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Introduction: Capillary hemangiomas are wide spread vascular tumors in ENT. Its presentation is common in nose while they are relatively rare in tongue and ear. Methodology: A retrospective study of 30 cases wherein majority of the cases came with preceding history of trauma and subsequently they were diagnosed with capillary hemangioma in ear, nose, and tongue. Histopathological picture of hemangioma showed clusters of thin-walled capillaries with single layer of endothelium. All patients were treated by wide excision with clear margin and cauterization, followed for 1 year and no recurrences were found. Result: Wide excision with cauterization of base is essential for the prevention of recurrence of traumatic hemangiomas. [ABSTRACT FROM AUTHOR]

H syndrome should be suspected in a case of insulin-dependent diabetes mellitus with hearing loss and pigmentary hypertrichosis. POEMS syndrome shares features of IDDM, hyperpigmentation, hypertrichosis, and skin thickening with H syndrome. [Extracted from the article]

Cavernous malformations affecting the CNS occur in ∼0.16-0.4% of the general population. The majority (85%) of cavernous malformations are in a sporadic form, but the genetic background of sporadic cavernous malformations remains enigmatic. Of the 81 patients, 73 (90.1%) patients were detected carrying somatic missense variants in two genes: MAP3K3 and PIK3CA by whole-exome sequencing. The mutation spectrum correlated with lesion size (P = 0.001), anatomical distribution (P < 0.001), MRI appearance (P = 0.004) and haemorrhage events (P = 0.006). PIK3CA mutation was a significant predictor of overt haemorrhage events (P = 0.003, odds ratio = 11.252, 95% confidence interval = 2.275-55.648). Enrichment of endothelial cell population was associated with a higher fractional abundance of the somatic mutations. Overexpression of the MAP3K3 mutation perturbed angiogenesis of endothelial cell models in vitro and zebrafish embryos in vivo. Distinct transcriptional signatures between different genetic subgroups of sporadic cavernous malformations were identified by single cell RNA sequencing and verified by pathological staining. Significant apoptosis in MAP3K3 mutation carriers and overexpression of GDF15 and SERPINA5 in PIK3CA mutation carriers contributed to their phenotype. We identified activating MAP3K3 and PIK3CA somatic mutations in the majority (90.1%) of sporadic cavernous malformations and PIK3CA mutations could confer a higher risk for overt haemorrhage. Our data provide insights into genomic landscapes, propose a mechanistic explanation and underscore the possibility of a molecular classification for sporadic cavernous malformations. [ABSTRACT FROM AUTHOR]

Background: Sturge-Weber syndrome (SWS) is a sporadic congenital disorder, characterized by unilateral facial nevus flammeus associated with ipsilateral glaucoma, choroidal angioma and leptomeningeal hemangiomas. SWS can comorbid with other disorders in some patients, however, there has been no prior described case of SWS and polydactyly occurring in the same patient.Case Presentation: A 15-year-old girl with diagnosis of SWS presented to our hospital. She had bilateral glaucoma and extensive port-wine stains distributing in bilateral faces, left neck and left upper limb. Meanwhile, the patient was noted to demonstrate the superfluous digit attaching on the left thumb and was diagnosed as polydactyly. Trabeculectomy, with intraoperative application of mitomycin C and postoperative subconjunctival injections of 5-fluorouracil, was successful in controlling the intraocular pressure in both eyes.Conclusions: We report a case with bilateral SWS coexisting with unilateral polydactyly, which, to our knowledge, has not been recognized previously and adds further evidence to the existing literature. In view of the rare concurrence of SWS and polydactyly, the etiology is unclear and further investigation is required to explore the underlying pathogenesis. [ABSTRACT FROM AUTHOR]

Aim: We aimed to evaluate the contribution of early magnetic resonance imaging (MRI) for the presymptomatic diagnosis of Sturge-Weber syndrome (SWS) in infants with a facial port-wine birthmark (PWB).Method: Asymptomatic infants with a facial PWB who performed a first MRI scan before 3 months and a second MRI scan after 9 months were included in this study. Leptomeningeal enhancement on T1-weighted imaging and four indirect signs of leptomeningeal angioma (choroid plexus enlargement, cerebral atrophy, signal inversion of the white matter with T2 hyposignal, and T1 hypersignal) were screened on the first MRI scan and correlated with clinical and/or radiological diagnosis of SWS.Results: Thirteen of 30 included patients had SWS with leptomeningeal angioma. Eleven had a leptomeningeal enhancement on the first MRI scan and 10 had associated indirect signs. The presence of a direct or at least one indirect sign of leptomeningeal angioma on the first MRI scan confirmed the diagnosis of SWS with a sensitivity of 100 per cent (95% confidence interval 75-100%) and a specificity of 94 per cent (71-100%).Interpretation: Early diagnosis of SWS is possible on contrast-enhanced MRI performed in asymptomatic infants with a facial PWB before the age of 3 months. This early detection would help to select patients who may benefit from early neuroprotective intervention.What This Paper Adds: Specific magnetic resonance imaging markers provide early diagnosis of leptomeningeal angioma in Sturge-Weber syndrome (SWS). Presymptomatic diagnosis of SWS should help to select patients for early therapy intervention. [ABSTRACT FROM AUTHOR]

