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.

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.

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.

Myopericytomatosis: A rare entity mimicking other vascular tumours and malformations.

Myopericytoma is a rare tumour which typically presents as a benign lesion that mimics features of other more common vascular tumours and malformations. We present a case of a symptomatic diffuse myopericytomatosis of the left abdomen presenting as multiple subcutaneous vascular tumours detected on ultrasound and treated with ultrasound-guided sclerotherapy.

Spinal intravascular papillary endothelial hyperplasia. Case report and review of the literature.

Intravascular papillary endothelial hyperplasia (Masson's vegetated hemangioendothelioma) is a rare condition affecting the neuroaxis. In the literature, only eight cases of this lesion involving the vertebral canal with spinal cord compression has been reported. We present a 37-year-old man with thoracic location mimicking schwannoma. Differential diagnosis, management, and review of literature are discussed in this short report.

Oncologic Outcomes Associated With MRI-detected Extramural Venous Invasion (mrEMVI) in Rectal Cancer: A Systematic Review and Meta-analysis.

BACKGROUND: The role of MRI-detected EMVI (mrEMVI) as a reliable prognostic factor in rectal cancer has been emphasized in recent years but this finding remains underreported by many institutions. OBJECTIVE: This review aimed to demonstrate the importance of pre- and post-treatment MRI-detected EMVI as independent prognostic factors of adverse oncologic outcomes in patients undergoing neoadjuvant therapy followed by total mesorectal excision. METHODS: This review was designed using the PRISMA guidelines. The following electronic databases were searched from January 2002 to January 2020: CENTRAL, Ovid MEDLINE, PubMed, and Ovid Embase. Main outcomes included DFS and overall survival (OS). Other outcomes of interest comprised positive resection margin and synchronous metastases. RESULTS: Seventeen studies involving a total of 3821 patients were included for data synthesis. For preneoadjuvant treatment mrEMVI, pooled hazard ratio (HR) estimate for DFS was 2.30 (95% confidence intervals (CI) 1.54-3.44) for higher recurrence in mrEMVI-positive patients. mrEMVI-positive patients were found to have a lower OS with a pooled HR of 1.68 (95%CI 1.27-2.22). Pooled risk ratio for synchronous metastasis was 4.11 (95%CI 2.80-6.02) for mrEMVI-positivity. For postneoadjuvant treatment EMVI (ymrEMVI), positive status showed a lower DFS with a pooled HR of 2.04 (95%CI 1.55-2.69). Risk ratio of having a positive resection margin status was 2.95 (95%CI 1.75-4.98) for ymrEMVI-positive patients. CONCLUSIONS: This review showed that oncologic outcomes are significantly worse for both pre- and post-neoadjuvant treatment mrEMVI-positive patients. MRI-detected EMVI should be consistently reported in rectal cancer staging and may provide guidance for the targeted use of additional systemic therapy.

LI-RADS Tumor in Vein at CT and Hepatobiliary MRI.

Background Diagnostic performance of the Liver Imaging Reporting and Data System tumor in vein (LR-TIV) category at CT and/or MRI has not yet been evaluated, to the knowledge of the authors. Purpose To assess the diagnostic performance of the LR-TIV category in detecting macroscopic tumors in veins (TIVs) at CT and hepatobiliary contrast agent-enhanced (HBA) MRI, with pathologic results used as the reference standard. Materials and Methods Between January 2010 and December 2019, consecutive patients with or without macroscopic TIV who underwent both CT and HBA MRI before hepatic resection or liver transplant were retrospectively included. Three radiologists independently assessed the LR-TIV features of enhancing soft tissue in vein and features suggestive of TIV (FSTIV) and reached a consensus. Macroscopic TIV at pathologic examination was the reference standard. Sensitivities and specificities of the LR-TIV category without and with FSTIV were calculated, and the added value of FSTIV was evaluated by using the McNemar test. Results In the 1322 patients with (n = 101) or without (n = 1221) macroscopic TIV (median age, 64 years [interquartile range, 58-70 years]; 1053 men), without consideration of FSTIV, the sensitivity and specificity of enhancing soft tissue in vein for detecting macroscopic TIV at pathologic examination were 64.4% (65 of 101) and 99.8% (1218 of 1221) with CT and 62.4% (63 of 101) and 99.8% (1218 of 1221) with HBA MRI, respectively. With consideration of FSTIV, the sensitivity and specificity of the LR-TIV category became 67.3% (68 of 101 patients) and 99.7% (1217 of 1221 patients) at both CT and HBA MRI. No difference was found between measurements without and with FSTIV (sensitivity, 62% vs 67% for CT [P = .45] and 64% vs 67% for HBA MRI [P = .18]; specificity, 99% for both CT and HBA MRI [P > .99 for both]). Conclusion The Liver Imaging Reporting and Data System tumor in vein category showed moderate sensitivity and high specificity in the detection of macroscopic tumors in veins at both CT and hepatobiliary contrast agent-enhanced MRI, with pathologic examination used as the reference standard. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Morrell in this issue.

