative recurrence, and can be used alone or as postoperative adjuvant therapy for conservative surgery., Conclusion: The treatment goal for DUL patients with fertility-sparing request should not aim at complete fibroid removal. A successful pregnancy can be achieved following conservative surgery and/or GnRH agonist therapy., (© 2023 International Federation of Gynecology and Obstetrics.)

Background: Intravenous leiomyomatosis (IVL) is a rare and specific type of smooth muscle tumor that is histologically benign but has a malignant biological behavior. It is commonly associated with a history of uterine leiomyomas., Case Presentation: A 36-year-old woman, G1P1, presented to the hospital with left lower abdominal pain for 2 months and she has accepted hysteroscopic myomectomy about 1 year ago. Ultrasound venography, echocardiography and computed tomography venography (CTV) of inferior vena cava were performed, which revealed IVL located in left intramural myometrium walls growing along the left ovarian vein reaching the level of the lumbar 5-sacral 1 disc. Laparoscopic bilateral salpingo-oophorectomy and hysterectomyis were scheduled. The IVL in the left ovarian vein and parauterine venous plexus were detected and excised completely during surgery. IVL was diagnosed by postoperative pathology and immunohistochemistry. The patient recovered well after surgery. No surgical-related or anesthesia-related complications occurred.The 3-month follow-up CTV of inferior vena cava and echocardiography examination revealed normal., Conclusions: The cause of IVL is unknown, this observation demonstrates that hysteroscopic myomectomy might lead to the occurrence of IVL., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Aim: To review and summarise the clinical and imaging characteristics of intravenous leiomyomatosis (IVL), a rare smooth muscle tumour originating from the uterus., Materials and Methods: Twenty-seven patients with a histopathological diagnosis of IVL who underwent surgery were reviewed retrospectively. All patients underwent pelvic ultrasonography, inferior vena cava (IVC) ultrasonography, and echocardiography before surgery. Computed tomography (CT) with contrast enhancement was performed in patients with extrapelvic IVL. Some patients underwent pelvic magnetic resonance imaging (MRI)., Results: Mean age was 44.81 years. Clinical symptoms were non-specific. IVL was intrapelvic in seven patients and extrapelvic in 20. Preoperative pelvic ultrasonography missed the diagnosis in 85.7% of patients with intrapelvic IVL. Pelvic MRI was useful to evaluate the parauterine vessels. Incidence of cardiac involvement was 59.26%. Echocardiography showed a highly mobile sessile mass in the right atrium with moderate-to-low echogenicity that originates from the IVC. Ninety per cent of extrapelvic lesions showed unilateral growth. The most common growth pattern was via the right uterine vein-internal iliac vein-IVC pathway., Conclusion: The clinical symptoms of IVL are non-specific. For patients with intrapelvic IVL, early diagnosis is difficult. Pelvic ultrasound should focus on the parauterine vessels, the iliac and ovarian veins should be explored carefully. MRI has obvious advantages in evaluating parauterine vessel involvement, which is helpful for early diagnosis. For patients with extrapelvic IVL, CT should be performed before surgery as part of a comprehensive evaluation. IVC ultrasonography and echocardiography are recommended when IVL is highly suspected., (Copyright © 2023 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Background: Intravascular leiomyomatosis (IVL) is a histologically benign smooth muscle tumor arising from the uterus that can spread through the pelvic veins and, on rare occasions, extend as far as the heart via the inferior vena cava. Despite its benign characteristics, it can behave like a malignant tumor leading to significant morbidity and even mortality if left untreated., Case Presentation: The patient is a 42-year-old woman with a past medical history of uterine leiomyomas. She presented with heavy bleeding and frequent spotting; therefore, she went to her gynecologist. After further evaluation, a mass within the uterus that expanded into the pelvic veins, inferior vena cava, and right atrium was discovered. After the complete removal of the mass, the patient underwent full recovery. IVL with cardiac extension was the final diagnosis., Conclusion: Although IVL is rare, it must be considered in women who underwent previous hysterectomies or myomectomies and present with symptoms of right heart failure. The ideal therapy will need the aid of a multidisciplinary team and will depend on the patient's symptoms, previous operative history, the tumor's extension, and resectability., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Intravenous leiomyomatosis (IVL) is a rare condition characterized by a tumor that originates within a pelvic vein wall or the uterine smooth muscle, with extension into the right heart (referred to as intracardiac leiomyomatosis) in approximately 10% of all IVL cases. Usually, computed tomography (CT) or magnetic resonance imaging (MRI) is performed for diagnostic imaging of IVL. Notably, this neoplasm presents with characteristic ultrasonographic findings. In this report, we present the case of a 49-year-old woman with IVL, which extended into the right heart. Echocardiography combined with abdominal ultrasonography was useful to outline the course of the tumor from the right heart to the uterus. Our findings suggest that in addition to CT or MRI, ultrasonography shows high diagnostic value in cases of IVL, and ultrasonography combined with CT or MRI can further improve the preoperative diagnosis rate of IVL., (© 2023 Wiley Periodicals LLC.)

