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

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%.

Extensively Myxoid and Hyalinized Sinonasal Capillary Hemangiomas: A Clinicopathologic Study of 16 Cases of a Distinctive and Potentially Confusing Hemangioma Variant.

Capillary hemangiomas, the most common vascular tumors of the sinonasal region, are benign endothelial neoplasms, typically growing in an easily recognized lobular pattern. Some sinonasal capillary hemangiomas may show atypical features, such as high cellularity or mitotic activity, and represent more challenging diagnoses. Over the past several years we have seen in consultation a number of examples of sinonasal capillary hemangiomas displaying very striking stromal myxoid change and hyalinization, features that have received scant attention in the past. Available slides from 16 sinonasal capillary hemangiomas previously coded as showing such changes were retrieved from our archives. Submitting diagnoses included "query angiofibroma, rule out malignancy" (N=4), "vascular polyp, rule out malignancy" (N=3), "query malignant vascular tumor" (N=4), "sinonasal hemangiopericytoma" (N=1), and "benign vascular tumor" (N=1). Available radiographic studies often showed worrisome features. Grossly, the tumors ranged from 1.1 to 6.0 cm and appeared as ulcerated, vascular-appearing polyps. Microscopically, the tumors showed striking stromal myxoid change and/or hyalinization, which largely obscured the underlying lobular capillary arrangement. Within this myxohyaline matrix, a florid capillary proliferation was present, frequently with nonatypical mitotic activity. In some instances a branching, "hemangiopericytoma-like" vascular pattern was present in areas. The overall cellularity was low to moderate, and endothelial atypia or hyperchromatism was absent. Ulceration and thrombosis were frequently present. Immunostains to CD31, CD34, and SMA highlighted areas of lobular growth pattern inapparent on the routinely stained slides. Four tested cases were negative for androgen receptors and ß-catenin. Follow-up from 12 patients revealed no local recurrences or metastases. Awareness of that sinonasal capillary hemangioma may show these unusual stromal changes, and the use of ancillary immunohistochemistry to highlight its lobular growth pattern should allow its confident distinction from more aggressive endothelial tumors (eg, angiosarcoma) and from nonendothelial tumors, including nasopharyngeal angiofibroma, solitary fibrous tumor, and sinonasal hemangiopericytoma-like tumor.

Vascular tumors of the iris in 45 patients: the 2009 Helen Keller Lecture.

OBJECTIVE: To report on a series of vascular tumors of the iris. DESIGN: Noncomparative case series. A retrospective medical record review of all patients with an iris vascular tumor was performed to identify the clinical features and develop a simple classification of these lesions. Included were demographics, clinical features, systemic associations, complications, management, and histopathology. RESULTS: There were 54 eyes in 45 patients with an iris vascular tumor. These were categorized as racemose hemangioma (41 eyes: 29 simple and 12 complex), cavernous hemangioma (3 eyes: 2 localized and 1 systemic), capillary hemangioma (1 eye, localized), varix (3 eyes, localized), and microhemangiomatosis (6 eyes, localized). The hemangiomas occurred in adults at a median age of 55 years, whereas capillary hemangioma occurred in infancy and cavernous hemangioma with systemic involvement occurred in a child. Of the 41 eyes with iris racemose hemangioma, none showed systemic involvement. Of all 54 eyes, transient hyphema was the main complication, found at some point in 30% or more of each affected eye except for iris capillary and racemose hemangioma. Surgical resection was necessary in 1 cavernous hemangioma and 1 varix. The remainder were managed with observation. CONCLUSIONS: There are now well-documented examples of iris racemose hemangioma, cavernous hemangioma, capillary hemangioma, varix, and microhemangiomatosis. Transient hyphema is the main complication. Observation is usually advised. Most are solitary lesions confined to the iris and some (cavernous hemangioma and microhemangiomatosis) can have important systemic associations.

[Vascular tumors of the orbit]. / Tumeurs vasculaires de l'orbite.

Vascular tumors of the orbit include capillary hemangioma, cavernous hemangioma, hemolymphangioma, hemangiopericytoma and a few rare tumors. Capillary hemangioma and hemolymphangioma, occurring mainly in children, are covered in the chapter devoted to childhood tumors. In this chapter, cavernous hemangioma and hemangiopericytoma are discussed as well as rare vascular tumors. Although orbital varix is not a tumor, it is also considered because of the diagnostic problems and the close correlation of orbital varix with a true tumor: hemolymphangioma.

[Superficial spreading capillary hemangioma: case report]. / Hemangioma capilar de extensión superficial: descripción de un caso.

Superficial spreading capillary hemangioma has been described for the first and sole time by Mihara and collaborators in 1986, as a proliferation of capillaries in the dermal papillae, separated by thin elongated rete ridges. Clinically it appeared as an asymptomatic, isolated, small and long-standing red plaque of slow growth located in the plantar surface of a 57-year-old Japanese woman. We report a 23-year-old Caucasian woman with an asymptomatic, long-standing, brownish plantar lesion of stable size. In the pathological study we observed a capillary proliferation in tangles with slack stroma located in the papillary dermis. We report, therefore, the second case of superficial spreading capillary hemangioma published in the literature.

Vascular tumors of infancy and childhood: beyond capillary hemangioma.

