Intravenous leiomyomatosis in the inferior vena cava and right atrium with pulmonary benign metastasizing leiomyoma secondary to a pelvic arteriovenous fistula: A case report and literature review.

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.

Haemodynamic study of left nonthrombotic iliac vein lesions: a preliminary report.

Nonthrombotic iliac vein lesions (NIVLs) are significant causes of chronic venous insufficiency (CVI) in the left lower limb and symptom recurrence following left lower limb varicose vein treatment. The goal of this study was to explore the haemodynamic and morphological characteristics of iliac veins in patients with NIVLs. Pressure at the caudal end of the stenotic left common iliac vein (LCIV) segment, local blood flow velocity, and time-averaged wall shear stress in the stenotic segment exhibited positive correlations with the clinical CVI classification (R = 0.92, p < 0.001; R = 0.94, p < 0.001; R = 0.87, p < 0.001), while the relative retention time showed a negative correlation (R = -0.94, p < 0.001). The pressure difference (∆P) between the two ends of the stenotic segment and the velocity difference (∆V) between the stenotic segment and the caudal end were positively correlated with the clinical classification (R = 0.92, p < 0.001; R = 0.9, p < 0.001). The cross-sectional area stenosis rate and length of the stenotic LCIV segment were positively correlated with the clinical classification (R = 0.93, p < 0.001; R = 0.63, p < 0.001). The results suggest that haemodynamic assessment of the iliac vein could effectively portray blood flow disturbances in stenotic segments of the LCIV, potentially reflecting the degree of iliac vein stenosis. Haemodynamic indicators are correlated with the severity of clinical CVI symptoms.

Experimental multiparametric magnetic resonance imaging characterization of iliocaval venous thrombosis pathological changes.

OBJECTIVE: Iliocaval thrombotic obstruction is a challenging condition, especially because thrombus age and corresponding pathological remodeling at presentation are unknown, which directly impacts management. Our aim was to assess the ability of magnetic resonance imaging (MRI) in determining age thresholds of experimentally created inferior vena cava (IVC) thrombosis in pigs. METHODS: We used a previously described swine model of IVC thrombosis. The animals underwent MRI at baseline, immediately after thrombosis creation, and after a follow-up period extending from 2 to 28 days. Thirteen pigs were divided into three groups according to disease chronicity: acute group (AG; n = 5), subacute group (SAG; n = 4), and chronic group (CG; n = 4), with a mean thrombosis age of 6.4 ± 2.5 days, 15.7 ± 2.8 days, and 28 ± 5.7 days, respectively. A T1-weighted volumetric interpolated breath-hold examination sequence was used to anatomically delineate IVC thrombus as a region of interest. Three other MRI sequences were used to assess the thrombus signal. RESULTS: The Kruskal-Wallis test showed a statistically significant difference in T1 relaxation times after contrast injection (P = .026) between the three groups of chronicity. The AG (360.2 ± 102.5 ms) was significantly different from the CG (336.7 ± 55.2 ms; P = .003), and the SAG (354.1 ± 89.7 ms) was significantly different from the AG (P = .027). There was a statistically significant difference in native T2 relaxation times (P = .038) between the three groups. The AG (160 ± 86.7 ms) was significantly different from the SAG (142.3 ± 55.4 ms; P = .027), and the SAG was significantly different from the CG (178.4 ± 11.7 ms; P = .004). CONCLUSIONS: This study highlighted MRI characteristics in a swine model that might have the potential to significantly differentiate subacute and chronic stages from an acute stage of deep vein thrombosis in humans. Further clinical studies in humans are warranted. CLINICAL RELEVANCE: In addition to providing a better understanding of venous thrombosis remodeling over time, magnetic resonance imaging has the potential to be a tool that could allow us to characterize the composition of venous thrombus over an interval, allowing for a refined analysis of the local evolution of venous thrombosis. We propose a noninvasive and innovative method to characterize different thresholds of chronicity with magnetic resonance imaging features of central deep vein thrombosis of the inferior vena cava experimentally obtained using a totally endovascular in vivo swine model, mimicking human pathophysiology. Being able to determine these features noninvasively is critical for vascular specialists when it comes to choosing between fibrinolytic therapy, percutaneous thrombectomy, or surgical management.