Background: Tufted angioma is a rare benign lesion with vascular proliferation.Aim: To retrospectively analyze the clinicopathological manifestations and immunohistochemical features of tufted angioma.Methods: Clinical and histopathological features of tufted angioma (n = 54) were evaluated and analyzed retrospectively in the Department of Dermatology, Xijing Hospital from 2003 to 2014.Results: Clinically, tufted angioma usually presented as erythematous plaques and papules on the head and neck (n = 11), trunk (n = 21) and extremities (n = 22), mainly in children (n = 48), without gender difference (24 males and 30 females). A total of 45 cases showed solitary lesions and nine cases showed multiple lesions. Common symptoms included pain (n = 11), tenderness (n = 7), itching (n = 1), hypertrichosis (n = 7), hyperhidrosis (n = 6) and Kasabach-Merritt phenomenon (n = 1). Histopathologically, typical tufted angioma (n = 37) showed proliferation of endothelial cells in a so-called cannonball pattern, while in the early (n = 4) and regressed (n = 13) stages the tufted appearance was not prominent. The proliferated endothelial cells were diffusely positive for CD31 and Wilms tumor 1, focally positive for D2-40 and Prox1, and negative for Glut-1.Limitations: Our research was confined to patients of Chinese origin and our sample size was limited.Conclusions: Tufted angioma is a rare vascular neoplasm with diverse clinical manifestations and unique pathological features. It should be recognized as a vascular tumor with lymphatic differentiation. We emphasize the importance of considering tufted angioma in the differential diagnoses of any congenital or acquired vascular tumor. [ABSTRACT FROM AUTHOR]

Résumé: Introduction: Les angiomes sont des anomalies vasculaires qui touchent moins de 1 % de la population dans le monde. Nous disposons de peu de données sur ces affections en Afrique. Le but de notre travail était d'étudier les aspects épidémiologique, clinique, des angiomes dans le service de dermatologie-vénérologie du Centre hospitalier universitaire Yalgado Ouédraogo de Ouagadougou (Burkina Faso) afin de contribuer à une meilleure connaissance de ce groupe de pathologies dans notre région. Patients et méthodes: Il s'agissait d'une étude transversale descriptive, rétrospective et prospective de 1998 à 2014. Résultats: Nous avons colligé 61 patients porteurs d'un angiome avec 67,21 % de nourrissons. Le sex-ratio était de 0,56. Les tumeurs vasculaires (hémangiomes) représentaient 43 cas (70,49 %) et les malformations vasculaires 18 cas (29,51 %). Les lésions apparaissaient entre 0 et 15 jours de vie dans 57,37 % des cas. La taille des lésions était comprise entre 1 et 3 cm dans 49,20 % des cas. Elles siégeaient plus fréquemment sur la tête (49,18 % des cas). Les formes cliniques les plus fréquentes étaient les hémangiomes cutanés (tubéreux) (36 cas), puis suivaient les angiomes plans (8 cas) et les hémangiomes mixtes (7 cas). Les formes complexes étaient représentées par un cas de syndrome de Klippel-Trenaunay. Conclusion: Les anomalies vasculaires superficielles sont rares dans notre contexte de phototype foncé, y compris les hémangiomes infantiles. Les particularités cliniques des angiomes observées dans cette série africaine à Ouagadougou semblent assez superposables aux caractéristiques décrites dans les séries européennes et américaines. Introduction: Angiomas are vascular abnormalities that affect less than 1% of the world's population. Data on these disorders in Africa are limited. The purpose of our work was to study the epidemiological and clinical aspects of angiomas in the Dermatology-Venerology Department of the University Hospital of Yalgado Ouedraogo in Ouagadougou (Burkina Faso) to contribute to improving knowledge of this group of pathologies in our region. Patients and methods: This cross-sectional descriptive, retrospective, and prospective study covers cases from 1998 through 2014. Results: We identified 61 patients with angioma, 67.2% of them younger than 30 months. The sex ratio was 0.56. Vascular tumors (hemangiomas) accounted for 43 cases (70.5%) and vascular malformations 18 (29.5%). Lesions appeared between 0 and 15 days of life in 57.4% of cases. Their size ranged from 1 to 3 cm in 49.2% of cases. They were most frequently located on the head (49.2%). The most frequent clinical forms were cutaneous hemangiomas (tuberous) (36 cases), followed by the nevus flammeus (8 cases), and mixed hemangiomas (7 cases). Only one complex forms was observed: one case of Klippel-Trenaunay syndrome. Conclusion: Superficial vascular abnormalities are rare in our dark skin type context including infantile hemangiomas. The clinical peculiarities of the angiomas observed in this African series in Ouagadougou seem quite similar to the characteristics described in European and American series. [ABSTRACT FROM AUTHOR]

We review the clinical aspects - diagnosis, prognosis, therapy - of neonatal congenital skin cancers, in particular of angiomas - infantile hemangioma, congenital hemangioma, "tufted" angioma, kaposiforme hemangioendothelioma, targetoid hemosiderotic hemangioma -, of mastocytosis, histiocytosis - juvenile xanthogranuloma and Langerhans cell histiocytosis -, fibromatous tumors - myofibroma and dermatofibrosarcoma protuberans -, neuroectodermal tumors - plexiform neurofibroma, benign and malignant proliferations on congenital melanocytic nevi -, and other malignancies - leukemia, rhabdomyosarcoma, neuroblastoma -. [ABSTRACT FROM AUTHOR]

Transcutaneous use of dual diode lasers is a new option in dermatology. The aim of the study was to demonstrate the transcutaneous application of a dual laser in dermatologic outpatients. We used a laser system that combines 980 nm and 1470 nm wavelengths for treatment of vascular lesions. Fifty‐six adult Caucasian patients of Fitzpatrick skin type I‐III, 32 females and 24 males with mean age of (41 ± 3.7) years were included. We treated 497 vascular lesions of skin and lips. Complete clearance was achieved in cherry angiomas, venous lakes, lip hemangioma, and spider nevi in 100% after a single treatment. Patients with couperose and/or facial telangiectasia needed several treatment sessions. Mild to moderate pain during the laser shots was noted in all patients. No adverse events were recorded. The dual 980‐nm/1470‐nm diode laser is a safe and effective tool for common vascular lesions. [ABSTRACT FROM AUTHOR]

