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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.)

Introduction: Intravenous leiomyomatosis (IVL), a rare type of uterine leiomyoma (its incidence is about 0.25% to 0.40% of patients who present uterine fibroma), is characterized by the formation and growth of benign leiomyoma tissue within the vascular wall or lymphatic lumen. Herein, we presented a case of early stage of IVL successfully treated by surgical removal and a review of actual medical recommendations., Observation: A 49-year-old woman, gravida 2 para 2, presented to our department with hypogastric pain. On physical examination, a palpable mass in the hypogastrium was noted. Pelvic ultrasound showed a huge uterus with multiple heterogeneous leiomyomas. As the patient was symptomatic and as she had completed their family plan, the decision to perform a total abdominal hysterectomy with bilateral salpingo-oophorectomy was taken. On pathological examination, intravascular growth of benign smooth muscle cell was found within venous channels lined by endothelium. The diagnosis of IVL of the uterus without malignant transformation was confirmed. The patient was monitored for 14 months, and subsequent computed tomography did not reveal any evidence of tumor recurrence., Conclusion: IVL is a benign, rare and potentially lethal pathology. Clinical manifestations are nonspecific. IVL needs surgical treatment for diagnosis and therapeutic purposes. They require close and prolonged follow-up because of the high risk of recurrence.

Background: Uterine Fibroids (UFs) are common benign tumors in the female reproductive tract, but their progression to intravascular leiomyomatosis (IVL) is rare. Presently, there are few reports on single-stage resection of UFs and IVL., Case Presentation: A 47-year-old woman, G2P2, had been diagnosed multiple UFs four years ago and now developed heart failure. Imaging examinations revealed that UFs had invaded the right iliac vein and extended into the right atrium through the inferior vena cava. Through multidisciplinary collaboration and a single-stage resection, the patient has survived for over 24 months post-surgery, and her heart function has significantly improved compared to preoperative levels, with no recurrence of UFs observed., Conclusions: Single-stage resection of IVL and UF is feasible and advantageous for this case, and selecting the appropriate surgical approach is crucial., (© 2024. The Author(s).)