Different clinical diagnosis end up in the same pathological diagnosis of intravascular leiomyomatosis: Two case reports.

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.

Histological Features of In-Stent Restenosis after Iliac Vein Thrombus Removal and Stent Placement in a Goat Model.

PURPOSE: To establish an animal model for in-stent restenosis (ISR) after postthrombotic iliac vein stent placement and characterize histopathological changes in tissue within the stented vein. MATERIALS AND METHODS: Iliac vein thrombosis was induced using balloon occlusion and thrombin injection in 8 male Boer goats. Mechanical thrombectomy and iliac vein stent placement were performed 3 days after thrombosis induction. Restenosis was evaluated by venography and optical coherence tomography (OCT) at 1 and 8 weeks after stent placement, and stent specimens were taken for pathological examination after the animals were euthanized. RESULTS: Thrombosis induction was successful in all 8 goats, with >80% iliac vein occlusion. After thrombus removal, OCT revealed considerable venous intimal thickening and a small number of mural thrombi. Neointimal hyperplasia with thrombus formation was observed in all goats 1 week after stent implantation; the degree of ISR was 15%-33%. At 8 weeks, the degree of ISR was 21%-32% in 3 goats, and stent occlusion was observed in 1 goat. At 1 week, the neointima predominantly consisted of fresh thrombi. At 8 weeks, proliferplastic fibrotic tissue and smooth muscle cells (SMCs) were predominant, and the stent surfaces were endothelialized in 2 of 3 goats and partially endothelialized in 1 goat. CONCLUSIONS: In the goat model, postthrombotic neointimal hyperplasia in the venous stent may result from time-dependent thrombus formation and organization, accompanied by migration and proliferation of SMCs, causing ISR. These results provide a basis to further explore the mechanism of venous ISR and promote the development of venous stents that reduce neointimal hyperplasia.

Tumor thrombus formation in the right common iliac vein after radical proctectomy in a patient with rectal cancer: a case report.

BACKGROUND: Intravascular tumor thrombi are mainly found in patients with liver cancer or renal carcinoma but rarely occur in those with rectal cancer. CASE PRESENTATION: This is a case report of a 58-year-old woman with a swollen right lower extremity 14 months after radical resection for rectal cancer. Although ultrasonography indicated the presence of deep venous thrombosis (DVT) located in the right common iliac vein, interventional angiography showed that a circular mass, considered a tumor thrombus, was located in the right common iliac vein. The tumor thrombus was cured by interventional therapy, and the pathological report confirmed that the metastatic tumor thrombus originated from the rectal cancer. The patient underwent concurrent chemoradiotherapy and systemic therapy. However, right lung, retroperitoneum, and 2nd sacral vertebral metastases were discovered during follow-up. CONCLUSION: The correct diagnosis of a tumor thrombus and its differentiation from DVT can prevent incorrect treatment and prolong the survival of patients with rectal cancer.

Antithrombotic Therapy After Venous Interventions: AJR Expert Panel Narrative Review.

Interventions for thrombotic and nonthrombotic venous disorders have increased with technical advances and more trained venous specialists. Antithrombotic therapy is essential to clinical and procedural success; however, postprocedural therapeutic regimens exhibit significant heterogeneity due to limited prospective randomized data and incomplete mechanistic understanding of the critical factors driving long-term patency. Postinterventional antithrombotic therapy for thrombotic venous disorders should adhere to existing venous thromboembolism management guidelines, which include 3-6 months of therapeutic anticoagulation at minimum and consideration of extended therapy in patients with higher risk of thrombosis because of procedural or patient factors. The added benefit of antiplatelet agents in the acute and intermediate period is unknown, having shown improved long-term stent patency in some retrospective studies. Dual- and/or triple-agent therapy should be limited based on individual risks of thrombosis and bleeding. The treatment of nonthrombotic disorders is more heterogeneous, though patients with limited flow, extensive stent material, or underlying prothrombotic states such as malignancy or chronic inflammation may benefit from single-agent or multiagent antithrombotic therapy. However, the agent, dose, and duration of therapy remain indeterminate. Future prospective studies are warranted to improve patient risk stratification and standardize postprocedural anti-thrombotic therapy in patients receiving venous interventions.

Bilateral external iliac vein compression-An important consideration in implantation of leadless permanent pacemaker.

