Vascular and Perivascular Lesions of Skin and Soft Tissues in Children and Adolescents

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 26-61 ◽  
Author(s):  
Elisabeth Bruder ◽  
Rita Alaggio ◽  
Harry P. W. Kozakewich ◽  
Gernot Jundt ◽  
Louis P. Dehner ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Soft Tissues ◽  
Vascular Malformations ◽  
Classification Systems ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Malignant Neoplasms ◽  
Clinical Expertise ◽  
Full Spectrum ◽  
Specific Diagnosis

Vascular anomalies in children and adolescents are the most common soft tissue lesions and include reactive, malformative, and neoplastic tumefactions, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic complexity and recent changes in classification systems, some of which are based on clinical features and others on pathologic findings. In recent decades, there have been significant advances in clinical diagnosis, development of new therapies, and a better understanding of the genetic aspects of vascular biology and syndromes that include unusual vascular proliferations. Most vascular lesions in children and adolescents are benign, although the intermediate locally aggressive and intermediate rarely metastasizing neoplasms are important to distinguish from benign and malignant mimics. Morphologic recognition of a vasoproliferative lesion is straightforward in most instances, and conventional morphology remains the cornerstone for a specific diagnosis. However, pathologic examination is enhanced by adjunctive techniques, especially immunohistochemistry to characterize the type of vessels involved. Multifocality may cause some uncertainty regarding the assignment of “benign” or “malignant.” However, increased interest in vascular anomalies, clinical expertise, and imaging technology have contributed greatly to our understanding of these disorders to the extent that in most vascular malformations and in many tumors, a diagnosis is made clinically and biopsy is not required for diagnosis. The importance of close collaboration between the clinical team and the pathologist cannot be overemphasized. For some lesions, a diagnosis is not possible from evaluation of histopathology alone, and in a subset of these, a specific diagnosis may not be possible even after all assembled data have been reviewed. In such instances, a consensus diagnosis in conjunction with clinical colleagues guides therapy. The purpose of this review is to delineate the clinicopathologic features of vascular lesions in children and adolescents with an emphasis on their unique aspects, use of diagnostic adjuncts, and differential diagnosis.

Fibroblastic and Myofibroblastic Tumors in Children and Adolescents

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 127-180 ◽  
Author(s):  
Cheryl M. Coffin ◽  
Rita Alaggio
Keyword(s):  
Children And Adolescents ◽  
Molecular Genetic ◽  
Metastatic Potential ◽  
Malignant Neoplasms ◽  
Clinical Genetic ◽  
Full Spectrum ◽  
Specific Diagnosis ◽  
Important Group ◽  
Genetic Abnormalities ◽  
Common Group

Fibroblastic and myofibroblastic tumors in children and adolescents are a relatively common group of soft tissue proliferations that range from reactive to hamartomatous to neoplastic, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic and immunohistochemical overlap, despite significant clinical, genetic, and prognostic differences. The fibromatoses are a major subgroup, and all types of fibromatoses can occur in the 1st 2 decades of life. Intermediate and malignant fibroblastic-myofibroblastic tumors are an important group that includes variants of fibrosarcoma and other tumors with recurrent cytogenetic or molecular genetic abnormalities and low metastatic potential. Pathologic examination is enhanced by adjunct techniques, such as immunohistochemistry, cytogenetics, and molecular genetics, although morphology provides the ultimate criteria for a specific diagnosis. This article reviews the clinicopathologic features of fibroblastic and myofibroblastic tumors with an emphasis on the unique aspects of these neoplasms in children and adolescents, the use of diagnostic adjuncts, and differential diagnoses.

scholarly journals Cavernous Hemangioma of the Tongue

2015 ◽  
Vol 6 (3) ◽  
pp. 109-111 ◽  
Author(s):  
Jatin P Shah ◽  
Ivana Petrovic ◽  
Ben Roman
Keyword(s):  
Cavernous Hemangioma ◽  
Soft Tissues ◽  
Vascular Malformations ◽  
Surgical Excision ◽  
Vascular Anomalies ◽  
Preoperative Imaging ◽  
Vascular Tumors ◽  
Adult Onset ◽  
Complete Excision ◽  
Intramuscular Hemangioma

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.

Is There an Association Between Hemangioma and Syndromes With Dysmorphic Features?