Diffuse oesophageal leiomyomatosis is a rare benign disease in the paediatric population. This report highlights a recent clinical case, together with a narrative review of current world literature.An early middle childhood girl with recurrent lower respiratory tract infections for 2 years was noted to have a retrocardiac lesion on chest X-ray, later confirmed to be an oesophageal mass on CT imaging. She underwent an Ivor-Lewis oesophagogastrectomy and a Heineke-Mikulicz pyloroplasty. Pathology examination revealed type I diffuse oesophageal leiomyomatosis. Alport syndrome was later confirmed following an episode of postoperative haematuria, which was corroborated by electron microscopy examination following renal biopsy.With an oesophageal mass lesion and Alport syndrome, oesophageal leiomyomatosis should be suspected in any patient with a clinical history of dysphagia and/or respiratory symptoms. Endoscopic ultrasound-guided tissue biopsy is valuable for diagnosis of all suspected lesions. Surgical resection is mandatory to effect cure., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is an autosomal-dominant disorder that results from a germline pathogenic variant in the fumarate hydratase (FH) gene on chromosome 1, characterised by renal cell carcinoma (RCC), cutaneous leiomyoma and uterine leiomyoma. Leiomyosarcomas are reported in less than 1% of those with HLRCC. We report a case of a man in his 30s who had a long-standing plaque excised from the left upper arm after undergoing a radical nephrectomy for a fumarate-deficient RCC, with histological exam revealing a grade 1 leiomyosarcoma. Genetic testing confirmed a heterozygous pathogenic variant in the FH gene. This is a rare case of leiomyosarcoma associated with HLRCC, and our patient remains under surveillance with interval abdominal imaging and skin examination. Leiomyosarcomas are difficult to distinguish clinically from their benign counterpart; therefore, histopathological examination is paramount with a low threshold for excision., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Disseminated peritoneal leiomyomatosis (DPL) is a rare and benign clinical entity. It is also known as leiomyomatosis peritonealis disseminata (LPD). Here, we report and discuss a case of a primiparous woman in her early 40s who presented with heavy, prolonged, painful menses and heaviness in her lower abdomen. She underwent a laparoscopic myomectomy for a fibroid uterus, 12 months ago for similar complaints. On workup, she was diagnosed with DPL. We performed a total abdominal hysterectomy with bilateral salpingectomy, low anterior resection with stapled colorectal anastomosis and excision of peritoneal tumour deposits in consortium with the gastrosurgery team. Her postoperative period was uneventful, and the patient was discharged on postop day 6. Her histopathology report was consistent with leiomyoma; the follow-up period was uneventful., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To analyse the risk factors for post-operative recurrence or progression of intravenous leiomyomatosis and explore the impact of different treatment strategies on patient prognosis., Methods: Patients with intravenous leiomyomatosis who underwent surgery from January 2011 to December 2020 and who were followed for ≥3 months were included. The primary endpoint was recurrence (for patients with complete resection) or progression (for patients with incomplete resection). Kaplan-Meier survival analysis was used to analyse the factors affecting recurrence., Results: A total of 114 patients were included. The median age was 45 years old (range 24-58). The tumors were confined to the uterus and para-uterine vessels in 48 cases (42.1%), while in 66 cases (57.9%) it involved large vessels (iliac vein or genital vein and/or proximal large veins). The median follow-up time was 24 months (range 3-132). Twenty-nine patients (25.4%) had recurrence or progression. The median recurrence or progression time was 16 months (range 3-60). Incomplete tumor resection (p=0.019), involvement of the iliac vein or genital vein (p=0.042), involvement of the inferior vena cava (p=0.025), and size of the pelvic tumor ≥15 cm (p=0.034) were risk factors for recurrence and progression. For intravenous leiomyomatosis confined to the uterus or para-uterine vessels, no post-operative recurrence after hysterectomy and bilateral oophorectomy occurred in this cohort. Compared with hysterectomy and bilateral oophorectomy, the risk of recurrence after tumorectomy (with the uterus and ovaries retained) was significantly greater (p=0.009), while the risk of recurrence after hysterectomy was not significantly increased (p=0.058). For intravenous leiomyomatosis involving the iliac vein/genital vein and the proximal veins, post-operative aromatase inhibitor treatment (p=0.89) and two-stage surgery (p=0.86) were not related to recurrence in patients with complete tumor resection., Conclusion: Incomplete tumor resection, extent of tumor lesions and size of the pelvic tumor were risk factors for post-operative recurrence and progression of intravenous leiomyomatosis., Competing Interests: Competing interests: None declared., (© IGCS and ESGO 2024. Re-use permitted under CC BY-NC. No commercial re-use. Published by BMJ.)

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.)

Rationale: Cellular uterine leiomyomas (CL) represent the prevailing subtype among uterine leiomyomas. In this study, we report a case of recurrent peritoneal disseminated uterine fibroids 2 years after single-port laparoscopic gasless myomectomy. This article endeavors to examine the potential limitations of the aforementioned surgical procedure and outline the distinguishing features of recurrent cases with primary postoperative pathology as CL. Additionally, it aims to provide a summary of previous retrospective studies on CL and propose the existence of immunohistochemical molecules that may serve as predictors for the postoperative recurrence of cellular uterine fibroids. The ultimate objective is to enhance clinicians' comprehension of the disease., Patient Concerns: Two years ago, the patient underwent a single-port gasless laparoscopic myomectomy for uterine fibroids. Gynecological color Doppler ultrasound conducted 3 months ago revealed recurrence of uterine fibroids, and the patient experienced abdominal distension, mild urinary frequency, and constipation for the past month., Diagnoses: After the second surgical procedure, a comprehensive pathological examination and immunohistochemical analysis of both the uterine mass and metastatic lesions revealed that the definitive diagnosis was CLs., Interventions: The patient underwent the total hysterectomy, bilateral salpingectomy, pelvic adhesiolysis, omental mass resection, mesenteric mass resection, and pelvic peritoneal mass resection. All specimens were sent for rapid frozen examination and showed to be leiomyomas., Outcomes: The patient was discharged from the hospital on the 10th day after the operation. At the date of writing the article, the patient had no recurrence for 1 year and 5 months., Lessons: The single-port gasless approach did not achieve the desired reduction in fibroid recurrence, as anticipated by the surgeon. The act of pulling the tumor towards the abdominal incision for resection, on the contrary, may serve as an iatrogenic factor contributing to postoperative recurrence of CL into peritoneal dissemination leiomyomatosis. The single-port gasless assisted bag may be a more suitable option for myomectomy. The utmost effort should be made to prevent the potential recurrence of myoma caused by iatrogenic factors., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Diffuse esophageal leiomyomatosis is a rare esophageal tumor characterized by circumferential thickening of smooth muscle layers of the entire esophagus. CLINICAL CASE: Herein, we describe the case of a 19-year-old girl, who presented with a history of long-standing dysphagia. On evaluation she was found to have diffuse esophageal leiomyomatosis and was managed successfully by thoracoscopy-assisted esophagectomy with intra-nodal indocyanine green injection., Discussion: In this report, we discuss the pre-operative workup and our surgical approach to managing this rare entity. We also discuss the available literature on the subject and the lessons learnt in managing this complex condition., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, 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).)

