A rare case of epithelioid angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis. We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall's integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery. To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm. Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.

Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review.

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

Contrast-Enhanced Ultrasonography in Diagnosing Intravascular Large B-Cell Lymphoma Infiltrating Liver Sinusoids.

BACKGROUND Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within vasculature. This presents a diagnostic challenge due to non-specific symptoms and lack of tumor formation. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) provides useful information in diagnosing FDG-avid lymphoma, but is not specific to  IVLBCL. Contrast-enhanced ultrasonography (CEUS) is useful in evaluating focal liver lesions; however, its efficacy in diagnosing IVLBCL involving the liver remains unknown. CASE REPORT We report the case of an 83-year-old woman presenting with fever, pancytopenia, liver dysfunction, and elevated LD and soluble interleukin-2 receptor levels. PET-CT showed multiple uptake lesions in the liver. We performed CEUS with Sonazoid® to evaluate the mass-like lesions; however, no nodular lesions were observed in B mode images. Systemic enhancement was seen in the early phase but no defect was observed in the post-vascular phase. The latter finding suggested preserved Kupffer cells function, excluding tumor-forming lymphoma and liver metastases. Suspecting IVLBCL, we performed a bone marrow examination, which showed sinusoidal infiltration of large neoplastic cells positive for CD20. The patient's condition deteriorated rapidly and she died 2 days after the examination. Autopsy revealed diffuse infiltration of lymphoma cells into liver sinusoids with preserved Kupffer cells, leading to the diagnosis of IVLBCL. CONCLUSIONS Our case shows that CEUS can distinguish IVLBCL from mass-forming lymphoma based on the absence of a defect in the post-vascular phase in a patient with clinically and radiographically suspected lymphoma involving the liver. This can assist clinicians to select appropriate lesions for biopsy.

Resection of primary left renal vein leiomyosarcoma with renal preservation following an unusual clinical presentation.

An elderly man was referred to vascular surgery on incidental discovery of a left retroperitoneal mass ultimately found to be of left renal vein (LRV) origin. He initially presented with recurring lower back pain. CT of the abdomen/pelvis showed a 6.0×5.5 cm lobulated retroperitoneal mass anterior to the infrarenal aorta. Resection of the mass necessitated a multidisciplinary team consisting of medical oncologists, radiation oncologists, urologists and vascular surgeons. In efforts to obtain an R0 margin, en-bloc resection of the LRV from its confluence with the inferior vena cava (IVC) was necessary. A primary repair of the IVC was performed with preservation of the left kidney. The patient's back pain has since resolved after the surgery. A literature search found IVC reconstructions to be safe and effective in the removal of vascular leiomyosarcomas.

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.

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.

Intravascular leiomyomatosis with extension to the inferior vena cava.

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.

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.

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.

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.

Leiomyosarcoma of Inferior Vena Cava in an Immunocompetent Young-adult Female Patient.

Leiomyosarcoma of the inferior vena cava is an extremely rare malignancy originating from the smooth muscle of the vessel wall, with only a few hundred cases reported in the literature. There are no clear guidelines for treatment, but surgical resection is currently the only curative option. Further research is needed to better understand the disease and guide its management. We report a case of a 39-year woman who presented to the emergency room with a four-day history of nonspecific abdominal pain, nausea and fever. An abdominal computed tomography revealed a mass in relation with the inferior vena cava and hepatic nodules. Histological examination proved it to be a leiomyosarcoma of inferior vena cava with liver metastases. Key Words: Leiomyosarcoma, Inferior vena cava, Sarcoma.

Histopathology of Vascular Tumors.

Vascular tumors are neoplasms of endothelial cell origin. These tumors comprise a broad and complex group of vascular anomalies. Diagnostic classification of these lesions is based on their unique clinical, radiologic imaging, histopathologic, and molecular characteristics. This article follows the published classification of the International Society for the Study of Vascular Anomalies. It describes characteristic histopathologic findings in vascular tumors and information on known genetic causes.