Location of cavernous hemangioma in nasal cavity is a very rare condition. Herein we will report an adult male diagnosed with cavernous hemangioma located in inferior concha and causing otitis media with effusion that was treated with the minimally invasive trans-nasal endoscopic technique. Although there are many options in treatment of lesions in nasal cavity, we recommend the trans-nasal endoscopic approach since it is minimally invasive and it is sufficient in both bleeding control and complete removal of the cavernous hemangioma. [ABSTRACT FROM AUTHOR]

Abstract: Background: Oral propranolol is the gold standard to treat infantile hemangiomas. There is better efficacy and a lower risk of sequelae if therapy is started before the end of the growth phase, but most children are referred too late. Herein, we report the first study to investigate the delay and its associated factors when referring infants with infantile hemangiomas that need propranolol therapy. Objectives: The primary objective was to determine the delay in referral (time between age at referral [first phone contact] and the optimal age for referral (fixed at 75 days). The second objective was to determine the impact of weighted factors associated with delayed referral assessed by logistic regression performed on two subgroups (referral ≤75 vs. >75 days). Methods: Monocentric, retrospective, observational study included infants with infantile hemangiomas treated with oral propranolol between August 2014 and May 2017. Results: Eighty‐two children (83% females) were included. Before referral, 81 (99%) children had seen another physician (a paediatrician in 67% of cases). Median age at referral was 99 days [2–478] and 63% phoned after 75 days. Median age at the first visit was 111 days [2–515], and median age when propranolol was started was 128 days [32–541]. After adjustment, in multivariate analyses, location on the lips (OR (CI 95%): 4.21[1.19–14.89]) and superficial hemangioma (OR (CI 95%): 4.19 [1.55–11.34]) emerged as the most significant factors to influence referral before 75 days. Conclusions: This study adds to our understanding regarding delayed referral and has identified targets for future information campaigns. [ABSTRACT FROM AUTHOR]

Objective: To analyze the endovascular, surgical, and clinical outcomes of patients with aggressive vertebral haemangiomas (VHs) treated over a 17 year period. Methods: All medical, radiological, and surgical records were reviewed retrospectively. All patients underwent follow-up evaluation for pain and neurological outcome. Results: A total of 10 patients diagnosed with aggressive VHs causing neurologic compromise or pain underwent 13 operative procedures for tumour resection/stabilization. All but one patient underwent preoperative procedures to minimize intraoperative blood loss. Mean follow-up length was 26.3 months. Of eight patients presenting with neurological deficits, all improved postoperatively. The two patients presenting with pain reported improvement postoperatively. Two patients had new postoperative lower extremity weakness, both of which improved during follow-up. One patient experienced tumour recurrence at 1 year, requiring a second staged surgery. A second patient required a staged surgery for resection of multiple VHs. Seven patients had subtotal resections, two patients had gross total resections, and one patient had an en bloc resection of tumour. Conclusions: Combined preoperative embolization followed by aggressive tumour resection and spinal stabilization are safe and effective procedures for the treatment of aggressive VH causing symptomatic stenosis. [ABSTRACT FROM AUTHOR]

Eruptive pseudoangiomatosis is a rare viral exanthem characterized by acute onset of hemangiomata-like lesions, however, histological findings are distinct from that of true angiomas. This entity has been reported from Europe, North America, Japan, and Korea till date. Here, we report 12 cases of eruptive pseudoangiomatosis from a tertiary care hospital in Punjab. [ABSTRACT FROM AUTHOR]

We report a rare case of intravascular papillary endothelial hyperplasia (IPEH) of the cheek. This neoplasm, known as Masson’s tumor, is an unusual vascular lesion of proliferating endothelial cells. It is usually confined to the lumen of preexisting vessels or vascular malformations. The principal significance of IPEH is its resemblance to a variety of benign and malignant diseases and possible misdiagnosis as such. Achieving a correct diagnosis is essential to avoid subjecting a patient to either unnecessarily aggressive or inadequate therapy. For this reason, awareness of this lesion is very important. [ABSTRACT FROM AUTHOR]

Background Cavernous hemangioma (CH) of the bone is a rare, benign neoplasm found usually in the vertebral body. This tumor rarely develops in the calvaria, with predilection to occur in the frontal and temporoparietal bones. Case Description A 56-year-old man with a right frontal palpable mass was admitted to our hospital. Magnetic resonance imaging (MRI) revealed a large extra-axial tumor in the right frontal bone. Intraoperatively, the bony mass involved the multilayers composed of extracranium-skull-dura-intradura-cortex. Pathologic examination revealed a typical calvarial CH penetrating dura mater. Conclusions This is the second reported case of calvarial CH with complete erosion of the dural plane and extension into subdural space. Although surgical resection is generally safe and easy, the possibility of dural invasion should be kept in mind for safe resection of calvarial CH, especially overlying the eloquent brain area. [ABSTRACT FROM AUTHOR]

Congenital vascular anomalies constitute a large group of lesions in children, and they differ in terms of their localization, outer appearance, as well as histology. Additionally, the nomenclature of this group of lesions has been confusing due to the indiscriminate and interchangeable use of the term haemangioma and vascular malformation in diagnosis and management. Whilst hemangiomas are true endothelial cell neoplasms, vascular malformations are localized congenital defects of vascular morphogenesis. The aim of this article is to overcome obstacles in communication when describing a vascular lesion by adhering to the correct terminology. This leads inevitably to the correct therapeutic guidelines, management and follow-up of these lesions. The therapy guidelines, management and follow-up of hemangiomas and vascular malformations differ and are beyond the scope of this article. [ABSTRACT FROM AUTHOR]