Rationale: Intravascular/intravenous leiomyomatosis (IVL) is a peculiar variant of uterine leiomyoma that is classified as a histologically benign smooth muscle tumor with a biological behavior similar to that of a malignant tumor. It is characterized by the proliferation of leiomyomas spreading along the uterine and extrauterine venous circulation., Patient Concerns: Herein, we present 2 cases of IVL who had completely different clinical manifestations to stress the need for constant vigilance of IVL diagnosis and the understanding of uterine leiomyoma heterogenicity. Case 1 was registered for fever without specific triggering factors, irregular menstruation and clinically diagnosed uterine diverticula, while no information about fibroids was mentioned. Case 2 was characterized by an aggressively growing abdominal mass. With a large space-occupying lesion in the right abdominopelvic cavity and no imaging evidence of involvement of the iliac vein or above vein, the patient was initially diagnosed with multiple myomata., Diagnoses: Both patients' diagnoses were confirmed as IVL by histopathology. To our knowledge, the mass of case 1 is the minimum IVL in the English literature., Interventions: Subtotal hysterectomy with bilateral salpingectomy was performed on the former, while total hysterectomy with bilateral salpingectomy was performed on the latter., Outcomes: Both patients were comfortable, and no relapse occurred., Lessons: Two cases in the study showed 2 different proceeding stages of the same disease and corroborated multiple pathogeneses, which have been mentioned in the available literature on IVL. Our work provides both supplement for clinical data to facilitate further research and better understanding of special types of fibroids to clinicians., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Reed Syndrome, or hereditary leiomyomatosis and renal cell carcinoma syndrome, is a rare, autosomal dominant genetic condition that predisposes individuals to a triad of cutaneous leiomyomas, uterine leiomyomas and renal cell carcinoma. Cutaneous leiomyomas are often the first manifestation of the syndrome, occurring in 76% of patients and average 26 in number. We present a case of a 47 year old female with Reed Syndrome with an unusually extensive cutaneous burden, with a total of 361 cutaneous lesions, far above the average reported number of 26. Due to the extent of her cutaneous burden, painful nature of the lesions and failure to respond to standard therapies, she was referred for fully ablative Erbium:Yag laser resurfacing therapy. The use of fully ablative Erbium:YAG laser resurfacing therapy for treatment of cutaneous leiomyomas has not been reported in the literature to date. One year following laser therapy, the treatment area not only began to repigment, but there was also no evidence of cutaneous leiomyomas recurrence or associated pain. Given the effectiveness of this unique therapy, fully ablative Erbium:YAG laser resurfacing should be kept in mind as a treatment option for both cosmetic and symptomatic cutaneous leiomyomas.

