Primary thoracic aorta angiosarcoma presenting with thromboembolism and progressive claudication despite anticoagulation.

Primary aortic angiosarcomas (PAA) are rare angiosarcomas, frequently diagnosed in advanced stages due to initial misdiagnosis. This case describes a 66-year-old woman, initially presenting with a distal thoracic aorta thrombus and symptomatic bilateral popliteal emboli. Despite initial management and therapeutic anticoagulation, she experienced progressive lower limb claudication and 12 months following initial presentation she re-presented with an obstructing distal thoracic aorta mass and metastatic disease. Histopathology confirmed metastatic epithelioid angiosarcoma. Despite urgent palliative radiotherapy, she died 6 weeks after diagnosis from complications of tumour thromboembolism. Suspicion for PAA should be raised in the case of thrombus in atypical segments (e.g. thoracic aorta) or progressive course despite anticoagulation. Multimodal imaging including MRI and FDG-PET is useful to distinguish from benign aetiologies.

[Multiple, eruptive epithelioid hemangiomas of the scalp]. / Multiple, eruptive epitheloide Hämangiome der Kopfhaut.

Epithelioid hemangioma is a benign vascular neoplasm with a characteristic histological and immunohistochemical pattern, characterized by a lymphocytic inflammatory infiltrate with admixed eosinophils and FOS­B expression. The correct diagnosis is of particular relevance, since malignant vascular tumors with differentiated epithelioid cells can also be considered in the differential diagnosis. We present a patient with multiple epithelioid hemangiomas of the scalp accompanied by severe pain and itching. The long history of multiple therapeutic attempts illustrates the limited success of currently available treatment options.

Contemporary diagnostic approach to atypical vascular lesion and angiosarcoma.

Vascular neoplasms account for a substantial fraction of cutaneous mesenchymal tumors, spanning from clinically indolent benign lesions to highly aggressive malignancies. These neoplasms present a distinctive challenge in terms of their diagnostic histopathology, both because of the breadth of their morphological manifestations and because of the significant histological overlap between different entities, even benign and malignant ones. The post-radiotherapy setting is particularly problematic diagnostically, insofar as radiation exposure predisposes not only to secondary angiosarcoma, but also to atypical vascular lesion, a largely benign proliferation of cutaneous blood vessels typically affecting the breast. To address these challenges, we explore the clinical, histological, and molecular features of malignant vascular neoplasia, including primary and secondary subtypes, through the comparative lens of atypical vascular lesion. In addition to highlighting the key morphological indicators of malignancy in superficial vasoformative tumors, we offer an approach that integrates clinical characteristics and molecular genetic profiling to facilitate accurate classification. With this current knowledge as our foundation, we also look ahead in an effort to frame some of the key unanswered questions regarding superficial vascular malignancies and their natural history, clinical management, and molecular underpinnings.

Intraoral healing type of pyogenic granuloma (A benign vascular tumor): A case report.

Pyogenic granulomas represent tumor-like lesions affecting the skin and the oral cavity. This classic definition can be somewhat misleading because such lesion is not associated with infection and lacks any clinical evidence of pus or histological evidence of actual granulation tissue. This case report describes a surgical excision of the growth to exclude angiomatous proliferation. The patient reported a chief complaint of localized gingival overgrowth since 4 months. Intraoral examination revealed an irregular, sessile exuberant growth in respect to labial aspect and interdental gingiva of 31, 32, and 33, measuring about 1.6 × 1.1 cm. Based on the clinical findings, the case was provisionally diagnosed as "pyogenic granuloma". A treatment was planned for the patient. A surgical excision was done irt 31, 32, 33, and the tissue was sent for histopathological examination, which was suggestive of a healing type of pyogenic granuloma.

[Infantile hemangioma : Clinical manifestation, treatment, and differential diagnoses]. / Infantile Hämangiome : Manifestationsformen, Therapie, Differenzialdiagnosen.