We report on an unusual case with asymptomatic bilateral external iliac vein non-thrombotic obstruction causing difficulty in delivery of the leadless permanent pacemaker and discuss on the strategies to overcome the problem.

Preoperative evaluation of iliac blood vessels for first kidney transplant recipients: Combination of conventional and contrast-enhanced ultrasonography.

OBJECTIVE: To assess iliac blood vessels using conventional ultrasound (US) and contrast-enhanced ultrasonography (CEUS) before kidney transplantation (KT) and determine whether US findings related to post-transplant outcomes. METHODS: A total of 119 patients received US and CEUS before KT waiting-list acceptance. The preoperative iliac blood hemodynamics and vascular conditions were evaluated. The operative strategy and follow-up outcomes were recorded. Logistic regression and correlation analysis were used. The accuracy in determining the patency of iliac blood vessels was calculated before and after the injection of contrast materials. RESULTS: CEUS can help to significantly improve the visualization of the internal iliac artery, but there was no significant correlation with post-transplant outcomes. In terms of accuracy, there were significant differences in determining the patency of internal iliac arteries between conventional US and CEUS (60.5% and 100%, p < 0.001). The surgical strategy of one patient was regulated and two patients were excluded from KT according to US findings. CONCLUSIONS: Compared with conventional US, CEUS helps to improve the visualization of the internal iliac artery. Conventional US and CEUS have the potential to serve as effective methods to evaluate anatomy and hemodynamics of iliac vessels and have a potential value while defining clinical algorithms in surgery decision-making.

Novel typing of iliac vein compression in asymptomatic individuals evaluated by contrast enhanced CT.

PURPOSE: Compression of the iliac vein between the iliac artery and lumbosacral vertebra can cause iliac vein compression syndrome (IVCS). The purpose of this study is to assess compression characteristics and establish a new sub-typing in asymptomatic IVCS individuals using contrast-enhanced CT. METHODS: A retrospective analysis of abdomen contrast-enhanced CT images from 195 asymptomatic subjects with iliac vein compressed was investigated. Patients had no history of venous pathology, and images were collected from June 2018 to January 2019. Qualitative and quantitative characteristics of compression were examined including the location, pattern, minor diameter, area, and the percentage compression on an orthogonal section by the post-processing of multiple planar reconstruction and volume rendering. RESULTS: There were 107 females and 88 males with age range 18-92 years. The most common site of iliac vein compression was localized to the left common iliac vein (LCIV) (178/195, 91.3%). Notably, four compression types (type I-IV) were established according to the compression location, with type II being the most common. The four compression types had differences in the upper limit and fluctuation range of compression. It was found that the average level of iliac vein compression was below 25%. The compression degree of the left common iliac vein in type II was relatively concentrated, and the upper limit of compression was close to 70%. CONCLUSION: Asymptomatic iliac vein compression was categorized according to compression location. The proposal of four types might help clinicians to predict which IVCS patients would benefit from interventional therapy.

Experimental analysis of aspect ratio in iliac vein stenosis.

BACKGROUND: Veins are thin-walled tubes. Their lumen is roughly circular with an aspect ratio close to 1:1 under physiologic pressures. When they collapse owing to decreased internal pressure or external compression, the aspect ratio changes. The vertical diameter is usually diminished more than the transverse, with a considerable decrease in the lumen area. The recent emergence of stent correction of many venous compression syndromes, particularly iliac vein stenosis, has brought attention to the importance of the aspect ratio, quite apart from an overall decrease in caliber. The iliac vein pressure is influenced not only by stenosis, but also intra-abdominal pressure, right atrial pressure, and collaterals. We investigated the impact of aspect ratio in an experimental model incorporating these factors. METHODS: Inflow was provided from a header tank at 25 mm Hg pressure into a Penrose tubing enclosed in a polyethylene cylinder pressurized (Starling pressure) to simulate intra-abdominal pressures of 5 and 10 mm Hg. The Penrose drained into an outflow tank with a pressure of 7 mm Hg, simulating right atrial pressure. Stenosis was simulated with a series of three-dimensional, printed plastic nozzles with caliber areas of 50, 100, and 200 mm2 and varying in aspect ratios of 1:1 to 1:4. The flow and pressure in this system was monitored with the use of overflow collaterals in some experiments. RESULTS: Free flow from the header tank through the Penrose (zero Starling pressure) with a 200 mm2 circle nozzle into the outflow tank with zero pressure resulted in flow pressure of approximately 1.5 mm Hg. Using nozzles of a smaller caliber or an increased aspect ratio resulted in an increase of flow pressures of up to approximately 3.7 mm Hg. Flow into an outflow tank of 7 mm Hg simulating right atrial pressure further increased flow pressures by approximately 7 mm Hg. The addition of Starling pressures of 5 and 10 mm Hg simulating abdominal pressure increased flow pressure even further to the 10 to 17 mm Hg range. When the Starling pressure was dominant, the additional contribution of nozzle caliber stenosis or aspect ratio reduction to the overall flow pressure ranged from 2 to 6 mm Hg. Collateral overflow varied inversely with collateral resistance. Some experiments yielded an anomalous flow/pressure phenomena known to occur in collapsible tube flows. CONCLUSIONS: A decrease in the caliber or the aspect ratio of iliac vein stenosis was among several other factors that generate peripheral venous hypertension in an experimental model. Increased intra-abdominal pressure is a major influence that amplifies the pressure effects of aspect ratio or caliber reduction.