Intravascular leiomyomatosis (IVL) is a rare benign smooth muscle neoplasm growing within the pelvic venous system, often with caval and intracardiac extension. It frequently coexists with uterine leiomyomas or occurs in women with a history of myomectomy or hysterectomy. IVL is often asymptomatic until intracardiac extension occurs, and carries a risk of sudden death, necessitating timely diagnosis and management. We present a case of IVL diagnosed on hysterectomy specimen with extension to the inferior vena cava found on follow-up imaging. The patient underwent complete resection with multidisciplinary involvement of Gynaecological Oncology and Vascular Surgery and remains disease free following 12 months of follow-up., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Study Objective: To show laparoscopic management of disseminated peritoneal leiomyomatosis (DPL)., Design: Stepwise demonstration of the technique with narrated video footage., Setting: DPL is characterized by dissemination and proliferation of peritoneal and subperitoneal lesions primarily originating from smooth muscle cells [1]. Generally considered benign, cases of malignant transformation to leiomyosarcoma have been reported [2,3]. Iatrogenic DPL occurs because of unconfined morcellation resulting in small fragments of myoma that may implant on any organ and start deriving blood supply from it or may be pulled into port site while withdrawing laparoscopic cannulas [4]. It is estimated that the overall incidence of DPL after laparoscopic uncontained morcellation was 0.12% to 0.95% [5]. Mainstay of treatment is surgical resection of myomas and regular follow-up with imaging. A 28-year-old unmarried girl presented with complain of lump abdomen increasing in size for 1 year. She also complained of a 15 kg weight loss in the last 1 year; 4 years ago, patient had undergone laparoscopic myomectomy with unconfined morcellation for a 10 × 8 cm cervical myoma. Presently her menses were regular with a 28-day cycle and 3 to 4 days' average flow. Magnetic resonance imaging showed multiple nodular lesions of varying sizes in relation to small bowel, colon, uterus, and anterior abdominal wall  suggestive of DPL. Bilateral ovaries were normal. Tumor markers were as follows: CA 125 23.2 (<35) U/mL Carcinoembryonic antigen 1.67 (<8) ng/mL CA 19-9 47 (<37) U/mL Lactate dehydrogenase 809 (180-360) IU/L Alpha-fetoprotein 2.03 (<10) ng/mL Beta human chorionic gonadotropin 1.2(<2) mIU/mL Tru-cut biopsy was done elsewhere to rule out peritoneal carcinomatosis in view of raised CA 19-9 and lactate dehydrogenase, history of weight loss, and imaging showing multiple abdominal masses. Histopathological examination showed leiomyomatosis and immunohistochemistry for smooth muscle actin, desmin, and vimentin were positive., Interventions: On laparoscopy the abdominal cavity was found studded with multiple leiomyomas of varying sizes deriving blood supply from ilium, transverse, descending and sigmoid colon, rectum, left tube, left ovary, pouch of Douglas, bilateral uterosacrals, uterovesical fold, and anterior abdominal wall. Large blood vessels were seen traversing between the descending and sigmoid colon and the myomas. Principles of surgery were as follows: 1. Complete removal of myomas 2. Cauterization of blood vessels feeding the parasitic myomas to minimize blood loss 3. Disscetion abutting the myoma to prevent injury to adjacent viscera. A total of 26 myomas were removed. All the myomas were retrieved by morcellation in a bag. Histopathology confirmed the diagnosis of diffuse peritoneal leiomyomatosis. Follow-up ultrasound at 6 months showed no recurrence of leiomyomatosis., Conclusion: Proper mapping of lesions and surgery for complete removal of all masses is the mainstay of treatment. Contained morcellation in bag should be the norm to prevent iatrogenic DPL. Regular follow-up with imaging is required to rule out recurrence., (Copyright © 2023 AAGL. Published by Elsevier Inc. All rights reserved.)