Purpose To evaluate the occurrence rate of lobulated margination of hepatic hemangiomas. Methods The study population included 585 hemangiomas in 261 patients (161 females; mean age: 51.9 years). Two readers independently reviewed all cases. Results Hemangiomas with lobulated margins accounted for 74.7% of all lesions. The incidence of lobulated margins was significantly higher ( P < .001) in medium- and large-sized hemangiomas (77.6% and 99.5%, respectively). The majority of hemangiomas with type-3 enhancement showed lobulated margins (83.5%) ( P < .001). Conclusion Virtually, all hemangiomas > 18 mm show lobulated margins. The majority of hemangiomas with type-3 enhancement show lobulated margins. Lack of lobulated margins in larger lesions lowers the likelihood ratio for being hemangiomas. [ABSTRACT FROM AUTHOR]

Background Oral propranolol is widely prescribed as first-line treatment for infantile haemangiomas ( IHs). Anecdotally, prescribing practice differs widely between centres. Objectives The Propranolol In the Treatment of Complicated Haemangiomas ( PITCH) Taskforce was founded to establish patterns of use of propranolol in IHs. Methods Participating centres entered data on all of their patients who had completed treatment with oral propranolol for IHs, using an online data capture tool. Results The study cohort comprised 1097 children from 39 centres in eight European countries. 76·1% were female and 92·8% had a focal IH, with the remainder showing a segmental, multifocal or indeterminate pattern. The main indications for treatment were periocular location (29·3%), risk of cosmetic disfigurement (21·1%) and ulceration and bleeding (20·6%). In total 69·2% of patients were titrated up to a maintenance regimen, which consisted of 2 mg kg−1 per day (85·8%) in the majority of cases. 91·4% of patients had an excellent or good response to treatment. Rebound growth occurred in 14·1% upon stopping, of whom 53·9% were restarted and treatment response was recaptured in 91·6% of cases. While there was no significant difference in the treatment response, comparing a daily maintenance dose of < 2 mg kg−1 vs. 2 mg kg−1 vs. > 2 mg kg−1, the risk of adverse events was significantly higher: odds ratio ( OR) 1 vs. adjusted OR 0·70, 95% confidence interval ( CI) 0·33-1·50, P = 0·36 vs. OR 2·38, 95% CI 1·04-5·46, P = 0·04, Ptrend < 0·001. Conclusions The PITCH survey summarizes the use of oral propranolol across 39 European centres, in a variety of IH phases, and could be used to inform treatment guidelines and the design of an interventional study. [ABSTRACT FROM AUTHOR]

Multiple synchronous tumors presenting in infancy raise concern for inherited or sporadic cancer predisposition syndromes, which include Beckwith-Wiedemann syndrome, familial adenomatous polyposis syndrome, and Li-Fraumeni syndrome. We report a case of a 7-month-old previously healthy male born following an in vitro fertilization-assisted twin pregnancy who presented with new-onset refractory shock, severe acidosis, and rapid decline over several hours. An autopsy revealed a ruptured liver involved by hepatoblastoma, an adrenal gland involved by neuroblastoma, and multiple cutaneous capillary hemangiomas. Standard genetic testing demonstrated that both twins were Gaucher disease (GD) carriers without evidence of other known cancer predisposition syndromes. This report describes a unique association of multiple synchronous tumors, which underscores the utility and importance of the pediatric autopsy. Moreover, given that the reported child was a GD carrier, the possibility the tumors were the result of a GD-mediated cancer-associated phenotype or an unrecognized sporadic clinical syndrome remains an unanswered, but intriguing, question worthy of further investigation. [ABSTRACT FROM AUTHOR]

Primary racemose hemangioma with bronchial-pulmonary arterial fistula is a very rare abnormality. We herein report an asymptomatic case of primary racemose hemangioma with no significant size change in 5 years. [ABSTRACT FROM AUTHOR]

The article presents a case study of a multifocal cardiac hemangioma in a 41-year-old female who presented with a mass in the right ventricle (RV). The patient underwent surgery, removing the masses with a small size of septal tissue. She had an uncomplicated postoperative result, and no residual masses after surgery.

This series reports 2 pediatric cases of calvarial cavernous hemangioma (cavernoma, cavernous malformation) treated surgically at Children's Hospital Colorado between 2008 and 2010. Both cases presented as painless bony masses which enlarged over time. Both patients underwent surgical resection without complication and have remained recurrence free since surgery. Because so few cases have been reported among pediatric populations, little is known regarding the epidemiology and prognosis of calvarial cavernous hemangiomas in children. These cases represent interesting additions to the small body of literature on these rare tumors. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Verrucous hemangioma (VH) is an uncommon, congenital, vascular malformation that involves dermis and subcutaneous connective tissue of skin. VH lesions are initially present at birth, and therefore, the diagnosis in the elderly may be difficult. Review of literature reveals that VH lesions are commonly located unilaterally on the lower extremities. VH may clinically present as keratotic, papular, nodular, or plaque-like lesions that are reddish-blue in color. VH does not resolve spontaneously and has a tendency to relapse. The diagnosis of VH is generally done on the basis of histopathology. Early diagnosis is important to get a better cosmetic result. VH requires a large, deep excision to avoid recurrence because of frequent extension into subcutaneous fat planes. Intra-oral lesions of VH have rarely been reported in the literature. We present an extremely rare case of VH occurring in the retromolar triangle area of oral cavity. [ABSTRACT FROM AUTHOR]