Femoral Artery Leiomyosarcoma: Case Report and Literature Review.

BACKGROUND: Vascular leiomyosarcoma is a rare type of malignant tumor which arises from the smooth muscle tissue of blood vessel walls; it involves veins five times more frequently than arteries. There are only a few cases published in the literature and consequently there is limited experience regarding treatment and prognosis. METHODS: A 66-year-old woman presented with left lower limb swelling and a left inguinal mass. Imaging revealed a seven-by-five cm well-circumscribed oval inguinal mass that incorporated the common femoral artery including its bifurcation and compressed the common femoral vein. Other malignancies or metastatic disease were excluded. The patient underwent en bloc resection of the tumor, including the common femoral artery and its bifurcation and arterial reconstruction, using the inverted contralateral great saphenous vein, was carried out. Histopathological examination of the mass revealed moderately-differentiated leiomyosarcoma arising from the femoral artery wall without invasion of the intima. The postoperative course was uneventful. 12 months after the procedure the patient was in good clinical conditions and a contrast enhanced CT scan showed patency of the arterial reconstruction without local recurrence or metastatic disease. RESULTS: A systematic literature search identified nine cases of femoral artery leiomyosarcoma; in the eight patients for whom follow-up data were reported, recurrent or metastatic disease developed in five and only three were alive and free of disease. CONCLUSIONS: As with any soft tissues sarcoma, complete surgical resection is the cornerstone of treatment and any directly involved adjacent structures must be sacrificed, as well as a margin of uninvolved normal tissue; consequently, a vascular reconstruction is almost always necessary.

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.

Pulmonary artery sarcoma treated with pulmonary endarterectomy and leaflet reconstruction using Ozaki technique.

Pulmonary arterial intimal sarcomas (PAIS) are rare malignancies with a poor prognosis. Sarcomas present with signs and symptoms mimicking pulmonary thromboembolic disease, delaying the diagnosis. We present a 29-year-old male patient diagnosed with PAIS in the right and main pulmonary arteries extending to the left pulmonary leaflet. The patient was treated with pulmonary endarterectomy and pulmonary leaflet reconstruction using the Ozaki technique.

Current progress and future perspectives of research on intravascular large B-cell lymphoma.

Intravascular large B-cell lymphoma is a rare disease of the large B cells characterized by selective growth in the lumina of small vessels in systemic organs. Since first reported in 1959, the difficulty of obtaining sufficient tumor cells from biopsy specimens has hampered the elucidation of its underlying biology. Recent progress using xenograft models and plasma cell-free DNA has uncovered genetic features that are similar to those of activated B-cell type diffuse large B-cell lymphoma, including MYD88 and CD79B mutations and frequent alterations in immune check point-related genes such as PD-L1 and PD-L2. Given the improvement in clinical outcomes and a higher risk of secondary central nervous system (CNS) involvement in the rituximab era, a phase 2 trial of R-CHOP combined with high-dose methotrexate and intrathecal chemotherapy as a CNS-oriented therapy has been conducted. This trial, the PRIMEUR-IVL study, has displayed good progression-free survival and a low cumulative incidence of secondary CNS involvement. Long-term follow-up within this trial is still ongoing. Further understanding of the pathophysiology of the disease and improvements in clinical outcomes are still needed.

Case 290: Intravascular Cystic Synovial Sarcoma.