Objectives: Describe the radiographic features of the different forms of extrauterine leiomyomatosis., Conclusions: Leiomyomas with a rare growth pattern occur most often in women of reproductive age and with a history of hysterectomy. Extrauterine leiomyomas present a greater diagnostic challenge because they may mimic malignancies, and serious diagnostic errors may result., (Copyright © 2023 SERAM. Published by Elsevier España, S.L.U. All rights reserved.)

Objective: To describe the reproductive outcomes of patients with diffuse uterine leiomyomatosis (DUL) treated with high-intensity focused ultrasound (HIFU) ablation., Materials and Methods: Three patients of reproductive age with DUL who underwent HIFU treatment were enrolled, all of whom had a strong desire to become pregnant. All patients underwent routine laboratory tests, electrocardiography (ECG), chest X-ray radiography, ultrasound, and magnetic resonance imaging (MRI) examinations after routine medical history collection and physical examination. The treatment time, treatment power, sonication time, and adverse events were recorded. One day after HIFU, MRI was performed to evaluate treatment efficacy. The patients were scheduled for follow-up at 3-, 6-, 12-, and 24-month after HIFU treatment., Results: All the three patients completed HIFU treatment successfully without any major complication. Uterine size and menstrual volume significantly decreased with the combination of medical and HIFU treatments. The shrinkage rate of uterine volume was 31-44% and the menstrual volume reduced by 1/2 or returned to normal at 3 months post-HIFU. Three patients had successful conceptions between 3 and 11 months after HIFU with healthy deliveries. No uterine rupture occurred during pregnancy and delivery., Conclusion: HIFU ablation may help achieve a successful pregnancy in patients with DUL.

Objective: To show a case of severe intravascular leiomyomatosis with intracardiac extension treated by a multidisciplinary minimally invasive surgery., Design: Stepwise demonstration of the technique with a video., Setting: General Hospital., Patient(s): A 40-year-old woman with palpitation and dyspnea., Intervention(s): The patient was diagnosed with intravascular leiomyomatosis by computed tomography scan. She underwent a successful single-stage minimally invasive surgery with complete excision., Main Outcome Measure(s): The feasibility and safety of using this technique for intravascular leiomyomatosis with intracardiac extension., Result(s): A combined thoracoabdominal surgery was successfully performed. During the procedure, cardiopulmonary bypass was maintained for 72 minutes. The patient soon recovered and was discharged., Conclusion(s): Minimally invasive surgery is a possible choice for intravascular leiomyomatosis with intracardiac extension., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Seven consecutive female patients with pathologically confirmed arteriovenous malformation (AVM) with intravenous leiomyomatosis (IVL) of the uterus (age range, 32-61 years; mean age, 43 years) treated between 2005 and 2021 from a single institution were reviewed. Computed tomography (CT) findings of congenital pelvic AVM of 10 female patients were compared with those of AVM with IVL. Characteristic CT findings of AVM with IVL revealed a focal soft tissue mass inside a dilated venous structure of the AVM. Multiple sessions of transvenous coil embolization of the AVM with or without the injection of ethanol were performed. After complete (6/7, 86%) or partial (1/7, 14%) embolization of the AVM, complete surgical resection of the IVL and embolized AVM mass was performed in 4 patients. Patients with lung metastasis or residual embolized AVM masses are under follow-up with antiestrogen hormonal therapy., (Copyright © 2022 SIR. Published by Elsevier Inc. All rights reserved.)