The aim of the present study was to investigate the clinical benefit and side effects of propranolol treatment in 106 children with infantile hemangiomas (IHs). A retrospective chart review was conducted on all children who attended the clinic between September 16, 2009 and November 11, 2013. Propranolol was administered in a progressive schedule reaching 1.0-1.5 mg/kg/day, divided into three doses. Demographic data, clinical features, imaging, treatment regimens and outcomes were investigated. Any adverse effects following medication were evaluated and managed accordingly. Preliminary analysis of the data showed the inclusion of 106 children (71 female and 35 male) with a mean age and weight at onset of treatment of 5.1 months and 7.3 kg, respectively. All 106 patients responded positively to treatment. Side effects that required intervention and/or close monitoring included diarrhea (n=10), hypotension (n=7), nightmares (n=2), agitation (n=1) and cold extremities (n=1). No long-term adverse effects were observed in any of the patients. In conclusion, propranolol administered orally at 1.0-1.5 mg/kg/day had a rapid therapeutic effect for resolving IHs with few complications. [ABSTRACT FROM AUTHOR]

Hemangiomas are benign dysplasias or vascular tumors consisting of vascular spaces lined with endothelium. Nowadays, radiotherapy for vertebral hemangiomas (VHs) is widely accepted as primary treatment for painful lesions. Nevertheless, the role of surgery is still unclear. The purpose of this study is to propose a novel algorithm of treatment about VHs. This is a case report of an extensive VH and a review of the literature. A case of vertebral fracture during radiotherapy at a total dose of 30 Gy given in 10 fractions (treatment time 2 weeks) using a linear accelerator at 15 MV high-energy photons for extensive VH is reported. Using PubMed database, a review of the literature is done. The authors have no study funding sources. The authors have no conflicting financial interests. In the literature, good results in terms of pain and neurological deficits are reported. No cases of vertebral fractures are described. However, there is no consensus regarding the treatment for VHs. Radiotherapy is widely utilized in VHs determining pain. Surgery for VHs determining neurological deficit is also widely accepted. Perhaps, regarding the width of the lesion, no indications are given. We consider it important to make an evaluation before initiating the treatment for the risk of pathologic vertebral fracture, since in radiotherapy, there is no convention regarding structural changes determined in VHs. We propose a new algorithm of treatment. We recommend radiotherapy only for small lesions in which vertebral stability is not concerned. Kyphoplasty can be proposed for asymptomatic patients in which VHs are small and in patients affected by VHs determining pain without spinal canal invasion in which the VH is small. In patients affected by pain without spinal canal invasion but in which the VH is wide or presented with spinal canal invasion and in patients affected by neurological deficits, we propose surgery. [ABSTRACT FROM AUTHOR]

Introduction. Intracranial cavernomas are rare neurovascular lesions, met frequently in patients with anomalies of the vasculature of the encephalon and medulla. Cavernomas account for 0.02-0.53% of all intracranial lesions and approximately 8-15% of all intracranial vascular malformations. A procentage of about 10-30% of all cases show an association between cavernomas and arteriovenous malformations. Clinically, the lesions become symptomatic when their size becomes larger than 1cm. The symptoms include headache, seizures, focal neurologic deficit and last but not least hemorrhage. Materials and methods. The authors present a study of 99 patients diagnosed and treated for intracranial cavernomas between January 2004 and January 2015 (11 years). The study encompasses 45 male patients and 44 female patients with ages ranging between 11 and 56, all treated at the Bagdasar-Arseni Emergency University Hospital in Bucharest, Romania. A large percentage of the cavernomas were supratentorial 72 cases (72.72%), while only 27 tumors were positioned in the infratentorial compartment of the skull. Regarding the position of the cavernomas, 29 of them (40.27%) were in the frontal lobe, 13 (18.05%) were in the parietal lobe, 20 (27.7%) were in the temporal lobe, while 3 were in the occipital lobe (4.16%). Infratentorial tumors affected the brainstem in 17 cases (62.9%) while 10 cases showed cerebellar implication (37.03%). There were 7 patients in which the authors described multiple cavernomas. The clinical onset was represented by seizures in 59 cases (59.59%), hemorrhage in 20 cases (20.20%) and focal neurologic deficit in 13 cases (13.13%). The symptoms consisted of seizures in 63 cases (63.63%), focal neurologic deficit in 16 cases (16.16%) and hemorrhage in 23 cases (23.23%) while 9 cases (9.9%) were completely asymptomatic. The authors chose to practice a conservative management for the 7 cases with multiple lesions, the 9 asymptomatic cases and 5 cases with deep positioning. In the 5 cases with deep cavernomas the patients were subjected to gamma knife stereotactic radiosurgery but only 2 patients showed response to treatment. Results. In the 99 patients presented by the authors, out of the 76 cases operated, a number of 57 interventions (75%) managed to completeley remove the lesion and perilesional gliosis. A number of 19 intervenfamations only managed to remove the tumor as perilesional gliosis was impossible to remove without lesions to eloquent areas. Conclusions. Intracranial cavernomas are rare lesions, usually incriminated when seizures appear. When they are asymptomatic the best option for the surgeon is to wait and see how the tumor behaves. When seizures appear in the array of symptoms of a given tumor the best prognosis is offered by lesionectomy with the removal of perilesional gliosis. Neuronavigation guided surgery has managed in most cases to facilitate complete removal of such tumors and to avoid postoperative defficit with the improvement of clinical results. Furthermore, neuronavigation removes the necessity of an unpleasant stereotactic frame. When intracerebral hemorrhage occurs, surgery is mandatory and represents a neurosurgical emergency. In multiple tumors, the bleeding cavernoma must be removed. The effectiveness of Gamma Knife Surgery (GKS) is debatable. [ABSTRACT FROM AUTHOR]