PEDIATRICS ◽  
1991 ◽  
Vol 88 (6) ◽  
pp. 1257-1267
Author(s):  
A. Jay Burns ◽  
Lawrence C. Kaplan ◽  
John B. Mulliken
Keyword(s):  
Aortic Arch ◽  
Vascular Malformations ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Dysmorphic Features ◽  
Vascular Birthmarks ◽  
Neonatal Tumor ◽  
Nevus Flammeus ◽  
Genitourinary Defects

Most vascular birthmarks can be categorized, based on clinical and cellular criteria, as either (1) a hemangioma, or (2) a malformation, or (3) a macular stain. Macular stains are commonly seen in newborns, and they consist of faint vascular stains of the glabella, eyelids, and nuchal region called "nevus flammeus," "stork bite," "salmon patch," etc. Unfortunately, the term "hemangioma" is frequently applied to all three types of cutaneous vascular lesions. Usually, these disparate vascular anomalies are listed in association with various malformative syndromes and are generically labeled "hemangioma." This study attempts to define accurately the specific vascular anomalies seen in children born with syndromes with dysmorphic features. This review of five standard textbooks of genetics showed that the majority of vascular anomalies reported in syndromic newborns are not hemangiomas. Rather, they are macular stains, and the vast majority of these fade with time. Congenital telangiectasias and other vascular malformations (capillary, lymphatic, venous, arterial, and combinations thereof) also occur in association with dysmorphic syndromes. contrast, hemangioma, the most common neonatal tumor, is seen only incidentally with rare dysmorphic conditions. Specifically, hemangioma was found to occur only in association with midline (sternal, abdominal) clefting, right-sided aortic arch coarctation, and with a constellation of sacral and genitourinary defects.

scholarly journals Cutaneous Vascular Anomalies Associated With Neural Tube Defects: Nomenclature and Pathology Revisited

Neurosurgery ◽  
2011 ◽  
Vol 69 (1) ◽  
pp. 112-118 ◽  
Author(s):  
Todd Maugans ◽  
Rachel M Sheridan ◽  
Denise Adams ◽  
Anita Gupta
Keyword(s):  
International Society ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Vascular Malformations ◽  
Strong Association ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Dermal Sinus ◽  
Cutaneous Lesions ◽  
Glut 1

Abstract BACKGROUND: Lumbosacral cutaneous vascular anomalies associated with neural tube defects are frequently described in the literature as “hemangiomas.” The classification system for pediatric vascular anomalies developed by the International Society for the Study of Vascular Anomalies provides a framework to accurately diagnose these lesions. OBJECTIVE: To apply this classification to vascular cutaneous anomalies overlying myelodysplasias. METHODS: A retrospective analysis of patients with neural tube defects and lumbosacral cutaneous vascular lesions was performed. All eligible patients had detailed histopathologic analysis of skin and spinal cord/placode lesions. Clinical and radiologic features were analyzed. Conventional histology and GLUT-1 immunostaining were performed to differentiate infantile capillary hemangiomas from capillary vascular malformations. RESULTS: Ten cases with cutaneous lesions associated with neural tube defects were reviewed. Five lesions were diagnosed as infantile capillary hemangiomas based upon histology and positive GLUT-1 endothelial reactivity. These lesions had a strong association with dermal sinus tracts. No reoperations were required for residual intraspinal vascular lesions, and overlying cutaneous vascular anomalies involuted with time. The remaining 5 lesions were diagnosed as capillary malformations. These occurred with both open and closed neural tube defects, did not involute, and demonstrated enlargement and darkening due to vascular congestion. CONCLUSION: The International Society for the Study of Vascular Anomalies scheme should be used to describe the cutaneous vascular lesions associated with neural tube defects: infantile capillary hemangiomas and capillary malformations. We advocate that these lesions be described as “vascular anomalies” or “stains” pending accurate diagnosis by clinical, histological, and immunohistochemical evaluations.

scholarly journals Glut 1, S-100, and Nerve Bundle Study in Vascular Anomalies

2021 ◽  
Author(s):  
Ajay K. Khanna ◽  
Akhilesh Kumar ◽  
Soumya Khanna ◽  
Amrita Kar ◽  
Puneet Kumar ◽  
...  
Keyword(s):  
Vascular Malformation ◽  
Vascular Malformations ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Histological Evaluation ◽  
Vascular Tumors ◽  
Nerve Bundle ◽  
Glut 1 ◽  
S 100 ◽  
Hematoxylin And Eosin

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.

scholarly journals Vascular Birthmarks as a Clue for Complex and Syndromic Vascular Anomalies