History A 26-year-old man presented with a 1-month history of chest pain, a palpable and painful right inguinal mass, and edema in the right lower extremity. One month earlier, he started to experience left chest pain with no cough. Pulmonary CT angiography (CTA) revealed a left lower lobe segmental pulmonary embolus. The local hospital made a diagnosis of pulmonary embolism. He received anticoagulants, and his chest pain was gradually relieved. At the time of current presentation, the patient was experiencing right lower extremity swelling and pain. Physical examination revealed a 4 × 3 cm palpable right inguinal mass with no redness. His medical history and family history were negative. The results of laboratory work-up were normal, with a d-dimer level of 0.16 mg/L fibrinogen equivalent units (reference range, <0.46 mg/L) and an international normalized ratio of 2.45 (therapeutic range, 2.0-3.0 for a patient taking warfarin), except the prothrombin time was 28.2 seconds (reference range, 9.6-12.8 seconds) and the activated partial thromboplastin time was 52.2 seconds (reference range, 24.8-33.8 seconds). Echocardiography, chest radiography, chest CT, and contrast-enhanced (CE) CT revealed no abnormalities. The patient underwent right lower extremity vascular conventional US (Philips IU22; Philips) with an L9-3 probe (3-9 MHz, venous condition) and contrast-enhanced US (1.5-2.0 mL, SonoVue; Bracco) with an intravenous bolus injection at the initial evaluation. Two days later, noncontrast and contrast-enhanced CT images of the lower abdomen (1.5 mL per kilogram of body weight, 300 mg/mL iomeprol, Iomeron; Bracco) were acquired for further evaluation.

Intimal Sarcoma of the Great Vessels.

Intimal sarcomas of the pulmonary artery and aorta are rare entities with a poor prognosis. In many instances, pulmonary artery sarcomas are misinterpreted as acute or chronic pulmonary thromboembolism, whereas aortic intimal sarcomas are often misdiagnosed as protuberant atherosclerotic disease or intimal thrombus. Discernment of intimal sarcomas from these and other common benign entities is essential for the timely initiation of aggressive therapy. The most useful imaging modalities for assessment of a suspected intimal sarcoma include CT angiography, fluorine 18-fluorodeoxyglucose PET, and MRI. The authors discuss the clinical features, current treatment options, characteristic imaging findings, and underlying pathologic features of intimal sarcomas. The authors emphasize imaging discernment of intimal sarcomas and how their differential diagnosis is informed by knowledge of radiologic-pathologic correlation. The most reliable distinguishing imaging features are also emphasized to improve accurate and timely diagnosis. Online supplemental material is available for this article. ©RSNA, 2021.

Primary pulmonary artery sarcoma versus pulmonary thromboembolism: a multimodal imaging comparison.

Primary pulmonary artery sarcoma (PPAS) is a rare malignancy that is commonly mistaken for pulmonary embolism due to similarities in clinical presentation and radiographic findings. Distinct radiographic findings to help differentiate between the two diseases are highlighted in the case presented. (1) Several nuances in various imaging modalities have been identified to help distinguish pulmonary artery sarcoma from pulmonary thromboembolic disease. (2) The wall eclipsing sign is considered pathognomonic for pulmonary artery sarcoma. (3) Positron emission tomography/computed tomography may help reduce time between diagnosis and treatment, which may ultimately prolong survival. (4) Providers should be well versed on the subtle differences on imaging to prevent future delays in diagnosis and treatment.

A Large Vascular Leiomyoma Arising from the Superficial Femoral Artery: Case Report and Systematic Review.

