Congenital Vascular Tumors.

Vascular tumors are benign neoplasms, which result from proliferating endothelial cells. These lesions present during infancy or childhood, may affect any location, and exhibit postnatal growth. Local complications include bleeding, tissue destruction, and pain whereas systemic sequelae include thrombocytopenia, congestive heart failure, and death. Vascular tumors should be differentiated from vascular malformations, which present at birth, have a quiescent endothelium, and grow in proportion to the child. Together, vascular tumors and malformations comprise the field of vascular anomalies.

Vascular anomalies: hemangiomas and beyond--part 1, Fast-flow lesions.

OBJECTIVE: The purpose of this study was to review the medical literature and the current classification of vascular anomalies to clarify common misconceptions and provide guidance for imaging and treatment. In this first article of a two-part series, we focus on the fast-flow vascular anomalies. CONCLUSION: Nonuniformity of terminology across the medical literature hampers understanding of the vascular anomalies. A familiarity with the classification and biology on which this terminology is based is essential for accurate and precise diagnosis.

Conditions masquerading as infantile haemangioma: Part 1.

Infantile haemangiomas are among the most common growths during infancy. Their rapid growth during infancy and vascularity can easily cause confusion with other, less common growths. This article focuses on the myriad of diagnostic mimics of haemangiomas, including other vascular anomalies, benign growths, and malignancies.

Verrucous lymphovascular malformation versus verrucous hemangioma: controversial nomenclature.

The International Society for the Study of Vascular Anomalies (ISSVA) divides congenital vascular anomalies into malformations and tumors and subclassified hemangiomas under tumors. However, evidence shows this accepted classification has not been widely employed. Particularly troublesome is the use of the term hemangioma, commonly used to describe a variety of vascular lesions (both malformations and tumors). The term verrucous hemangioma has been used to describe a congenital vascular anomaly with a progressive verrucous epidermal surface persisting throughout life unless surgically excised. Recent evidence suggests that some of these lesions may share histologic features of both hemangiomas and malformations, thereby causing nosologic confusion. We report a 15-year-old adolescent girl with such a lesion and review the literature and controversy of verrucous hemangiomas. In our case, the most appropriate diagnosis is verrucous lymphovascular malformation. Further testing of similar lesions will be necessary to fully understand the nature and classification of these lesions.

Vascular update: morphogenesis, tumors, malformations, and molecular dimensions.

This vascular review is organized under the following headings: vasculogenesis and angiogenesis; vascular endothelial growth factors, their receptors, TIE receptors, and angiopoietins; other factors in blood vessel formation; parallel patterning in blood vessels and nerves; physiological and pathological neovascularization; the role of VEGF receptors in metastasis; anti-angiogenic therapy for tumors; association of blood vessels with fat; vascular malformations and vascular tumors; infantile hemangiomas; congenital hemangiomas; lymphatic malformations; molecular characteristics of some disorders with vascular malformations; Kasabach-Merritt phenomenon; Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and Parkes Weber syndrome; diagnostic and laboratory studies; and future perspectives.

[Classification of vascular anomalies (tumours and malformations). Clinical characteristics and natural history]. / Clasificación de las anomalías vasculares (tumores y malformaciones). Características clínicas e historia natural.

Vascular anomalies are divided into tumours and malformations. Haemangiomas are the most frequent amongst the former. Not normally present at birth, except in a premonitory form, they grow for 10-12 months due to hyperplasia, to subsequently undergo a progressive involution for a period that might last from ten to twelve years. They have an incidence of up to 12% in newborns; they are more common amongst girls; and are divided into superficial, deep and compound. Congenital haemangiomas and those that do not undergo involution are considered to be rare entities. Vascular malformations, with a lower incidence than haemangiomas, are always present at birth, they grow by hypertrophy and never undergo involution. According to the classification of the ISSVA, vascular malformations are divided - depending on the vessel affected - into capillary or venular (port-wine stain), venous, lymphatic, arteriovenous and combined or complex. Each of these has certain defining clinical and haemodynamic peculiarities. Within the final group are included some with a low flow, such as the Klippel-Trenaunay syndrome (venous and lymphatic venular vascular malformation associated with the muscular-skeletal hypertrophy of an extremity), and others with a high flow, such as the Parkes-Weber syndrome.