2021 ◽  
Vol 9 ◽  
Author(s):  
Andrea Diociaiuti ◽  
Guglielmo Paolantonio ◽  
Mario Zama ◽  
Rita Alaggio ◽  
Claudia Carnevale ◽  
...  
Keyword(s):  
Vascular Malformation ◽  
Early Recognition ◽  
Vascular Malformations ◽  
Clinical Signs ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Vascular Tumors ◽  
Capillary Malformation ◽  
Wide Range ◽  
Vascular Birthmarks

Vascular birthmarks are common in neonates (prevalence: 20–30%) and mostly incidental findings sometimes with spontaneous regression (salmon patch and nevus simplex). Capillary malformations are found in about 1% and infantile hemangiomas are found in 4% of mature newborns. Vascular malformations are classified according to their most prominent vessel type. The term “capillary malformation” (port wine stain) includes a wide range of vascular lesions with different characteristics; they may be isolated or part of specific syndromic conditions. Part of the infantile hemangiomas and of the vascular malformations may require treatment for functional or cosmetic reasons, and in rare cases, investigations are also necessary as they represent a clue for the diagnosis of complex vascular malformation or tumors associated with extracutaneous abnormalities. Complex vascular malformations are mostly mosaicism due to early somatic mutations. Genetic advances have led to identify the main pathogenic pathways involved in this disease group. Diffuse capillary malformation with overgrowth, Klippel–Trenaunay syndrome, CLAPO syndrome, CLOVES syndrome, and megalencephaly-capillary malformation belong to the PIK3CA-related overgrowth. Capillary malformation–arteriovenous malformation underlies a fast-flow vascular malformation, sometimes manifesting as Parkes–Weber syndrome. Recognition of these different types of capillary vascular stains is sometimes difficult; however, associated findings may orient the clinicians while genetic testing may confirm the diagnosis. Lymphatic malformation frequently manifests as large masses that compress and/or infiltrate the surrounding tissues, representing a neonatal emergency when airways are involved. Infantile hemangiomas may cause functional and/or permanent esthetical damage, depending on their localization (such as periorbital area, lip, nose); large (more than 5 cm) infantile hemangiomas with a segmental distribution can be associated with obstruction or malformations of the underneath organs with complications: PHACE syndrome, LUMBAR/SACRAL syndrome, and beard infantile hemangioma. In our review, we discuss controversies regarding the international classification and emerging concepts in the field of vascular anomalies. Finally, we discuss potential developments of new, non-invasive diagnostic techniques and repurposing of target therapies from oncology. Complex and/or life-threatening vascular tumors and malformations are extremely rare events and they represent a considerable therapeutic challenge. Early recognition of clinical signs suggestive for a specific disease may improve therapeutic outcomes and avoid severe complications.

scholarly journals Lobular Capillary Haemangioma of the Oral Cavity in a 11-Year-Old Child - A Case Report

2021 ◽  
Vol 10 (28) ◽  
pp. 2132-2134
Author(s):  
Meghana Vasant Phadnis
Keyword(s):  
Young Adults ◽  
Oral Cavity ◽  
Buccal Mucosa ◽  
Soft Tissues ◽  
Vascular Malformations ◽  
Wide Spectrum ◽  
Case Reports ◽  
Pyogenic Granuloma ◽  
Vascular Lesions ◽  
Capillary Haemangioma

Lobular capillary haemangiomas are benign vascular neoplasms that typically affect the skin or mucous membrane. Majority of the cases reporting lobular capillary haemangioma commonly involve the gingiva. There are rare cases presenting involvement of other parts of the oral cavity like buccal mucosa. The management of vascular lesions is more complex in children due to fear of uncontrollable bleeding. The case presented here reports a successful management of lobular capillary haemangioma of the buccal mucosa in an 11 - year - old child. Vascular lesions are one of the rare disorders affecting the overall quality of life of a child. American Academy of Paediatric Dentistry (AAPD) has presented numerous lesions, masses, or tumour-like conditions of hard and soft tissues within the oral and maxillofacial regions of children and young adults; most of these lesions being mucosal conditions. Oral vascular tumours encompass a wide spectrum of anomalies in children. In spite of the fact that a few cases are congenital, most of them arise during childhood.1 The vascular lesions of childhood are classified into two categories: haemangiomas (proliferating or involuting) and vascular malformations. Haemangioma is a term that envelops a heterogeneous gathering of clinical benign vascular lesions that share similar histologic features and presents as a proliferating mass of blood vessels. Haemangiomas are classified on the basis of their histological1 appearance as capillary, mixed or cavernous haemangiomas depending on the size of the vascular spaces or a sclerosing variety that tends to undergo fibrosis. Capillary haemangiomas are of two types; lobular capillary haemangioma and non-lobular capillary haemangioma depending on their histological features. Lobular capillary haemangioma (LCH) is a common reactive angiomatous proliferation of skin and mucous membranes.2,3 LCH (Mills et al. 1980 ) represents an essential, underlying lesion of pyogenic granuloma (PG).4,5 Based on the available literature and case reports, several clinical and histological differences were seen between PG and LCH, yet there is no clear distinction between the two and still remains a topic of debate. The peak prevalence of LCH is in paediatric population and young adults, with a female predilection. Although LCH is one of the most common soft tissue tumours of head and neck accounting for almost 6 % of all cases, there is a striking predilection for lesions on the gingiva. The occurrence of intraoral haemangiomas at sites such as lips, tongue, buccal mucosa, and palate are extremely rare. LCH is a tumour-like growth of the oral cavity or skin that is considered to be non-neoplastic in nature. The purpose of this paper is to report a case of lobular capillary haemangioma in a paediatric patient and describe the successful treatment of this case.