Background: Disseminated peritoneal leiomyomatosis (DPL) is an extremely rare benign disease characterized by widespread lesions of the abdominal cavity, pelvis, and retroperitoneal space with tumor nodules of varying size and number, which are benign neoplasms consisting of smooth muscle fibers in their histological structure., Aim: To analyze clinical cases of DPL with a concise review of the current state of the DPL diagnosis and treatment., Materials and Methods: We analyzed 5 clinical cases of DPL of female patients aged 39-50 years (mean age 46.2 years) who underwent surgical treatment at the National Cancer Institute from 2010 to 2021. In all 5 patients, the diagnosis of DPL (8898/1) was verified according to pathological (using routine hematoxylin/eosin staining) and immunohistochemical (IHC) studies., Results: All patients underwent surgical treatment with a laparotomy approach, the extent and radicality of which depended on the location and number of tumor lesions. At the time of follow-up, all 5 patients were alive and did not receive any special oncological treatment., Conclusions: DPL is characterized by a variety of clinical manifestations from polyserositis to acute abdomen, depending on the location and size of the main tumor focus. IHC analysis is the criterion for the final diagnosis, and radical removal of all tumor foci provides the best therapeutic prognosis. The treatment should be carried out in highly specialized cancer centers where surgeons have gained sufficient experience in performing cytoreductive surgery.

Intravenous leiomyomatosis is a rare type of tumor that is histologically benign but biologically invasive. It originates from the smooth muscle of the uterine or the uterine vein. It can grow through the uterus and extend into the pelvic cavity, or grow along the veins without invading the wall of the venous vessel itself. The tumors are estrogen-dependent and can metastasize through the bloodstream. Thus, in addition to continuous growth, some tumors exhibit isolated growths in the venous system and heart chambers or show disseminated growth in the lungs, although distant metastasis to other regions usually do not occur. Currently, there is limited research on this disease, the majority of which are case reports, surgical experience summaries, and differentiation from ordinary gynecological myomas in terms of pathogenesis and radiological diagnostic experience. There are two main theories on the origin of the disease: uterine smooth muscle and smooth muscle of the uterine veins. Some studies have verified the role of estrogen, progesterone receptor-related pathways, and angiogenesis in the development of the disease. The clinical symptoms of this disease are varied, depending on the affected area. In the early stages, when the tumor only affects the pelvic cavity, patients show mild symptoms resulting from pelvic organ compression. When it progresses to the inferior vena cava and heart, patients show more complex symptoms resulting from venous return obstruction, cardiac obstruction, and hemodynamics appearing. Different institutions have proposed different disease staging and classification strategies for different clinical purposes. Some are based on the affected area of the lesion; others are based on the size of the tumor. Although surgery remains the main treatment for this disease, the specific surgical approach, adjuvant drug therapy, and prognosis still need further exploration.

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.)

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.)

Leiomyomas are common benign uterine smooth muscle tumours. Rarer subsets may demonstrate aggressive extrauterine growth which mimic metastatic disease. We discuss the case of a female patient in her 40s, with a long-standing atrophic right kidney, presenting with a 17 cm uterine mass demonstrating bilateral para-aortic and pelvic sidewall spread. Although biopsies favoured the diagnosis of a benign tumour, a leiomyosarcoma could not be excluded. The surgical complexity of the case was compounded by a tumour residing close to the only functioning kidney and engulfment of the inferior mesenteric artery. The surgical procedures indicated were a radical hysterectomy, the laterally extended endopelvic resection procedure to achieve clear margins in the pelvic sidewall and a left hemicolectomy. In the absence of formal guidelines, we present this challenging case to provide clarity into the histological assessment and surgical management of rare leiomyomas, as well as an overview of the current literature., 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.)

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.)

Benign metastasising leiomyomatosis (BML) is a rare disease, predominantly seen in premenopausal women. It poses a diagnostic dilemma and can be misdiagnosed as malignancy. Here we present a case of 41-year-old woman with a previous history of hysterectomy 10 years ago for multiple fibroids. She presented with shortness of breath and chest discomfort. Chest X-ray showed pulmonary infiltrates. She was diagnosed with sarcoidosis and treated with steroids without any improvement. Further investigations including CT scan and bronchoscopy and lavage failed to confirm a diagnosis. Subsequently she underwent video-assisted thoracoscopic surgery and histopathology revealed leiomyomatosis (so-called leiomyomatous hamartomas/benign metastasising leiomyomatosis). Oestrogen and progesterone receptors showed diffuse and strong nuclear staining. The patient was commenced on tamoxifen and a repeat chest X-ray in 8 weeks showed significant improvement. In women of reproductive age with previous hysterectomy and multiple lung nodules on imaging, the diagnosis of BML should be taken into consideration., (© Royal College of Physicians 2023. All rights reserved.)