The perplexity of catheter-directed thrombolysis for deep venous thrombosis: the approaches play an important role.

The recent adjunctive catheter-directed thrombolysis (ATTRACT) trial rose a controversy about the treatment effect of catheter-directed thrombolysis (CDT) in deep venous thrombosis (DVT). In fact, most studies including the ATTRACT trial did not perform subgroup analysis of catheterization approaches. Different approaches would confound the conclusions. Therefore, a single-center retrospective analysis was performed to compare the differences between the antegrade (AGA) and retrograde (RGA) approaches. Total 217 DVT patients treated with CDT were enrolled from January 2010 to December 2017, with mean age of 55.3 years (67 received antegrade approach, 150 received retrograde approach). The clot burden reduction by segment was evaluated. The mean access establishment time and thrombolytic time were compared. The patency of the iliofemoral vein at 6 months was evaluated. The rate of PTS, quality of life and venous insufficiency were assessed at 1 year. AGA group showed better thrombolytic effect in popliteal and femoral vein than RGA group. The rate of iliofemoral clot burden reduction in RGA group was mostly at Grade II, while most were at Grade III in AGA group. The retrograde approach showed better thrombolysis effect in iliofemoral DVT than popliteal to iliac DVT. The RGA group reported longer mean access establishment time (5.4 ± 1.8 vs 27.0 ± 7.5 min, p < 0.001) and thrombolytic time (6.9 ± 1.5 days vs 6.8 ± 1.5 days, p = 0.586). At 6 months, RGA group had a lower rate of femoral vein patency (52.0% vs 89.6%, p < 0.001) and a higher rate of venous insufficiency (52.0% vs 29.9%, p < 0.001), compared with AGA group. Although there was no difference in the rate of PTS, the RGA group showed higher Villalta scores in the free and mild PTS. The antegrade approach was preferably recommended over the retrograde approach for CDT treatment.

Primary external iliac vein leiomyosarcoma in a young female diagnosed by contrast-enhanced ultrasound: A case report.

RATIONALE: Venous leiomyosarcoma (LMS) is a malignant tumor arising from the smooth muscle cell of the vein wall. The diagnosis of venous LMS is usually delayed owing to its rarity, absence of serological markers, and mimicry with deep vein thrombosis (DVT). Herein, we report a case of a primary external ilian vein LMS characterized by long-term, unexplained DVT in the left lower limb. Contrast-enhanced ultrasound (CEUS) played a crucial role in the preoperative diagnosis. No improvement was observed in the lower limb status; a rapid, high-level, heterogeneous wash-in and wash-out mass of the vein, as seen with CEUS, could indicate angiogenic malignancy. CEUS also helped evaluate the percent of intratumoral necrosis, which is an important parameter for predicting the prognosis. PATIENT CONCERNS: A 37-year-old Chinese women presented to the Vascular Surgery Department of our hospital for accurate diagnosis of her condition. She began experiencing edema and pain in her left leg 2 years ago. She was diagnosed with DVT in the left lower extremity and was administered anticoagulant therapy since then. However, her symptoms started to aggravate 8 months ago. DIAGNOSES: The laboratory results including D-dimer, prothrombin time (PT), activated partial thrombin time (APTT), and prothrombotic conditions screening were within normal ranges. A pelvic ultrasound detected a heterogeneous, hypoechoic mass compressing the external iliac vein and obstructing the venous drain of the lower extremity. The mass showed a rapid, high-level, heterogeneous wash-in and wash-out on CEUS, which suggested angiogenic malignancy. Contrast-enhanced CT (CECT) confirmed the result of CEUS but revealed no metastasis. INTERVENTIONS: She underwent complete surgical removal of the tumor, which was resected successfully. There was no infiltration in the inguinal nodes sent for the study. OUTCOMES: Pathological examination and immunohistochemistry confirmed that the mass was a well-differentiated LMS originating from the external iliac vein. There was no sign of local recurrence or distant metastasis during a 12-month follow-up. LESSONS: Effective imaging techniques and differential diagnosis of venous LMS is vital and should be considered for patients with chronic thrombosis presenting with normal laboratory results.