With an incidence of approximately 4% infantile hemangiomas are the most common vascular tumors in children and show characteristic growth dynamics. In order to avoid erroneous treatment, they need to be differentiated from other vascular tumors (granuloma pyogenicum and kaposiform hemangioendothelioma) and vascular malformations. Of all infantile hemangiomas 85% are uncomplicated and undergo spontaneous resolution starting towards the end of the first year of life. First-line treatment for complicated infantile hemangiomas (15%), i.e. those with imminent obstruction (eyes and nose), ulceration or permanent disfigurement, is oral propranolol (2 mg/kg BW and day for at least 6 months).

Incidence and Outcome of Breast Sarcomas in England (2013-2018): An analysis from the National Cancer Registration and Analysis Service.

BACKGROUND: Breast sarcomas (BS) are rare cancers originating from mesenchymal breast tissue with a paucity of national population level data detailing their incidence and outcomes. METHODS: We performed an analysis of data collected by National Cancer Registration and Analysis Service (NCRAS) for patients diagnosed with BS between 2013 and 2018. Chi-square test was used to compare groups. Overall survival (OS) was calculated by Kaplan-Meier. Specialist sarcoma centres (SSC) were defined as centres with a sarcoma multidisciplinary team (MDT). RESULTS: There were 684 patients with BS (357 malignant phyllodes tumours [PTs], 238 vascular tumours, 93 other morphology) with a median age of 64 (range 14-96); 187 (27%) had received breast radiotherapy for a prior malignancy; 633 (92%) had resection of the tumour within 12 months of diagnosis. Five-year OS was 82%, 54% and 48% in patients with PT, vascular tumours and other sarcomas, respectively, and 55% for those with radiation-induced BS. Patients managed within SSC more frequently had a biopsy prior to surgery 83% versus 72%, p < 0.05) and were less likely to require multiple operations (26% versus 41%, p < 0.05). Tumour stage and grade data were not available. CONCLUSION: This is the first population series evaluating incidence and outcomes for BS. Patients treated at non-specialist sarcoma centres (NSSCs) are less likely to have a biopsy prior to surgery and more likely to require multiple operations. Based on these observational data, we would recommend all BS are discussed at a sarcoma MDT meeting early in their pathway and surgery to be considered at SSC where possible.

HEPATIC VASCULAR TUMORS IN CHILDREN: POTENTIAL RISKS, OPTIMAL IMAGING AND THE ROLE OF SURGICAL INTERVENTION.

OBJECTIVE: The aim: To revise the case-series of hepatic vascular tumors (HVT), particularly to identify optimal visualization, management and role of surgical intervention. PATIENTS AND METHODS: Materials and methods: Out of 96 children with hepatic tumors who hospitalized in a single center from 2011 to 2020, 20 (20,8%) were diagnosed HVT. Hepatic Hemangiomas (HHs) were presented in 19 patients and Kaposiform hemangioendotelioma (KHE) in one case. To determine the type of HH we used radiological classification. For visualisation contrast-enhanced MRI (n=7, 30%) and cCT (n=15,70%) were used. Follow-up period was 14-77 months. RESULTS: Results: All HVT were revealed by sonogram at the age of 0-5 m, with 4 (20%) diagnosed prenatally. Male to female ratio was 3:2. Beta-blockers were prescribed to 12 patients with HHs. Treatment duration was from 6 to 24 month. Steroid therapy was initial in cases when it was impossible to prescribe the curative dose of beta-blockers. Complications of propranolol treatment were transitory bradycardia (n=7) and transitory hypoglycemia (n=2). After vincristine chemotherapy decreases the tumor size by 54%, that allowed a safe liver resection. 4 (20%) patients - two multifocal HHs, one diffuse HH and in patient with KHE manifested congestive heart failure and pulmonary hypertension Mortality rate is 5% (n=1), this patient died against progressive cardiovascular failure. CONCLUSION: Conclusions: life-threatening complication of HVT was congestive heart failure. Early treatment is beneficial for complications prevention. Surgical treatment is optional for KHE when can be removed safely.