Purpose: Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is associated with an aggressive form of renal cell carcinoma with high risk of metastasis, even in small primary tumors with unequivocal imaging findings. In this study, we compare the performance of ultra-high b-value diffusion-weighted imaging (DWI) sequence (b = 2000 s/mm 2 ) to standard DWI (b = 800 s/mm 2 ) sequence in identifying malignant lesions in patients with HLRCC., Methods: Twenty-eight patients (n = 18 HLRCC patients with 22 lesions, n = 10 controls) were independently evaluated by three abdominal radiologists with different levels of experience using four combinations of MRI sequences in two separate sessions (session 1: DWI with b-800, session 2: DWI with b-2000). T1 precontrast, T2-weighted (T2WI), and apparent diffusion coefficient (ADC) sequences were similar in both sessions. Each identified lesion was subjectively assessed using a six-point cancer likelihood score based on individual sequences and overall impression., Results: The ability to distinguish benign versus malignant renal lesions improved with the use of b-2000 for more experienced radiologists (Reader 1 AUC: Session 1-0.649 and Session 2-0.938, p = 0.017; Reader 2 AUC: Session 1-0.781 and Session 2-0.921, p = 0.157); whereas no improvement was observed for the less experienced reader (AUC: Session 1-0.541 and Session 2-0.607, p = 0.699)., Conclusion: The inclusion of ultra-high b-value DWI sequence improved the ability of classification of renal lesions in patients with HLRCC for experienced radiologists. Consideration should be given toward incorporation of DWI with b-2000 s/mm 2 into existing renal MRI protocols., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Background Noninvasive in vivo detection of fumarate accumulation may help identify fumarate hydratase deficiency in renal cancer related to hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome. Purpose To investigate the feasibility of MR spectroscopy (MRS) in detecting elevated fumarate levels in HLRCC-associated renal cancers. Materials and Methods This study included an experimental xenograft mouse model and prospective clinical cohort. First, MRS was performed on patient-derived tumor xenograft models and control models to detect fumarate. Then, consecutive participants with clinical suspicion of HLRCC-associated renal tumors were enrolled. For the detection of fumarate, MRS results were classified as detected, borderline, undetected, or technical failure. The sensitivity, specificity, and accuracy of MRS for diagnosing HLRCC-associated renal cancer were assessed. The signal-to-noise ratio (SNR) of the fumarate peak was calculated and evaluated with receiver operating characteristic curve analysis. Results Fumarate peaks were detected at 6.54 parts per million in all three patient-derived xenograft models. A total of 38 participants (21 men; mean age, 47 years [range, 18-71 years]) with 46 lesions were analyzed. All primary HLRCC-associated renal cancers showed a fumarate peak; among the seven metastatic HLRCC-associated lesions, a fumarate peak was detected in three lesions and borderline in two. When only detected peaks were regarded as positive findings, the sensitivity, specificity, and accuracy of MRS at the lesion level were 69% (nine of 13 lesions), 100% (33 of 33 lesions), and 91% (42 of 46 lesions), respectively. When borderline peaks were also included as a positive finding, the sensitivity, specificity, and accuracy reached 85% (11 of 13 lesions), 88% (29 of 33 lesions), and 87% (40 of 46 lesions), respectively. The SNR of fumarate showed an area under the receiver operating characteristic curve of 0.87 for classifying HLRCC-associated tumors. Conclusion MR spectroscopy of fumarate was sensitive and specific for hereditary leiomyomatosis and renal cell carcinoma-associated tumors. © RSNA, 2022 Online supplemental material is available for this article.

Leiomyomatosis peritonei or disseminated peritoneal leiomyomatosis (DPL) is an extremely rare condition affecting mainly women of childbearing age with estrogen hypersecretion. It is characterized by the presence of multiple histologically benign smooth muscle nodules on the peritoneal surface and subperitoneal tissue mimicking peritoneal carcinomatosis. There are no clear guidelines for the primary management and follow-up o these patients. Case report: We represent a 44-years-old woman admitted due to two retroperitoneal tumors diagnosed on routine ultrasound screening. There was a past history of hysterectomy for uterine leiomyoma. The blood assay was normal. MRI revealed two well-circumcised tumors with a size of 15 cm and 6 cm, respectively, with a characteristic of leiomyoma. The tumors were removed completely. Macroscopically, they appeared as well-circumscribed masses with grey to white color, a rubbery texture, and a whorled cut surface. On the first postoperative day, due to a sudden drop of hemoglobin from 127 g/L to 6.8 g/L and clinical signs of acute intra-abdominal bleeding, the patient was reoperated. The site of bleeding was from the ileocolic vein located at the lower pole of the tumor. It was successfully controlled by ligation. The patient had an uneventful recovery. After five years she is free of recurrence. DPL is a rare disease, sometimes mimicking peritoneal carcinomatosis. The preoperative diagnosis should be suspected in women of childbearing age with estrogen hypersecretion and concurrent uterine leiomyomas. The management depends on the patient's age, reproductive plans, multiplicity, and symptomatology of DPL. Single tumors are best treated by surgery with synchronous hysterectomy or myomectomy in case of uterine myomatosis. In the case of symptomatic multiple DPL and fulfilled reproductive plan, bilateral salpingo-oophorectomy or treatment with Gonadotropin-releasing hormone analogs is indicated. In asymptomatic multiple DPL, the removal of all nodules is not mandatory because of the benign indolent course. The long-term prognosis of DPL is excellent.