Vascular tumors of infancy and childhood represent a number of clinicopathologically distinct entities for which precise histopathological diagnosis is often essential in determining effective therapeutic approach. Unfortunately, pathologists and clinicians alike have traditionally tended to lump these tumors, in addition to small vessel vascular malformations, under overly generic terms like capillary hemangioma that do little, if anything, to guide proper clinical management. In the last decade this nosologic oversimplification has begun to wane as important new diagnostic tools and better understanding of etiology have evolved, facilitated by international recognition of the need for a multidisciplinary approach in dealing with these perplexing and often clinically devastating lesions. This article provides a brief historical perspective on this progress, and then focuses on the current clinical, histological, and immunophenotypical features that distinguish the major types of vascular tumors of infancy and childhood, also reviewing new evidence regarding their mechanisms of pathogenesis.

[Vascular tumours and malformations, classification, pathology and imaging]. / Tumeurs et malformations vasculaires, classification anatomopathologique et imagerie.

The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects in vascular morphogenesis. These two types of lesions have different clinical behaviour and require different diagnostic and therapeutic strategies. The most frequent vascular tumour is infantile hemangioma. Its clinical aspects and evolution are well-known. New data have been recently obtained concerning the phenotype of tumour cells and its histogenesis. Of the numerous new vascular tumours, which have been recently described, only the congenital hemangiomas, the vascular tumours associated with the Maffucci syndrome and the tumours that may be complicated by a profound thrombocytopenia (Kasabach and Merritt phenomenon) will be considered. Vascular malformations can be classified according to the vessel(s) types they are composed of. A classification table is presented, separating the malformations of vascular trunks from tissular malformations which are more intimately embedded in the surrounding tissues. The different syndromes associated with vascular anomalies take also place in this table. The clinical, imaging and histological aspects of the most frequent malformations (capillary, venous, lymphatic and arteriovenous) are presented. This classification intend to clarify the nosology and terminology of the complex field of vascular tumours and malformation and to offer a common language to the different physicians and specialists contributing, preferably with a interdisciplinary approach, to the diagnosis and treatment of these difficult lesions.

[Vascular tumours and malformation of the orbit]. / Les lésions vasculaires de l'orbite.

Vascular diseases are an important part of orbital pathology. We describe vascular tumours of the orbit and vascular diseases with repercussion on the orbit, from intra or extra orbital origin. The classification of these abnormalities is difficult and several terms are used to describe the same histological entity. The objective of this work is, using the current classification, to illustrate the different imaging aspects of the most frequent vascular diseases of the orbit.

Laser treatment of hemangiomas of the larynx and trachea.

BACKGROUND AND OBJECTIVE: Hemangiomas have a typical clinical course and may lead to life-threatening obstruction if the central respiratory tract is involved. STUDY DESIGN/PATIENTS AND METHODS: This was observed in 32 children over a period of 20 years. The radiation parameters and application procedure of Neodymium:Yttrium-Aluminum-Garnet-laser (Nd:YAG-laser) therapy were adjusted for the degree of obstruction and the type of disease. RESULTS: The success rate was 93.8%, of which a maximum of one application was sufficient in 24 children (75.0%). Subglottic cicatricial stenosis was considered a laser-related complication. CONCLUSION: Nd:YAG-laser treatment is a safe and effective therapeutic measure for eliminating respiratory tract obstruction caused by hemangiomas.

A study of small superficial capillary hemangiomas on the lips in children.

PURPOSE: This study describes 77 small vascular lesions on the lips of 74 children and adolescents. RESULTS: Of the 77 lesions, 70% were on the lower lip and 30% on the upper. The diameter range was 0.5-5.0 mm (mean 1.4 mm). On both lips the lesions were in the vermilion zone, close to the border with the skin. The surface of the lesions was smooth and slightly raised, and varied in color from red (49%) through bluish red (45%) to purple (6%). The borders were well defined in most cases. The histology of the lesions was consistent with capillary hemangioma. Small superficial capillary hemangiomas (SSCH) in this study behaved differently from most hemangiomas and vascular malformations in that they did not increase in size, and were persistent in the age group in which most hemangiomas are reported to involute or disappear. The gender distribution of SSCH also differed from the female predominance in hemangioma. SSCH is a solitary benign lesion on the lips of children and adolescents, which has not been separately classified before. CONCLUSIONS: Based on the differences from hemangioma in gender distribution, size, border definition, and growth characteristics, it is suggested that SSCH be classified as a variant or a subgroup of hemangioma.

Hemangiomatose neonatal benigna: relato de caso / Benign neonatal hemangiomatosis: a case report

Os hemangiomas säo lesöes da pele, relativamente comuns no período neonatal. O acometimento de vários segmentos e órgäos do corpo é chamado de hemangioma difuso ou hemengiomatose, condiçäo bastante rara, que pode ser caracterizada sob duas formas: circunscrita (benigna) e difusa. O objetivo deste relato é a descriçäo de um caso de hemangiomatose neonatal benigna, de início mais tardio (dois meses de idade) e com proliferaçäo bastante rápida, além de chamar a atençäo para a exclusäo da forma difusa, por sua alta morbimortalidade. Os autores sugerem um roteiro propedêutico para facilitar a diferenciaçäo precoce das duas formas de hemangiomatoses