SURGICAL TECHNIQUE IN LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DEPENDING ON ITS LOCATION.

OBJECTIVE: The aim: To improve the outcomes of inferior vena cava (IVC) leiomyosarcoma, propose own classification of IVC segments, which correlates with surgical access, methodology, sequence and amount of surgery. PATIENTS AND METHODS: Materials and methods: In the period from 1991 to 2021 in the Transcarpathian Regional Clinical Hospital named after A. Novak and in the Transcarpathian Antitumor Center 8 patients with IVC leiomyosarcoma were operated. The prevalence of leiomyosarcoma in IVC was determined according to the division of IVC into 7 segments. Defeat of one segment of IVC was in 50% of cases, two - in 37.5%, three - in 12.5%. In 5 (62.5%) cases circular resection and alloprosthesis of IVC were performed; in 2 (25%) - circular resection, alloprosthesis of IVC and implantation of the right and left renal veins in the prosthesis; in 1 (12.5%) - circular resection, alloprosthesis of IVC and implantation of the left renal vein in the prosthesis. All surgeries were performed with laparotomy access (87.5% by Chevron type). RESULTS: Results: The average operation time was 215 (160-320) minutes, the average blood loss was 305 (250-500) ml. Postoperative complications were recorded in 2 (25%) cases. There were no cases of pulmonary embolism, venous thrombosis, prosthesis thrombosis, perioperative mortality. In 7 (87.5%) cases, surgery was radical. The overall 1-year, 2-year and 3-year survival rates were 87.5%, 71.4% and 57.7%. CONCLUSION: Conclusions: The division of IVC into 7 segments characterizes the detailed definition of the cranial limit of leiomyosarcoma and segmental involvement of IVC in the tumor process, which allows to choose the right surgical tactics, perform radical resection of IVC and maintain laminar blood flow to IVC and its tributaries.

Pulmonary artery intimal sarcoma: A rare cause of filling defects in pulmonary arteries.

Pulmonary artery intimal sarcomas are very rare and arise from primitive pluripotent mesenchymal cells. They are often misdiagnosed as pulmonary thromboembolism, leading to futile anticoagulation treatment and delayed diagnosis. We present a case of a patient who showed nonspecific pulmonary symptoms and characteristic imaging manifestation. Progressive symptoms and additional imaging led to the suspicion of a pulmonary artery intimal sarcoma, which was finally confirmed by pathological biopsy. This case serves as a reminder to consider pulmonary artery intimal sarcomas in the differential diagnosis of patients with dyspnea and filling defects on computed tomography pulmonary angiography or contrast-enhanced computed tomography.

TRIANGULAR SIGN OF AMALRIC IN INTRAVASCULAR LYMPHOMA.

PURPOSE AND METHODS: To report a case of bilateral ocular ischemia caused by intravascular lymphoma with the presence of bilateral triangular sign of Amalric. RESULTS: A 67-year-old woman was admitted to our hospital for progressive paraplegia and a 6-month history of acute painless vision loss in the right eye. Initial examination showed vision of counting fingers in the right eye and 20/20 in the left eye with normal anterior examination. Fundus examination revealed a likely previous central retinal artery occlusion in the right eye with pale nerve and attenuated vessels. Both fundi had triangular regions of pigmentary change known as the triangular sign of Amalric, indicative of choroidal ischemia. However, the left eye was asymptomatic. Neuroimaging revealed multifocal enhancing lesions throughout the central nervous system of unclear etiology. An extensive neurologic and systemic workup was unrevealing, including a brain biopsy, and empiric treatment for an unspecified inflammatory condition with intravenous corticosteroids was initiated. During her hospitalization, she developed acute painless vision loss in the left eye, and examination showed no light perception vision in both eyes with signs of acute retinal and choroidal ischemia in the left eye. A subsequent brain biopsy revealed intravascular lymphoma. CONCLUSION: Triangular pigmentary changes indicate choroidal ischemia and can be seen in many conditions. This patient presented with the triangular sign of Amalric in both eyes, including her asymptomatic left eye. Intravascular lymphoma should be considered in cases of concomitant inflammatory brain lesions and chorioretinal ischemia.

Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism.

BACKGROUND Intravenous leiomyomatosis (IVL) is a rare benign smooth muscle tumor originating in the uterus or in the uterine vessels. It is characterized by continuous intraluminal growth that may extend through iliac veins and inferior vena cava (IVC) to right chambers of the heart and pulmonary vasculature, leading to life-threatening complications. This case report describes an uncommon cause of non-thrombotic pulmonary embolism in young woman caused by extensive IVL. CASE REPORT A 39-year-old woman was admitted after multiple syncopal episodes. She was initially found to have a bilateral pulmonary embolism and large right atrial mass believed to be a thrombus. After an unsuccessful attempt to remove the thrombus with AngioVac (AngioDynamics, Latham, NY), subsequent sternotomy revealed a large pedunculated mass extending to the infra-hepatic IVC. Further abdominal imaging showed multiple uterine masses, with the largest about 17 cm, infiltrating the parauterine vessels and extending through the right iliac vein and inferior vena cava up to the right atrium. Pathology examination of the atrial mass revealed benign leiomyoma consistent with further pathology findings after hysterectomy. The pulmonary embolism was believed to be caused by tumor tissue, and anticoagulation was abandoned. Pulmonary nodule raised a suspicion of benign pulmonary metastases, but, fortunately, remained stable during follow-up and the patient had a successful recovery. CONCLUSIONS Available information about IVL is scarce. This tumor, although benign and rare, should be included in the differential diagnosis of cardiac tumors and non-thrombotic pulmonary emboli in women with predisposing risk factors, as potential complications are life-threatening.

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.

Intravenous leiomyomatosis misdiagnosed with large thrombosis in inferior vena cava.

OBJECTIVE: The aim of this report is to highlight the importance of a comprehensive preoperative evaluation in the case of intravenous leiomyomatosis. CASE REPORT: A 49-year-old women was presented with dyspnea and abdominal distension. Imaging studies revealed a large leiomyoma with intravenous leiomyomatosis from this mass to the right parauterine veins, right ovarian vein reaching the inferior vena cava. Complete resection was performed by a two-stage operation by a multidisciplinary team. Final pathology confirmed it to be intravenous leiomyomatosis and uterine leiomyomas. CONCLUSION: Intravenous leiomyomatosis is a benign and rare disease that can be a fatal condition. Precise diagnosis and appropriate treatment are important for the best outcome. Gynecologists should consider this rare disease when a patient with a uterine tumor shows symptoms such as chest pain and dyspnea.

Primary marginal zone B-cell lymphoma of the cavernous sinus: a case report and review of the literature.

BACKGROUND: Primary lymphoma of the cavernous sinus is a rare form of extranodal non-Hodgkin lymphoma, of which very few cases have been reported in the published literature. This report presents the MRI findings with apparent diffusion coefficient (ADC) value in an exceedingly rare primary marginal zone B-cell lymphoma (MZBCL) of the cavernous sinus. CASE PRESENTATION: The case in this study is a 59-year-old immunocompetent male patient with a 2-month history of right ptosis and blurred vision. Right third cranial nerve palsy and binocular diplopia were observed upon neurological examination. Preoperative brain CT showed an extra-axial enhancing mass lesion in the right cavernous sinus. On MRI, ipsilateral internal carotid arterial encasement was noted without causing stenosis of the vessel. Isointense signal on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and diffusion restriction were also observed. The mean ADC value of the tumor is 0.64 × 10-3 mm2/s (b value = 1000 s/mm2). Subtotal resection of the tumor was performed, and improvement of clinical symptoms were observed. The pathologic diagnosis of MZBCL was established by immunohistochemical examinations. CONCLUSIONS: Primary MZBCL of the cavernous sinus is exceedingly rare, and preoperative confirmation poses a major challenge with CT and conventional MRI only. In this case, preoperative quantitative ADC value is shown to offer valuable additional information in the diagnostic process.