Effect of intentional restriction of venous return on tissue oxygenation in a porcine model of acute limb ischemia.

INTRODUCTION: A sufficient oxygen supply to ischemic limb tissue is the most important requirement for wound healing and limb salvage. We investigated whether partial venous occlusion in the common iliac vein (CIV) causes a further increase of venous oxygenation in a porcine model of acute hindlimb ischemia. MATERIALS AND METHODS: In 7 pigs, the model of acute hindlimb ischemia was created with intra-vascular embolization of the common iliac artery (CIA). The arterial and venous oxygen saturation was evaluated at different moments. Oxygen saturation was evaluated at baseline (T0), just after the arterial embolization (T1), at 10 minutes (T2), at 20 minutes (T3), and at 40 minutes (T4). Next, an intentional partial venous occlusion was achieved by inflating the vascular balloon at the level of the right CIV. Then, blood sampling was repeated at 5 minutes (T5), at 15 minutes (T6), and at 25 minutes (T7). RESULTS: The arterial oxygen saturation in the right SFA was similar during all phases. In contrast, after arterial embolization, an immediate reduction of venous oxygen saturation was observed (from 85.57 ± 1.72 at T0 to 71.86 ± 7.58 at T4). After the partial venous occlusion, interestingly, the venous oxygen saturations (T5-T7) were significantly increased, again. The venous oxygen saturations evaluated in the hindlimb ischemia with partial venous occlusion and in the control limb (without partial venous occlusion) were significantly over time. Venous oxygen saturations in the experimental limbs were higher than those in the control limbs (79.28 ± 4.82 vs 59.00 ± 2.82, p-value <0.001, 79.71 ± 4.78 vs 60.00 ± 4.24 at T7, p-value <0.001). CONCLUSIONS: Partial venous occlusion results in an increase of venous oxygen saturation in the ischemic limb, while significant changes in venous oxygen saturation are not observed in the control limb. An explanation for this may be that the oxygen consumption in the limb tissue is increased because it gets congested with the partial venous occlusion in the right CIV.

Idiopathic spontaneous extensive iliocaval thrombosis presenting with renal infarct.

We describe an extremely rare case of idiopathic spontaneous extensive venous thrombosis in a young man involving the inferior vena cava, the iliac veins and both renal veins associated with right haemorrhagic renal infarction with non-functioning right kidney.

Critical Review of Large Animal Models for Central Deep Venous Thrombosis.

OBJECTIVE: To review the existing literature on large animal models of central venous thrombosis (CVT) and to evaluate its relevance in regard to the development and testing of dedicated therapeutics applicable to humans. METHODS: A systematic literature search was conducted in PubMed and Embase. Articles describing an in vivo experimental protocol of CVT in large animals, involving the iliac vein and/or the vena cava and/or the brachiocephalic vein, were included. The primary aim of the study, animal characteristics, experimental protocol, and thrombus evaluation were recorded. RESULTS: Thirty-eight papers describing more than 30 different protocols were included. Animals used were pigs (53%), dogs (21%), monkeys (24%), and cattle (3%). The median number of animals per study was 12. Animal sex, strain, and weight were missing in 18 studies (47%), seven studies (18%), and eight studies (21%), respectively. CVT was always induced by venous stasis: solely (55%), or in addition to hypercoagulability (37%) or endothelial damage (10%). The size of the vessel used for thrombus creation was measured in four studies (10%). Unexpected animal death occurred in nine studies (24%), ranging from 3% to 37% of the animals. Twenty-two studies (58%) in the acute phase and 31 studies in the chronic phase (82%) evaluated the presence or absence of the thrombus created, and its occlusive characteristic was reported, respectively, in five and 17 studies. Histological examination was performed in 24 studies (63%) with comparison to human thrombus in one study. CONCLUSION: This review showed advantages and weaknesses of the existing large animal models of CVT. Future models should insist on more rigour and consistency in reporting animal characteristics, as well as evaluating and comparing the thrombus created to human thrombus.

