Diffuse uterine leiomyomatosis: a rare discovery.

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.

Unraveling the challenges of intravenous leiomyomatosis: a retrospective analysis of 11 cases.

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.

Arteriovenous Malformation Associated with Intravenous Leiomyomatosis of the Uterus: Characteristic CT Findings and Treatment by Embolization and Surgery.

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.

Successful pregnancies in women with diffuse uterine leiomyomatosis after high-intensity focused ultrasound ablation: report of three cases.

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.

Imaging diagnosis of intravenous leiomyomatosis: an institutional experience.

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.

"Evolution" of intravascular leiomyomatosis.

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.

Laparoscopic Management of Disseminated Peritoneal Leiomyomatosis.

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.

Intravenous leiomyomatosis involving the right heart: Comprehensive evaluation using echocardiography combined with abdominal ultrasonography.

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.

MR Spectroscopy for Detecting Fumarate Hydratase Deficiency in Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome.

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.

Echocardiographic Characteristics and Contrast-Enhanced Imaging of Intravenous Leiomyomatosis With Intracardiac Extension.

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.

[Clinical analysis of 81 cases of intravenous leiomyomatosis confined to pelvic cavity].

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.

DISSEMINATED PERITONEAL LEIOMYOMATOSIS - A RARE ENTITY, COMPLICATED BY LATE BLEEDING FROM THE ILEOCOLIC VEIN.

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.

Tricky Diagnosis and Robot-laparoscopic Surgical Approach to Disseminated Peritoneal Leiomyomatosis.

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.

High intensity focused ultrasound treatment for diffuse uterine leiomyomatosis: a feasibility study.

OBJECTIVE: To investigate the safety and efficacy of high-intensity focused ultrasound (HIFU) treatment for diffuse uterine leiomyomatosis (DUL). METHODS: Eight patients with DUL were admitted to the Department of Gynecology of Shanghai First Maternity and Infant Hospital and underwent HIFU treatment. MRI was performed before and one day after HIFU treatment for the evaluation of lesion ablation. The uterine size was measured at 3-8 months after HIFU ablation. The menstrual volume score and serum levels of hemoglobin and CA-125 were measured pre-HIFU ablation and 12-36 months post-HIFU ablation. RESULTS: After an average of 5.9 months of follow-up after HIFU treatment, an average uterine volume reduction of 67.6% was observed. Menstruation returned to normal in all patients, and their serum HGB and CA-125 levels also returned to normal after an average of 19.1 months of clinical follow-up. The quality of life of all patients improved significantly. CONCLUSION: HIFU treatment is safe and effective in the treatment of patients with DUL.

Disseminated peritoneal leiomyomatosis after uterine artery embolization, laparoscopic surgery, and high intensity focused ultrasound for uterine fibroids：a case report.

BACKGROUND: Disseminated peritoneal leiomyomatosis (DPL) is a rare, generally benign disorder. With the advent of laparoscopic surgery for uterine fibroids, the reported number of cases of DPL has significantly increased since the introduction of unconfined power morcellation. Morcellation and other procedures may be associated with DPL. METHODS: We present the case of a 48-year-old patient with DPL who underwent uterine artery embolization (UAE), laparoscopic myomectomy and high intensity focused ultrasound (HIFU) 11 years, 6 years, and 2 years before the final diagnosis of DPL was made. A subtotal hysterectomy with bilateral salpingo-oopherectomy was performed to remove the uterus, the fallopian tube and the ovaries. We carefully excised as many visible lesions as possible. RESULTS: After the surgical treatment performed in our center the patient became free of symptoms. CONCLUSION: In our case, the occurrence of DPL is most likely associated with laparoscopic myomectomy using power morcellation. In addition, it should be noted whether some other factors played a role in stimulating the growth of the multiple nodules.

Intravascular Leiomyomatosis Extending to Right Atrium: A Rare Caused Syncope.

Intravascular leiomyomatosis (IVL) is a variant of leiomyoma characterized by intravascular proliferation of a histologically benign smooth muscle tumor extending beyond the uterus into distant great vessels or the heart. It is a rare disease and results in death. Here, we reported the case of 48-year-old, otherwise well woman, who presented to the emergency department with syncope. Pulmonary computed tomography (CT) demonstrated a large low-density shadow originating from the inferior vena cava (IVC) extending into the right atrium (RA). Magnetic resonance venography (MRV) showed that a neoplasm was "snakelike," which completely occluded the right internal iliac vein (RIIV), the common iliac vein (CIV), and IVC. A multidisciplinary team of specialists consisting of vascular surgeons, cardiac surgeons, gynecologists, anesthesiologists, and radiologists reviewed the history, clinical examination findings, and diagnostic imaging of the patient. A decision was made to proceed with one-stage surgery (resection of thoracoabdominal tumor extension at one operative setting). After surgery, the patient's vital signs were restored, and her symptoms were disappeared. She was discharged on hospital day 21 without complications. One-stage surgical approach to completely remove an IVL with RA involvement is an optimal choice if the patient's physical condition permits.

Surgical removal of intravascular leiomyomas with intracardiac extension: A two-case report.

Leiomyomatosis is a uterine condition that can rarely present with extrauterine involvement. Intravenous and intracardiac extensions are extremely rare presentations and, in these cases, the intra-cardiac mass may be confused with a cardiac myxoma and can be associated with pulmonary embolism. Here we report two cases of successful surgically removed intracardiac leiomyomas under cardiopulmonary bypass.

Leiomyomatosis peritonealis disseminata: An exceptional case.

Leiomyomatosis peritonealis disseminata is a rare clinical disorder characterised by proliferation of smooth muscles spread all over the peritoneal cavity. History and examination do not help in establishing a proper diagnosis, which is possible only by histopathology. Although LPD is a benign disease which regresses spontaneously, it may degenerate to malignancy, so proper follow up is mandatory. A 38-year-old lady presented with lower abdominal pain on 27th day of her menstrual cycle. Due to suspicion of ovarian cyst accident, laparotomy was performed during which innumerable nodules were found scattered all over the peritoneal cavity. Histopathology revealed leiomyomatosis peritonealis disseminata. The patient was followed up for two years without any treatment and she remained asymptomatic.

Obstruction of the Hepatic Venous Flow Caused by Intravenous Leiomyomatosis.

Budd-Chiari syndrome (BCS) is a rare intrahepatic vascular disease that is characterized by a hepatic venous outflow obstruction. Intravenous leiomyomatosis (ILs) is a rare complication of a myoma. Here, we report a case of BCS that was caused by intracaval ILs. A woman presented to the emergency department (ED) with abdominal distension that had gradually progressed over a period of 3 years. Bedside ultrasonography and contrast-enhanced computed tomography (CECT) showed a large ascites and pelvic mass. The mass continued to the inferior vena cava and the right atrium. The intracaval mass was obstructing the left and middle hepatic veins. We established a tentative diagnosis of BCS caused by intracaval ILs and attempted surgical resection. Complete resection of the intracaval mass failed because of adhesion; however, she was discharged from the hospital without any postoperative complications. After 3 months, a pelvic ultrasonography showed a recurrence of a 4 × 3 cm pelvic mass. The mass size increased to 6 cm after 30 months. ILs can cause secondary BCS and can lead to life-threatening conditions. Owing to its extreme rarity, early detection in the ED is challenging. Bedside ultrasonography and CECT can enable the early recognition of BCS by ILs.