Aim: Histopathologic diagnosis of a subset of uterine smooth muscle tumors is challenging. We report a critical review regarding the clinicopathological point of view of 62 cases of subsequently recurred or metastasized leiomyoma., Methods: Medical records and glass slides of 62 cases of uterine smooth muscle tumor diagnosed as leiomyoma, which subsequently recurred or metastasized, were critically reviewed by pathologists specializing in gynecologic pathology and oncology., Results: In 47 (75.8%) of 62 cases, the diagnosis of leiomyoma was confirmed, including 11 intravascular leiomyomatosis (IVL) and benign metastasizing leiomyoma (BML). In 29 cases (46.8%) laparoscopic surgery was performed, of which morcellator without a bag was employed in 23 cases. Fifteen cases (24.2%) appeared to be underestimated and were re-classified as smooth muscle tumor of uncertain malignant potential (STUMP), leiomyosarcoma, or other malignant mesenchymal tumors. Recurrences in seven cases (11.3%) were interpreted to be a malignant transformation, and one STUMP recurred as STUMP., Conclusion: The recurrence or metastasis in cases of "leiomyoma" is attributed to iatrogenic or under-evaluation of primary tumors, although a subset of cases is a rare example of biological progression., (© 2022 Japan Society of Obstetrics and Gynecology.)

Intravenous leiomyomatosis (IVL) is characterized by the presence of vascular extension and invasion of benign smooth muscle lesions in a worm-like manner from uterine fibroids into the systemic vasculature system. Surgery with complete tumour resection remains the main treatment approach, however both treated and untreated of this disease is associated with high morbidity and mortality. The aim of this systematic review is to highlight the systemic manifestations and surgical management of IVL., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

BACKGROUND Intracardiac leiomyomatosis (ICLM) is an extremely rare tumor which is benign but presents with aggressive behavior. To date, there is still no standard of care for ICLM therapy, and treatment for complicated ICLM has obtained even less attention. Radical surgery was usually recommended to remove the patients' tumors completely. Since initial complete surgical resection cannot be performed in all cases, bilateral salpingo-oophorectomy (BSO), via its effects of estrogen deprivation, may be a feasible primary step in the treatment of premenopausal women with unresectable ICLM. CASE REPORT We describe a case of a residual mass in the inferior vena cava and right atrium that shrank dramatically after BSO. The patient was a 41-year-old woman with initially unresectable ICLM. Total hysterectomy with BSO and excision of the retroperitoneal mass was performed, but the intracaval tumor above L5 was not removed. Pathology revealed a benign leiomyoma which was strongly positive for both estrogen receptor and progesterone receptor. Two weeks after the BSO, the patient's serum estradiol level had decreased to a postmenopausal level. At the same time, the proximal end of the intracaval tumor shrank dramatically from the level of the right atrium to the level of L3 only 2 weeks after the surgery. Therefore, this may provide a therapeutic window for a second reduction surgery. CONCLUSIONS BSO, via its estrogen deprivation effect, may provide a simple but effective initial treatment choice for premenopausal women who suffer from primary unresectable ICLM.

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.)

Background: Leiomyomatosis peritonealis disseminata (LPD) is a rare disease characterized by multiple leiomyomas spread all over the peritoneal cavity. It is commonly described as benign in women of reproductive age. Malignant LPD is much rarer., Methods: We present a case of LPD with low potential malignant change in a 43-year-old female, who felt a lump in her abdomen after laparoscopic myomectomy 10 years ago and laparoscopic hysterectomy 8 years ago. The patient underwent exploratory laparotomy and salpingectomy, greater omentectomy, and pelvic and abdominal mass resection were performed during the surgery. The pathological findings revealed LPD with low potential malignant change, with strong expression of estrogen receptor and progesterone receptor. The patient refused oophorectomy and chose gonadotropin-releasing hormone agonists injection postoperatively., Results: No recurrence was found during the follow-up to date., Conclusion: Surgery is the main treatment for LPD, and endocrine therapy is another choice. Although it is reported mostly benign, we need to be alert to the possibility of malignancy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

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.)