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.

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.

Clinical outcomes after inferior vena cava resection for malignant disease. A single center experience of 51 vena cava resections.

BACKGROUND AND OBJECTIVE: For tumors involving inferior vena cava (IVC), surgery with complete resection remains the first line treatment. Management of IVC after resection, either ligation without reconstruction or primary reconstruction, is debated. Our study aimed to evaluate type of venous reconstruction, anticoagulation management and morbidity. METHODS: A French single center database of patients who underwent partial or total circumferencial resection of the IVC for malignant disease was analyzed. Inclusion criteria were any oncologic procedure for a retroperitoneal neoplasm requiring concomitant resection of the IVC with or without venous reconstruction with prosthesis. Exclusion criteria were surgery before year 2000. Data were descriptive and reverse Kaplan Meier was used for follow-up calculation. The endpoints were the rate of prosthetic reconstruction, the use of anticoagulation and the post-operative outcomes. RESULTS: Fifty - one patients were included with a median duration of follow-up of 54.8 months. The majority of patients were men (56.9%). Median age of the population was 44.1 years. Most of the patients underwent surgery for primary testicular cancer and for sarcoma. Complete IVC resections were performed in 46 (90,2%) patients, 32 having a concomitant prosthetic replacement. Eight patients underwent aortic resection in the same operative time. Postoperative morbidity was 33.3%. Post-operative anticoagulation was done in 24 patients. At 1 month, four patients developed thrombosis in the prosthesis. CONCLUSIONS: IVC resections are feasible and safe. Venous reconstruction and postoperative management were planned according to the preoperative imaging and intraoperative findings. We propose a decision-tree for peri-operative management and anticoagulation.

Analysis of risk factors for post-operative recurrence or progression of intravenous leiomyomatosis.

OBJECTIVE: To analyse the risk factors for post-operative recurrence or progression of intravenous leiomyomatosis and explore the impact of different treatment strategies on patient prognosis. METHODS: Patients with intravenous leiomyomatosis who underwent surgery from January 2011 to December 2020 and who were followed for ≥3 months were included. The primary endpoint was recurrence (for patients with complete resection) or progression (for patients with incomplete resection). Kaplan-Meier survival analysis was used to analyse the factors affecting recurrence. RESULTS: A total of 114 patients were included. The median age was 45 years old (range 24-58). The tumors were confined to the uterus and para-uterine vessels in 48 cases (42.1%), while in 66 cases (57.9%) it involved large vessels (iliac vein or genital vein and/or proximal large veins). The median follow-up time was 24 months (range 3-132). Twenty-nine patients (25.4%) had recurrence or progression. The median recurrence or progression time was 16 months (range 3-60). Incomplete tumor resection (p=0.019), involvement of the iliac vein or genital vein (p=0.042), involvement of the inferior vena cava (p=0.025), and size of the pelvic tumor ≥15 cm (p=0.034) were risk factors for recurrence and progression. For intravenous leiomyomatosis confined to the uterus or para-uterine vessels, no post-operative recurrence after hysterectomy and bilateral oophorectomy occurred in this cohort. Compared with hysterectomy and bilateral oophorectomy, the risk of recurrence after tumorectomy (with the uterus and ovaries retained) was significantly greater (p=0.009), while the risk of recurrence after hysterectomy was not significantly increased (p=0.058). For intravenous leiomyomatosis involving the iliac vein/genital vein and the proximal veins, post-operative aromatase inhibitor treatment (p=0.89) and two-stage surgery (p=0.86) were not related to recurrence in patients with complete tumor resection. CONCLUSION: Incomplete tumor resection, extent of tumor lesions and size of the pelvic tumor were risk factors for post-operative recurrence and progression of intravenous leiomyomatosis.

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.

[Diagnosis and treatment of intravenous leiomyomatosis].

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

Large retroperitoneal sarcoma invading the inferior vena cava successfully resected. Technical notes of two cases.

Retroperitoneal sarcomas are rare neoplasms . They frequently reach a very large size and invade adjacent organs before they are detected. Involvent of the inferior vena cava is uncommon. Distant metastases are a late feature. The mainstay of treatment is compartmental resection and contiguous organ resection. We report two cases of right-sided massive primary retroperitoneal leiomyosarcoma in pauci symptomatic women. In both cases treatment consisted of radical surgery. En bloc resection of the tumor and surrounding tissues and organs as well as part of the right wall of the subrenal IVC. To close the wall defect direct suture repair was used resulting in a reduced caliber but no hemodynamic sequelae or endoluminal thrombi. All the resection margins, including the inferior vena cava wall, were negative. The postoperative course was unremarkable and caval blood flow was optimal. The current gold standard treatment for retroperitoneal sarcoma is en bloc multivisceral resectionresection. KEY WORDS: Peritoneal sarcoma, Surgery, Vena cava.

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.

A rare case of retro-hepatic inferior vena caval leiomyosarcoma - Computed tomography, surgical and intraoperative echocardiography imaging.

Leiomyosarcoma of retro-hepatic portion of inferior vena cava (IVC) is a rare vascular tumor with poor prognosis if adequate surgical resection is not achieved. Surgical repair includes dissection of the tumor and reconstruction of the IVC with a tube graft. Establishing a normal flow and gradient in IVC and hepatic veins is imperative for a successful repair. We report a case of retro hepatic IVC leiomyosarcoma where the preoperative computed tomography described the anatomy and extension of the tumor whereas intraoperative transesophageal echocardiography helped in the assessment of adequacy of surgical repair.

Gonadal vein leiomyosarcoma, from clinical practice to a literature review: surgical, oncological and histopathologic correlation.

BACKGROUND AND AIM: Vascular leiomyosarcomas are rare and generally originate from the muscular wall of the inferior vena cava. Leiomyosarcomas originating from the wall of the gonadal veins are rare and just about ten cases are described in literature. In the present paper, we have described a case of a LMS originating from the left gonadal vein. METHODS: A 44-year-old woman presented in March 2020 pain symptoms at the level of the left renal lodge. The subsequent CT and the biopsy confirmed the diagnosis of G2 grade LMS. The mass was then removed en bloc from the posterior and inferior pancreatic plane, from the aortic plane and from the retroperitoneal plane, post chemoteraphy. RESULTS: Pathologic report revealed a typical leiomyosarcoma, moderately differentiated G2 with minor dedifferentiated areas of pleomorphic leiomyosarcoma. CONCLUSIONS: The LMSs originating from gonadal veins represent an uncommon oncologic challenge. The radical en bloc excision represents the therapeutic gold standard.

Harmonic Scalpel-Assisted Endonasal Endoscopic Resection of the Vascular Tumors of the Anterior Skull Base.

Vascular tumors represent a challenging pathologic subset for surgical treatment as they show a propensity for profuse bleedings. This is especially applicable to the skull base region, where surgical access is difficult due to its complex anatomy. To overcome this problem, the authors introduced the use of a harmonic scalpel in endoscopic skull base surgery for vascular tumors. Here, the authors report the outcomes of endoscopic harmonic scalpel-assisted surgery in 6 juvenile angiofibromas and 2 hemangiomas. All surgeries were performed using Ethicon Endo-Surgery HARMONIC ACE 5 mm Diameter Shears. The median intraoperative blood loss was 400 mL (range: 200-1500 mL). The median length of hospital stay was 7 days (range: 5-10 days). Recurrence was recorded in 1 patient with juvenile angiofibroma, which was successfully resolved with revision surgery. In this institutional experience, ultrasonic technology showed precise cutting with minimal bleeding, resulting in reduced surgical morbidity compared with conventional endoscopic instruments.