[Gynecological Lemierre's syndrome: A case report and literature review]. / Syndrome de Lemierre inversé : à propos d'un cas et revue de la littérature.

INTRODUCTION: Lemierre's syndrome is defined as an oropharyngeal infection due to Fusobacterium necrophorum, associated with septic thrombophlebitis of the internal jugular vein. The uncommon pelvic variant of the syndrome is a rare condition, poorly described in literature. CASE REPORT: We report a case of gynecological Lemierre's syndrome in a 19-year-old woman after a first sexual intercourse, who presented acute respiratory failure, left internal iliac vein thrombosis with pulmonary embolism, in the setting of salpingitis and F. necrophorum bacteriemia. CONCLUSION: Gynecological Lemierre's syndrome is a rare and unrecognized condition, which could be lethal. Early recognition of the disorder enables initiation of appropriate antibiotic therapy for 4 to 6 weeks, and discussion of anticoagulant therapy which indications are not yet well defined.

Fibromuscular dysplasia presenting with a deep vein thrombosis.

A 41-year-old male patient presented with isolated right lower limb swelling. An ultrasound scan showed right external iliac and femoral vein deep vein thrombosis due to extrinsic compression by an aneurysm of the right common iliac artery. Investigations including imaging and a tissue biopsy of right and left femoral arteries confirmed a rare clinical presentation of fibromuscular dysplasia involving iliac, coeliac, renal and pulmonary vessels. The common iliac artery aneurysm was successfully treated with endovascular repair. Six months later, he developed coronary artery involvement with spontaneous dissection of left anterior descending artery diagnosed on coronary angiogram which was managed conservatively. At 6-year follow-up, he remains clinically asymptomatic and continues with regular surveillance imaging. Iliac arterial fibromuscular dysplasia is uncommon and clinical presentation with a complication such as a deep vein thrombosis is atypical.

Primary iliocaval leiomyosarcomas: The path beyond surgery.

INTRODUCTION: Iliocaval leiomyosarcoma (ICLM) is a rare and aggressive form of sarcoma within the retroperitoneum. Surgery is the mainstay of treatment, with no consensus on the benefit of chemoradiotherapy in the neo/adjuvant setting. This study aims to describe the natural history of a chemotherapy-naïve ICLM treated in a tertiary cancer centre and to explore potential directions to improve oncological outcome. MATERIALS AND METHODS: A prospective database was used to identify patient demographics, clinicopathological variables and oncological outcomes in 30 patients who underwent surgical resection in our institution for primary non-metastatic ICLM between 2003 and 2018. RESULTS: There was no 90-day mortality. With a median follow-up time of 70.0 months (95% CI 52.6-87.4), 5/30 patients (16.7%) developed local recurrence while 11/30 (36.7%) developed distant metastatic disease. 1 patient (3.3%) developed both local and distant recurrence. Median overall survival of our cohort was 41.0 months (95% CI 33.6-48.4) and 5-year overall survival rate was 32.1%. Multivariate survival analysis using the Cox proportional hazard model identified tumour grade and blood loss of more than 600 mL as key prognostic factors in our model. CONCLUSION: Management of ICLM should be centralised in high-volume sarcoma centres with expertise in the management of retroperitoneal sarcomas. Integration of tumour biology with a concerted effort to conduct conclusive multi-centre phase III in histological and molecularly defined sarcoma subgroups is necessary to improve patient outcome. We eagerly await the results of STRASS 2 study to gain more insights to the efficacy of neoadjuvant chemotherapy on patient prognosis.