The literature is rich in case reports of intraosseous haemangioma, although most of these are actually cases of venous or capillary malformations. To illustrate this confusion in terminology, we present three cases of slow-flow vascular malformations misnamed as intraosseous haemangioma. A retrospective study of children diagnosed with intraosseous haemangioma was conducted. Clinical and radiological data were evaluated. Histopathological examinations and immunohistochemical studies were redone by three independent pathologists to classify the lesions according to the International Society for the Study of Vascular Anomalies (ISSVA) and World Health Organization (WHO) classifications. Three children who had presented with jaw haemangiomas were identified. Computed tomography scan patterns were not specific. All tumours were GLUT-1-negative and D2-40-negative. The lesions were classified as central haemangiomas according to the WHO, and as slow-flow malformations according to the ISSVA. The classification of vascular anomalies is based on clinical, radiological, and histological differences between vascular tumours and malformations. Based on this classification, the evolution of the lesion can be predicted and adequate treatment applied. The binary ISSVA classification is widely accepted and should be applied for all vascular lesions. [ABSTRACT FROM AUTHOR]

A 61-year-old male who originally visited a different hospital, underwent a health checkup in which multiple lung nodules were detected. Multiple well-defined small nodules were observed in both lungs, with lesions reaching the arteries. Metastatic lung cancer, with unknown origin, was suspected. A computed tomography-guided percutaneous lung biopsy was performed; however, a pathological diagnosis could not be established. Then the patient was referred to our hospital for surgical lung biopsy. Macroscopically, the nodule was dark-red in color and solid without a capsule. Microscopically, the nodule was composed of dilated vascular spaces lined by flattened bland cells. They were positive for CD34, but were negative for TTF-1, consistent with lesions of endothelial origin. Microscopic and immunohistochemical findings supported the diagnosis of multiple pulmonary cavernous hemangiomas. After diagnosis, the lesions were left untreated and thereafter showed no signs of deterioration. [ABSTRACT FROM AUTHOR]

Background: Most spinal cavernous haemangiomas occur in the vertebral body and purely extradural cavernous hemangiomas without any vertebral body involvement is rare and account for only 4% of all extradural spinal tumors. Dumbbellshaped spinal cavernous angioma is extremely rare, only 10 cases have been reported in the literature. Case Description: A 77yearold female presented with a oneyear history of lumbago and rightsided L3 dermatomal hypoesthesia. A dumbbell mass at the L2/3 vertebral level was identified on lumbar MRI. The lesion was irregularly shaped and isointense on T1W and hyperintense on T2W and DWI images with homogenous contrast enhancement. A presumptive diagnosis was schwannoma, but other malignant neoplasms were also considered because of its irregular shape, minimally dilated neural foramen and the involvement of the nonenhanced L3 nerve root. The patient underwent surgery with a lateral extracavitary approach. A histopathological examination revealed cavernous hemangioma. Conclusion: Cavernous hemangioma should be included in the differential diagnosis of dumbbellshaped spinal tumors when the intervertebral foramina is not highly dilated and nonenhanced nerve root is identified in the tumor. [ABSTRACT FROM AUTHOR]

Acquired lymphangiectasis (AL) occurs due to the blockage of deep lymphatic vessels. The latter is responsible for dilated dermal lymphatic channels that can induce superficial vesicle formation. We report an 11-year-old girl presented to us with a 10-year history of translucent thick-walled vesicles, which was diagnosed as AL according to the typical clinical lesions. [ABSTRACT FROM AUTHOR]

Objectives: A traumatic splenic rupture is a rare clinical entity with multiple possible underlying etiologies. The aim of this article is to report a case of unusual presentation of an average size splenic hemangioma of an asymptomatic patient with multiple other cystic lesions in kidneys and liver and to summarize the syndromes that include this pathologic entity. Report of case: We present a case of a 33 years old Caucasian male patient with no medical history known who presented at E.R. with acute abdominal pain and intense pallor. Emergency laparotomy was performed. Intraoperatory a massive hemoperitoneum and laceration of the spleen was found; splenectomy was performed. The surgical specimen was histologically analyzed. Results: The histological examination revealed vascular spaces of irregular size delimited by a single layer of blood endothelial cells with nuclei bulging into the lumen, without mitoses, separated by fibrous trabeculae. Diagnosis of splenic hemangioma was confirmed. Imagistic also found multiple cystic lesions in kidneys and liver. Conclusions: The case is notable because the patient was asymptomatic and developed the most severe complication of the splenic hemangioma and also because cystic lesions in other sites were associated. This is the reason why we made a literature review of syndromes which includes splenic hemangioma. [ABSTRACT FROM AUTHOR]

Purpose: Hemangiomas are benign vascular anomalies characterized by benign proliferation of blood vessels. There are no well-defined criteria for the diagnosis and treatment of oral capillary hemangioma (OCH). The objective of this study is to report a safe, effective, and low-cost protocol for diagnosis and treatment of OCH. Materials and methods: Eight patients were diagnosed with OCH, through two physical examination maneuvers-the diascopy and head lowering maneuver with abdominal compression (HLMAC). The treatment performed was sclerotherapy with ethanolamine oleate, weekly, until the disappearance of the lesion. No local anesthetics were administered prior to the sclerosing agent injection. Results: All cases showed a complete remission of the lesions after sclerotherapy. There were no visible scars or compromise esthetics or normal function, and there was no evidence of recurrence. Only one patient had superficial ulceration, which remained asymptomatic and healed without specific treatment. Conclusions: This protocol was effective in the diagnosis of OCH through two physical examination maneuvers with low-cost and easy implementation, the diascopy and the HLMAC. The low concentration associated with a reduced amount of the sclerosing agent was responsible for the excellent results in the cases treated. [ABSTRACT FROM AUTHOR]