[Classification of vascular abnormalities and neoplasms]. / Zur Klassifikation vaskulärer Fehl- und Neubildungen.

Vascular malformations and neoplasms are very common skin disorders, found in up to 5% of newborns. However, a clear distinction has to be made between proliferating vascular lesions and permanent malformations. An exact classification is also extremely useful, since many new specific diagnostic and therapeutic measures have been developed in recent years. True proliferating tumors are, for example, childhood hemangiomas, glomus tumors, granuloma pyogenicum, tufted angiomas, senile angiomas, and malignant vascular lesions. Vascular malformations can affect capillaries, veins or arteries, as well as lymphatic vessels. Arteriovenous shunts and combined malformations may also exist (Hamburg classification). Nevi flammei, nevi anaemici, hematolymphangiomas, angiokeratotic nevi, circumscribed venous-arterious malformations, and the blue-rubber-blebnevus-syndrome may either be infiltrating or circumscribed and are characterised by a persistence of the primitive vessel network. In contrast, other malformations involve various vascular trunks, showing vessel dilation or obstruction, often combined with changes in bone or soft tissue. Significant large vessel malformations are the Bockenheimer syndrome, the Klippel-Trenaunay syndrome and the Parkes-Weber syndrome. Combinations involving both large trunks and extravascular space such as the Klippel-Trenaunay syndrome also occur.

Congenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis.

Infantile myofibromatosis occurs in solitary, multiple, and generalized forms, with similar histology but different clinicopathologic and prognostic implications. We report the findings in two male infants with fatal congenital generalized myofibromatosis (CGMF) who presented with multiple dermal and subcutaneous nodules at birth. Imaging studies revealed bony and visceral lesions, which progressed despite chemotherapy. One infant had severe hypercalcemia associated with extensive lytic bone lesions. Both infants died in respiratory failure and had a combination of pulmonary CGMF and diffuse alveolar damage. Involvement of skin, soft tissue, bone, heart, lungs, liver, gastrointestinal tract, and endocrine organs was confirmed at autopsy in each case. A consistent histologic pattern of interlacing fascicles of myofibroblasts with abundant eosinophilic cytoplasm was noted, with variable necrosis and calcifications in some sites. The myofibroblasts displayed vimentin and smooth muscle actin immunoreactivity. The lungs in each case had the presumably early lesions of CGMF with an angiocentric and perivascular growth of myofibroblasts. A similar vascular pattern was present in all affected organs. These two cases demonstrate the extraordinary presentation of CGMF, which suggests its multifocal origin from vascular subintimal mesenchymal or smooth muscle cells whose phenotype is that of myofibroblasts.

Benign and malignant vascular tumors of the upper extremity.

Vascular tumors of the upper extremity comprise a wide and varied group of pathologies. A variety of benign and malignant tumors exist. Proper diagnosis is imperative for the proper management of these lesions. On occasion, diagnosis can be made by a careful history and physical examination. However, the differentiation of benign from malignant tumors often requires permanent histologic section analysis by an experienced tumor specialist. The malignant vascular lesions, although rare, are aggressive in their behavior and require prompt diagnosis and treatment for patient survival.

Congenital soft tissue dysplasia: a new malformation entity and concept.

We report 185 children with clinical manifestations of various conditions classically described as giant hamartoma, angiodysplasia, congenital hypertrophy, congenital trophoedema, localised gigantism (e.g. macrodactyly), etc. It is proposed to group all these conditions into a single entity: congenital soft-tissue dysplasia (CSTD). According to recent advances in fundamental embryology and cell biology, CSTD appears to be a consequence of embryonal or fetal cell biosynthetic dysregulation. The concept of the CSTD entity leads to a common protocol for clinical investigation and a common therapeutic plan, with special reference to the stability and the benign nature of the condition. Treatment should be confined to improving function rather than attempting to correct cosmetic deficits.

Congenital neck masses.

Neck masses are frequently encountered in children. Although they are most often due to past infections, they may be of congenital origin. A neck mass in an adult may also be benign and of congenital origin. Common congenital neck masses include thyroglossal duct cysts, branchial anomalies, cystic hygromas and hemangiomas. Cysts, sinuses and fistulas may arise from the branchial apparatus.