Vascular anomalies of the tympanic membrane

2006 ◽  
Vol 120 (9) ◽  
pp. 796-799
Author(s):  
L O Redaelli de Zinis ◽  
C Galtelli ◽  
M G Barezzani
Keyword(s):  
Tympanic Membrane ◽  
Rare Case ◽  
Vascular Malformations ◽  
Incidental Finding ◽  
Vascular Anomalies ◽  
Vascular Lesions

Benign vascular lesions include various forms whose classification has created some controversies in the literature. The observation of a rare case of vascular bulge of the eardrum in a 57-year-old man prompted us to analyse the essential features of these lesions. This was an incidental finding and the mass was removed by a transcanal approach. The patient is free of disease four years later. Vascular malformations can be differentiated from vascular tumours since they are present at birth, are generally stable, do not involute, and do not necessitate treatment unless symptoms occur.

scholarly journals Emerging importance of molecular pathogenesis of vascular malformations in clinical practice and classifications

2020 ◽  
Vol 25 (4) ◽  
pp. 364-377 ◽  
Author(s):  
Calver Pang ◽  
Chung Sim Lim ◽  
Jocelyn Brookes ◽  
Janice Tsui ◽  
George Hamilton
Keyword(s):  
Growth Factor ◽  
Signaling Pathways ◽  
Vascular Malformations ◽  
Therapeutic Targets ◽  
Classification Systems ◽  
Molecular Pathogenesis ◽  
Vascular Anomalies ◽  
Molecular Pathways ◽  
Molecular Signaling ◽  
Molecular Signaling Pathways

Vascular malformations occur during early vascular development resulting in abnormally formed vessels that can manifest as arterial, venous, capillary or lymphatic lesions, or in combination, and include local tissue overdevelopment. Vascular malformations are largely caused by sporadic somatic gene mutations. This article aims to review and discuss current molecular signaling pathways and therapeutic targets for vascular malformations and to classify vascular malformations according to the molecular pathways involved. A literature review was performed using Embase and Medline. Different MeSH terms were combined for the search strategy, with the aim of encompassing all studies describing the classification, pathogenesis, and treatment of vascular malformations. Major pathways involved in the pathogenesis of vascular malformations are vascular endothelial growth factor (VEGF), Ras/Raf/MEK/ERK, angiopoietin-TIE2, transforming growth factor beta (TGF-β), and PI3K/AKT/mTOR. These pathways are involved in controlling cellular growth, apoptosis, differentiation, and proliferation, and play a central role in endothelial cell signaling and angiogenesis. Many vascular malformations share similar aberrant molecular signaling pathways with cancers and inflammatory disorders. Therefore, selective anticancer agents and immunosuppressants may be beneficial in treating vascular malformations of specific mutations. The current classification systems of vascular malformations, including the International Society of the Study of Vascular Anomalies (ISSVA) classification, are primarily observational and clinical, and are not based on the molecular pathways involved in the pathogenesis of the condition. Several molecular pathways with potential therapeutic targets have been demonstrated to contribute to the development of various vascular anomalies. Classifying vascular malformations based on their molecular pathogenesis may improve treatment by determining the underlying nature of the condition and their potential therapeutic target.

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
Keyword(s):  
Soft Tissues ◽  
Vascular Malformations ◽  
Screening Method ◽  
Clinical Manifestations ◽  
Diagnostic Procedures ◽  
Vascular Tumor ◽  
Vascular Anomalies ◽  
Low Flow ◽  
Vascular Tumors ◽  
Patients Âgés

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%.

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