Introduction Infantile hemangiomas (IHs) are the most common benign tumors of the soft tissue in infants and children and they often represent a serious challenge for the treating physician. Hemangiomas located in the anogenital region represent only about 1% of all IHs, but raise special concerns as they have the propensity to ulcerate. This condition may appear spontaneously, or could result from therapeutic procedures. Ulceration is extremely painful and takes many weeks of conservative therapy to heal. Material and Methods. The aim of this study is to present the surgical approach of the IHs located in the anogenital area and the outcomes of this treatment option. Results. During a period of 36 months, 11 children (nine girls, two boys) were referred to our plastic surgery department with hemangiomas involving the anogenital, groin and perineum areas. The average follow-up period was of 8 months, during which 82% of cases experienced complications, especially ulceration. All the target hemangiomas were removed through a lenticular excision and the wound closed with a linear suture. Conclusions. Our study has shown that surgical excision of a complicated anogenital hemangioma or of a 'healthy' hemangioma at high risk for ulceration in the anogenital region is an effective treatment, with fast healing and complete resolution of the pathogenic condition. Lenticular excision and linear closure represent a convenient surgical technique that can be performed as early surgery, during the proliferative stage, or at any time later, when the patient needs treatment, in safety conditions and with good results. [ABSTRACT FROM AUTHOR]

The distribution by sex and age of 104 cases of eruptive lobular hemangioma (ELH) observed in the last eight years in children aged 0 to 13 years was analyzed. Females (62) outnumbered males (42) by 1.5 to 1. The average age in this population was 5.5 years, but half of the cases was included in the first 3 years of life and 26% between 10-13 years. The head was the most common site, the lower limbs the rarest. The average diameter of the lesions at the time of the removal was 5 mm (1-12). We did not find a relationship between trauma and ELH, except in a case of satellitosis after removal of an angioma; exceptionally a case of ELH developed under a molluscum contagiosum. [ABSTRACT FROM AUTHOR]

Objective To assess the effect of propranolol in growing infantile hemangioma (IH). Methods 122 children 68 girls and 54 boys, mean age 4.5 months, having IH were treated with oral propranolol. After taking baseline pulse, blood pressure, blood glucose level and echocardiography, propranolol was started at 0.5mg/kg body weight. Patients were monitored hourly for pulse, blood pressure and blood glucose level were noted and if no side effects noted for four hours, treatment was continued at home. Patients were reevaluated on day 10 and the dose was increased to 1.5-2 mg/kg daily in divided doses. Patients were followed up monthly for response and side effects. To avoid hypoglycemia in infants under the age of 3 months, mothers were advised to feed them every 2 hours. Results The earliest effect was change in color seen in all cases. Objective clinical evidence of softening and regression were seen after 2-3 weeks. Drug was given for mean period of 6.5 months. Overall clinical response was seen in 115 (94.3%) patients. It was graded as excellent (>75% improvement) in 92 (75.4%) and good (50-75% improvement) in 23 (18.9%) patients. Adverse effects during treatment were hypoglycemia 4 (3.3%), hypotension 3 (2.5%), cold extremities 2 (1.6%) and constipation 1 (0.8%). Relapse rate was none. Conclusion Propranolol given orally at 1.5-2.0 mg/kg/day has consistent, rapid therapeutic effect in shortening of natural course of IHs with good clinical safety. [ABSTRACT FROM AUTHOR]

BACKGROUND Sclerotherapy is a safe, effective, and easily available treatment modality, its role in cherry hemangioma is still unexplored. OBJECTIVE This study aims at establishing the role and efficacy of sclerotherapy in treating cherry angiomas and its dermatological complications. MATERIALS AND METHODS This prospective study included 20 patients with 100 lesions of cherry hemangiomas of size >0.2 mm. Intralesional injection of 0.1 mL of 3% sodium tetradecyl sulfate was used. Scarring, if any, was evaluated using "The Stony Brook Scar Evaluation Scale." Patients were called for weekly sessions for a maximum of 4 weeks. RESULTS Of 100 lesions treated, 42 lesions responded with a single dose of sclerosant, 44 lesions required a second setting. Remaining lesions were injected for 3 weeks, of which 14 lesions did not remit completely and required a fourth sitting. Depending on response to sclerotherapy, patients were divided into 2 groups (Group A and Group B). On comparing these groups, no statistically significant (χ2 test) difference in the rate of healing was observed. CONCLUSION Sclerotherapy with sodium tetradecyl sulfate 3% is effective in the treatment of cherry hemangiomas. It offers an economical alternative to other available conventional methods. [ABSTRACT FROM AUTHOR]