We report the case of a woman in her 60s with intravascular leiomyomatosis. She suffered from numerous non-specific symptoms including weight loss, anaemia and sudden swelling of the left lower extremity. CT imaging showed the presence of an enlarged left ovary and a thrombus extending from the left ovarian venous plexus intruding into the right atrium of the heart. Cancer antigen 125 was 20 U/mL. Pelvic transvaginal ultrasound examination identified two normal ovaries and a mass adjacent to the left ovary. A second opinion on the CT scan was requested at a oncogynaecological multidisciplinary team meeting, where the radiologist of the team identified an intervascular leiomyomatosis. After further investigation, surgical treatment was planned and completed in collaboration with the departments of cardiothoracic and vascular surgery. The patient recovered fully., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Intravascular tumour extension can occur in many different types of cancer. Those with the highest tendency include renal cell carcinoma, adrenal cortical carcinoma and hepatocellular carcinoma. Inferior vena cava (IVC) tumour thrombus in gynaecological malignancy is rarely reported. We present a report on a female patient with extensive IVC tumour thrombus (intravenous leiomyomatosis) with concurrent intrauterine leiomyomatosis. She underwent a single-stage procedure, involving laparotomy and a sternotomy to remove her pelvic tumour, as well as the intracaval and intracardiac thrombus. The clinical presentation and management of this rare tumour will be detailed in this case report., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Intravenous leiomyomatosis (IVL) is a histologically benign but biologically aggressive tumor. This study aimed to summarize the echocardiography and contrast-enhanced ultrasound (CEU) characteristics of IVL to provide a basis for clinical diagnosis and therapy., Methods: Fourteen IVL patients with uterus leiomyoma history (female, 46.4 ± 5.6 years) were enrolled in this study from March 2008 to December 2020 in our hospital. Preoperative imaging examination data were collected, including echocardiography computed tomography data; six patients also underwent CEU. All patients underwent successful resection, confirmed by histopathology., Results: Echocardiographic characteristics: The mean sizes of intracardiac parts of IVL tumors were 54.0 ± 17.9 mm (length) and 24.6 ± 9.8 mm (width). IVL tumors exhibited two echocardiography types: isoechoic solid mass (71.4%, 10/14) and anechoic cystic conduits (28.6%, 4/14), with enlargements of the right atrium (57.1%,8/14), right ventricle (1 patient, 7.1%), and inferior vena cava (57.1%, 8/14). About 21.4% of the patients (3/14) had right ventricular dysfunction. Right heart obstruction was observed in 42.8% (6/14) of the patients. CEU characteristics: the solid mass type exhibited an earlier perfusion and lower perfusion intensity than the conduits type. CEU was helpful in determining origins and pathways: from the internal iliac vein (pathway I, 71.4%), from the ovarian vein (pathway II, 14.3%), or both (14.3%). The echocardiographic appearances of the 14 cases were consistent with the features of the resection specimens., Conclusion: Combined echocardiography and CEU can provide a more valuable information for the diagnosis of IVL and essential basis for treatment., (© 2021 American Institute of Ultrasound in Medicine.)

