scholarly journals A Diagnostic Program of Vascular Tumor and Vascular Malformations in Children According to Modern Classification

2017 ◽  
Vol 60 (1) ◽  
pp. 19-26
Author(s):  
Iryna Benzar

The aim of the study was to analyze the cohort of inpatient children with vascular anomalies according to the globally accepted classification introduced by the ISSVA. Methods: The study included 205 inpatient children within the time period of the years 2010–2015. Types of vascular anomalies (VAs), age of patients, diagnostic procedures, and anatomical localization of VAs were analyzed. Results: 65 patients of first year of life had vascular tumors, with prevalence of infantile hemangiomas (IHs) in 57 (87.7%) patients. 45 children had IHs localized within soft tissues, whereas 7 patients suffered from IHs of the liver, and 5 children from IHs of the respiratory tract. Most patients with soft tissue IHs were diagnosed only with ultrasound; СT or MRI diagnostics were performed on 5 (8.8%) patients, and biopsy was carried out in 2 (4.4%) children. Vascular malformations (VM) were diagnosed in 140 (68.3%) patients. Ultrasound investigation (US) was the screening method. MRI was performed to confirm the diagnosis of low-flow VM, whereas for high-flow VM CT angiography and selective angiography were useful. Venous malformations were diagnosed in 17 (12.1%) patients, and 112 (80.0%) had cystic LM, among them children under the age of 2 years prevailed. Arteriovenous malformations were diagnosed in 5 (3.8%) patients, ages 2–14 years. Conclusions: Clinical manifestations of vascular anomalies have clear age features. Among hospitalized children vascular tumors add up to 31.7% and VM – up to 68.3%.

2015 ◽  
Vol 6 (3) ◽  
pp. 109-111 ◽  
Author(s):  
Jatin P Shah ◽  
Ivana Petrovic ◽  
Ben Roman

ABSTRACT Background Vascular anomalies are divided into vascular tumors, hemangiomas being the most common, and vascular malformations. Most vascular anomalies are noticed at birth or occur during infancy, and generally involve skin or subcutaneous soft tissues. Adult onset hemangiomas are rare, and intramuscular location is extremely rare. Surgical excision is recommended for hemangiomas in adults, if they are symptomatic, or manifest growth. Materials and methods We report a rare case of a 51-year-old woman, with an intramuscular hemangioma of the tongue, presenting as a submental mass. Preoperative imaging for assessment of tumor extent was followed by a successful surgical excision. Results Postoperative course was uneventful with primary healing of the wound, and with no functional deficit of tongue function. Conclusion Although a variety of treatment approaches are reported for childhood hemangiomas, surgical excision is the preferred treatment for adult onset symptomatic hemangiomas. Preoperative work up should include imaging preferably with contrast enhanced magnetic resonance imaging (MRI). Embolization may be considered for larger lesions. Intraoperative hypotension should be avoided to ensure identification of the entire lesion to ensure complete excision. How to cite this article Petrovic I, Roman B, Shah JP. Cavernous Hemangioma of the Tongue. Int J Head Neck Surg 2015;6(3):109-111.


2019 ◽  
pp. 241-260
Author(s):  
Lara Wine Lee ◽  
Cameron C. Trenor

The chapter provides a description of the main anomalies characterized by excessive proliferation of the vascular tissue and the major syndromes associated with them. The predominant types of vascular anomalies are vascular tumors and vascular malformations. Vascular tumors are further subdivided in benign, locally aggressive, and malignant, according to the clinical course of the lesions, while vascular abnormalities are classified as simple (capillary, lymphatic, venous, arteriovenous, and arteriovenous fistula) or combined. For each subgroup of vascular anomalies, clinical presentation and diagnostic procedures are discussed, as well as pathogenic mechanisms and potential comorbidities. The management of vascular anomalies may be oriented to treat the aesthetic discomfort as well as the functional impairment and may involve both pharmaceutical and surgical approaches.


Author(s):  
Anushka Mittal ◽  
Rama Anand ◽  
Richa Gauba ◽  
Subhasis Roy Choudhury ◽  
Pooja Abbey

AbstractVascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.


Author(s):  
R. BECKERS ◽  
D. GOVAERTS ◽  
M. DESMEDT ◽  
G. MALEUX ◽  
F. COMBES ◽  
...  