OBJECTIVE: Vascular leiomyomas are rare begin tumor comprising mature vascular smooth muscles that originate in the tunica media of the blood vessels. Most of the tumors arise from the veins. Only a dozen cases of artery-arising vascular leiomyoma have been reported, most of which are presented as small nodules in the hand. METHODS: Here we report an interesting case of a large artery-arising vascular leiomyoma and perform a systematic review. RESULTS: A 55-year-old man complained a 6 × 3 cm firm, mobile, tender and pulseless mass in the medial region of his left thigh. The computed tomography showed a well-demarcated fusiform tumor encircling the superficial femoral artery and was enhanced significantly with contrast. The patency of the superficial femoral artery was intact. The magnetic resonance image exhibited slightly hypointense relative to skeletal muscle on T1-weighted images and a heterogeneous appearance on T2-weighted images. We performed the en bloc resection of the tumor and used a vascular graft to revascularize the artery through end-to-end anastomosis. After histopathological assessments, the tumor was diagnosed as an artery-arising vascular leiomyoma. We also performed a systematic review on artery-arising leiomyomas, discovering 21 cases. Most of the artery-arising vascular leiomyomas were small nodules (mean length: 2.4cm) and most of them were superficial solitary mass located in the hand (13 cases, 62%). Excision of the tumor was an effective treatment. The histological subtype of the artery-arising vascular leiomyoma in all cases was solid type. During the follow-up of each patient, there was no recurrence. CONCLUSIONS: Artery-arising vascular leiomyomas are extremely rare. Most of them are painless and locate in the hand. Their pathological subtype is solid type in all patients. Due to their begin nature, excision is a cure with little chance of recurrence.

Abdominal Aorta Angiosarcoma after Endovascular Aneurysm Repair.

Primary tumors originating within the wall of the arteries are rare and they frequently manifest late, making effective treatment a challenge. We describe here a case of Abdominal Aorta AngioSarcoma masqueraded as an infected EndoVascular Aortic Repair. The knowledge of this pathology from vascular surgeons and radiologist is crucial, because a prompt diagnosis and treatment can improve the prognosis.

An epithelioid haemangioma of the ulnar artery masquerading as an aneurysm.

Epithelioid haemangiomas are rare benign vascular tumors that usually present as subcutaneous nodules in the head and neck area. Occasionally these tumors can arise in a peripheral artery. When it does so, it is often confused with an aneurysmal dilatation of the respective vessel. In these circumstances, surgical resection with vascular reconstruction is the preferred treatment option.

Epithelioid Angiosarcoma of the Popliteal Artery- Primary or Secondary? A Rare Disease Entity Difficult to Diagnose and Easily Overlooked-Literature Research and Case report.

INTRODUCTION: The popliteal artery is a common site of aneurysm formation, whereas sarcomas of the vascular system are very rare. The diagnosis is very difficult to establish. During our literature research we found only seven reports about angiosarcomas of the popliteal artery. Four of them were associated with aneurysms. Because of the poor prognosis early diagnosis is the key to successful treatment. REPORT: We present a well-documented case of an 83-year-old patient with an angiosarcoma of the popliteal artery diagnosed as a popliteal artery aneurysm at first. CONCLUSION: It is important to think of this rare, highly aggressive tumor entity. Especially the aneurysms that need revision surgery should cause suspicion- histological samples from the aneurysm wall should always be taken.

Femoral Vein Leiomyosarcoma Mimicking Thrombosis.

BACKGROUND: Leiomyosarcoma represents a diagnostic challenge since it is often misdiagnosed as deep venous thrombosis (DVT). METHODS: A 49-year-old woman with history of DVT and diagnosed with recurrency of thrombosis came to our observation for right thigh pain persistency. RESULTS: Duplex-Ultrasound (US) examination showed an oval mass inside the superficial femoral vein with color spots and blood flow signal in its context. Contrast enhanced computed tomography and magnetic resonance of the lower limbs showed the presence of vascularized lesion into the distal superficial femoral vein. Echo-guided biopsy revealed the presence of high grade leiomyosarcoma. A total body 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography demonstrated a rounded area of pathological increased FDG uptake, at the distal thigh level without metastatic localization. CONCLUSIONS: Our experience demonstrated that a multimodality approach may help to distinguish leiomyosarcoma from a blood clot in doubtful cases.

Epithelioid Hemangioendothelioma of the Lower Limb, Discovered by a Claudication.

Epithelioid hemangioendothelioma (EHE) is a rare case of a tumor with different clinical behaviors and a difficult anatomopathological diagnosis. The diagnosis of EHE is usually confirmed by postoperative histopathologic examination. Actually, it is a challenge to put a correct diagnosis and to propose aggressive treatment. We report a case of an EHE of the left lower limb discovered in a 53-year-old claudicant woman. Surgical resection, arterial, and venous bypass were performed. The histology demonstrated EHE with a low mitotic index, emerging for the femoral vein.