Objective: To investigate the clinical features, treatment and recurrence factors of intravenous leiomyomatosis (IVL) confined to the pelvic cavity after the operation. Methods: The clinical data of 81 patients who underwent surgery and were pathologically diagnosed with IVL at Women's Hospital, School of Medicine, Zhejiang University from January 2014 to March 2021, were analyzed retrospectively to explore the influencing factors of postoperative recurrence of IVL, including age, gravidity and parity, surgical methods, intraoperative conditions and so on. Results: (1) Clinical features: the age of 81 IVL patients was (43.9±8.1) years old; increased menstrual volume in 26 cases (32%, 26/81), prolonged menstrual period in 31 cases (38%, 31/81), frequency and urgency to urinate in 4 cases (5%, 4/81), abdominal pain and abdominal distension in 8 cases (10%, 8/81), and pelvic masses in 34 cases (42%, 34/81). IVL was diagnosed right in 4 of 72 patients (6%, 4/72) underwent preoperative ultrasound, right in 11 of 51 patients (22%, 11/51) underwent magnetic resonance imaging (MRI), and right in 4 of 19 patients (4/19) underwent CT. (2) Treatment: all patients with IVL underwent surgical treatment. Surgical procedure: myomectomy in 37 cases, total hysterectomy and bilateral salpingectomy in 19 cases, total hysterectomy and bilateral salpingo-ophorectomy in 25 cases. Surgical approach: hysteroscopic operation in 6 cases, transabdominal operation in 52 cases, laparoscopic operation in 23 cases. Fifty-three cases underwent rapid intraoperative pathological examination, 17 cases (32%, 17/53) of them were diagnosed right as IVL. (3) Influencing factors of IVL postoperative recurrence: among 81 patients with IVL, 3 cases were lost to follow-up, and 8 cases (10%, 8/78) had recurrence during follow-up. Age<35 years, number of pregnancies<2, number of births<2, number of fibroids ≥10, abnormal appearance of fibroids (long, vermicular, beadlike, cystic, etc.) and IVL invasion into adjacent vessels were all the risk factors influencing postoperative recurrence of IVL (all P <0.05). Methods of operation, ovariectomy or not, IVL invasion or not, maximum diameter of IVL, abundant blood vessels near uterine fibroids were not associated with postoperative recurrence of IVL (all P >0.05). Conclusions: The clinical manifestations and preoperative auxiliary examination of IVL are lack of specificity. Doctors need to pay attention to young patients with uterine fibroids, and choose the appropriate surgical scope when the characteristic manifestations of IVL be found during the operation, or the right diagnoses of IVL in the surgery's rapid intraoperative pathology be examined, and should remove the IVL lesions to reduce the recurrence as far as possible.

Study Objective: To show the challenging diagnosis of, and safe robotic surgical approach to, a rare case of disseminated peritoneal leiomyomatosis (DPL)., Design: A clinical case shown by a sequential demonstration of investigation, diagnosis, and surgical approach, with narrated video footage., Setting: DPL is a rare disease, with only a little more than 150 cases reported in the literature [1]. It is defined by subperitoneal proliferation of benign smooth muscle cell nodules, macroscopically mimicking peritoneal carcinomatosis [2]. The etiology remains unclear, but different hypotheses have been put forward, such as subperitoneal mesenchymal stem cell metaplasia and iatrogenic origin after myomectomy [3]. Despite its usual benign behavior, DPL can rarely present with malignant degeneration, and therefore a complete resection of multiple lesions is recommended [4]. This case involves a 45-year-old patient presenting with dysmenorrhea, abdominal pain, and major abnormal uterine bleeding, requiring previous blood transfusion and no response after 2 years of clinical treatment. She had a previous cesarean delivery, with no reproductive desire at present and no history of other pelvic surgeries. Pelvic examination showed an enlarged mobile uterus at the height of the pubic symphysis, and both ultrasonography and magnetic resonance imaging confirmed an enlarged uterus due to multiple myomas., Interventions: (1) Diagnostic laparoscopy with implant biopsies and uterine curettage for investigation of DPL and its differential diagnoses, followed by robot-assisted laparoscopic approach, with key strategies for a safe performance. (2) Radical hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and wide pelvic peritoneal resection were performed. (3) For this complex procedure, the identification and preservation of important landmarks and pelvic anatomy were mandatory, as well as removal of all surgical specimens in monobloc. Final pathology report: disseminated leiomyomatosis with no evidence of malignancy. The patient had no complications after surgery and was discharged on the second postoperative day with mild abdominal pain., Conclusion: DPL diagnosis can be tricky owing to its macroscopic similarity to peritoneal carcinomatosis and the difficulty of identification in imaging examinations. Moreover, the robotic platform can be a helpful and safe tool for the surgical treatment of DPL and complete resection of all peritoneal lesions., (Copyright © 2021 AAGL. Published by Elsevier Inc. All rights reserved.)