Extraoral vascular anomalies in the head and neck area Vascular anomalies have a prevalence of 4.5%, of which most occur in the head and neck area. In this retrospective cohort study, extraoral vascular anomalies are categorized based on the classification of the International Society for the Study of Vascular Anomalies (ISSVA). Subsequently, an analysis of the clinical presentation, treatment and complications of the 75 included patients was performed. The proportion of vascular tumors and malformations in this study was 24% (n = 18) and 76% (n = 57) respectively. Most of the extraoral vascular tumors and malformations occurred respectively in the lip (66.7%; n = 12) and cheek area (40.4%; n = 23). The most frequent complaint when patients presented with a vascular tumor, was the wish to be diagnosed (77.8%; n = 14). Swelling was the most frequent complaint when patients (35.1%; n = 20) presented with a vascular malformation. In 48.1% (n = 26) of the patients receiving interventional treatment, at least one recurrence was seen (n = 54), and almost exclusively in vascular malformations (96.2%; n = 25). Interventional therapy of vascular malformations is complex and associated with a pronounced risk of complication and recurrence, which is why multidisciplinary care for these patients is often an added value. The treatment of vascular tumors results in minimal morbidity or recurrence.


2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Gresham T. Richter ◽  
Adva B. Friedman

Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies.


2018 ◽  
pp. 301-314
Author(s):  
David T. Jacobs ◽  
David C. Moe ◽  
Patricia E. Burrows

This chapter, on peripheral malformations, provides an overview of vascular anomalies encountered in the practice of interventional radiology (IR), with a brief discussion of vascular tumors followed by vascular malformations. These include low-flow lesions, lymphatic malformations (LMs) and venous malformations (VMs); high-flow lesions, arteriovenous malformations (AVMs) and arteriovenous fistula (AVF); and Klippel-Trenaunay syndrome (KTS), the combined vascular lesion syndrome of the lower extremity. The natural history, signs and symptoms, clinical appearance, imaging appearance, and treatment of these lesions will be discussed, and illustrative photographs and radiologic studies of typical cases are provided. In recent years, much has been learned about the genetic basis of vascular malformations, which are caused by somatic mutations in a number of angiogenic proteins. Pharmacological treatment, based on these molecular findings, is available for some lesions. Extensive discussion on the biological basis for vascular anomalies is beyond the scope of this chapter.


Author(s):  
Maliha Sadick ◽  
René Müller-Wille ◽  
Moritz Wildgruber ◽  
Walter Wohlgemuth

Background Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities. Method A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed. Results Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient’s age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %). Conclusion The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies. Key Points  Citation Format


Author(s):  
Maliha Sadick ◽  
Daniel Overhoff ◽  
Bettina Baessler ◽  
Naema von Spangenberg ◽  
Lena Krebs ◽  
...  

Background Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning. Method In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools. Results Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics. Conclusion Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies. Key Points:  Citation Format


Author(s):  
E. K. Donyush ◽  
Z. A. Kondrashova ◽  
Yu. A. Polyaev ◽  
R. V. Garbuzov

Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.


Author(s):  
Ajay K. Khanna ◽  
Akhilesh Kumar ◽  
Soumya Khanna ◽  
Amrita Kar ◽  
Puneet Kumar ◽  
...  

Abstract Vascular anomalies grouped into vascular tumors (hemangioma) (HI) and vascular malformation (VM) are benign vascular lesions that are difficult to distinguish from one another clinically and often confused with each other at histopathology. This confusing terminology leads to improper diagnosis, illogical treatment, and misdirected research. This study aimed to study GLUT 1, S-100, and nerve bundle to differentiate hemangioma and vascular malformation. Thirty two cases of vascular lesions (26 vascular malformations and 6 hemangiomas) were taken into the study. For histological evaluation and immunohistochemistry (IHC), samples of vascular lesions were collected in formalin. All the hematoxylin and eosin-stained slides were evaluated under light microscope for histology and nerve bundles. Immunohistochemical staining was performed by streptavidin–biotin method for GLUT 1 and S-100. GLUT 1 was positive in all 6 cases of hemangiomas (100%) and only in 1 case of vascular malformation. Nerve bundle was present in 24 cases of vascular malformation (92.3%) out of 26 cases but not in any cases of hemangioma and S-100 was found positive in all vascular malformation cases (100%) but not in hemangioma. So GLUT 1 expression, S-100, and presence of nerve bundle in vascular lesions can help to differentiate hemangioma and vascular malformation.


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