Purpose The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma. Methods In this clinicopathologic series, clinical features were obtained from patient records. All imaging studies were reviewed. All specimens were reviewed with haematoxylin and eosin, and 10 were subject to a staining protocol including: Movat Pantachrome, periodic acid Schiff, D2-40, CD31, GLUT-1, Ki-67, vascular endothelial growth factor receptor 1 (VEGF-r1) (flt-1), VEGF-r2 (Flk-1), VEGF, anti-smooth muscle actin (SMA), CD20, CD4, CD8 and CD68. Imaging and pathology were reviewed in a systematic fashion. Results Clinically, lesions were more common in middle-aged females presenting with axial proptosis and pain. One-third demonstrated signs of optic nerve dysfunction. Dynamic imaging revealed focal early and diffuse late enhancement. Lesions demonstrated slow growth at 0.2 cm³/year. Histologically, all lesions demonstrated large vascular channels with mature-appearing endothelium and abundant stroma. Three salient features were noted and characterised: thrombosis, nests of perivascular hypercellularity and expanded stromal elements. Acute thrombosis was a feature of each specimen (<10% of channels). Fibrin clots were lined by a layer of CD31+ endothelium. Perivascular hypercellular areas stained uniformly with CD31 and less so with VEGFr2. Additionally, focal areas of Ki67+ and CD68+ cells were found in these regions. Expanded stroma contained CD31+ microcapillary networks and stained diffusely with anti-SMA. Conclusions Cavernous haemangioma demonstrate clinical features and growth characteristics of a benign mass. Dynamic imaging highlights their slow flow vascular nature. Histologically, the hypercellularity and stromal changes identified can be understood within the pathogenic context of thrombosis and recanalisation in an organised lesion. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas (IH) are common vascular tumours. IH have a characteristic natural course. They proliferate rapidly during the early infantile period followed by a period of gradual regression over several years. Most of the uncomplicated IH undergo spontaneous involution, with a small proportion of cases requiring intervention. These are children with IH in life-threatening locations, local complications like haemorrhage, ulceration and necrosis and functional or cosmetic disfigurements. Systemic corticosteroids have been the first line of treatment for many years. Recently, non-selective beta-blockers, such as oral propranalol and topical timolol, have emerged as promising and safer therapies. Other treatment options include interferon and vincristine which are reserved for life-threatening haemangiomas that are unresponsive to conventional therapy. This review mainly focuses on the current trends and evidence-based approach in the management of IH. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to compare the efficacy and safety of propranolol versus corticosteroids for the treatment of periorbital infantile haemangiomas (IHs). Methods A literature review using PubMed, Ovid Medline, EBSCO, Springer, Web of Knowledge, Cochrane Library, CNKI and associated references before 2 March 2013 was conducted. The main outcomes were distribution of locations, response rate, rebound growth rate, spherical and cylinder power before and after treatment, amblyopia rate and adverse events. Results Thirty-one studies including 425 patients met the inclusion criteria. A total of 70.6% of patients were female, 89.6% of the periorbital IHs were located in the upper or lower eyelid area. The most common administration routes involved oral propranolol and intralesional injection of corticosteroids. The mean response rate was 94.0% for propranolol and 82.3% for corticosteroid ( P = 0.001). The rebound growth rate was 13.9% for propranolol and 12.0% for steroids ( P = 0.71). Astigmatism was reduced in both propranolol and steroid studies ( P < 0.0001, P < 0.0001), but a significant reduction in spherical power was only demonstrated in propranolol studies ( P = 0.005). A total of 31.1% of patients treated with corticosteroids developed post-operative amblyopia compared with 16.7% of patients treated with propranolol ( P = 0.04). Oral propranolol seemed to induce more temporary adverse events than intralesional corticosteroids administration (24.0% vs. 9.6%, P = 0.006). Conclusion Propranolol may represent an effective therapy for periorbital IHs compared with the use of corticosteroids; however, further randomised control studies are needed to compare adverse events. [ABSTRACT FROM AUTHOR]

BACKGROUND Despite several recent reports on the efficacy of long-pulse pulsed dye laser (LP-PDL) in treating infantile hemangiomas, controversy remains. OBJECTIVES To determine the beneficial effects of early therapeutic intervention with LP-PDL in superficial and mixed hemangiomas. METHODS AND MATERIALS Clinical data from 40 children with 47 hemangiomas in preproliferative or proliferative phase treated with LP-PDL in a Korean tertiary hospital over 5 years were analyzed retrospectively. Treatments were repeated at 1- to 4-week intervals until growth stopped. RESULTS Of the 47 hemangiomas, 32 were superficial, and 15 were mixed at presentation. Age was 9.6 ± 5.9 weeks at initiation of treatment and 18.0 ± 8.6 weeks at completion, and a positive linear correlation existed between the two. There were a mean of 4.6 ± 2.6 treatments per hemangioma, which was lower in superficial than in mixed hemangiomas. Improvement in color was documented in 84.4% of superficial and 86.7% of mixed hemangiomas, and 75.0% of superficial and 66.7% of mixed shrank. Hyperpigmentation occurred in two superficial hemangiomas. CONCLUSION Early intervention in hemangiomas using LP-PDL successfully prevents further growth and accelerates a transition to plateau or involution phase with minimal adverse events, achieving good cosmetic outcomes. [ABSTRACT FROM AUTHOR]

Background Internet-mediated communication in health care is becoming increasingly important. The purpose of this study was to analyze internet-mediated physician-patient interaction in an angioma center. Methods Patient-related e-mails received between January 2002 and June 2009 were retrospectively analyzed regarding the diagnosis of hemangiomas or vascular malformations. Additionally, the visitors' statistics of the corresponding website ''www.angiome.de' was evaluated. Results Five hundred forty-eight e-mails matched the criteria of the study. From 2002 to 2008, the number of messages registered annually increased by a factor of 20 and the average number of e-mails per patient tripled. The number of new patients contacting the center via e-mail increased from 12 to 72 per year. The website ''www.angiome.de' was visited 8490 times in 2008 and 13,291 times in 2009. Conclusion The presence of the internet is relevant to get in touch with new patients and to provide information for nonprofessionals and experts especially in diseases with low incidence. © 2013 Wiley Periodicals, Inc. Head Neck 36: 187-190, 2014 [ABSTRACT FROM AUTHOR]

Infantile hemangioma (IH), a representative vascular liver tumor, usually occurs in infancy or early childhood but rarely in adults. In this study, we describe a case of IH in a 47-year-old female and we also review the literature. A plain computed tomography (CT) image revealed five hypoattenuating masses in the liver. A dynamic study revealed the masses appeared to be well-enhanced in the arterial phase, and were considered to be high-flow hemangiomas. The tumors appeared as hypointense tumors on the T1-weighted images and as hyperintensities on fat-suppression T2-weighted images. Following the administration of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA), tumors appeared to be well-enhanced in the arterial phase. In the portal phase, tumors demonstrated isointensity compared with the surrounding liver parenchyma, and hypointensity in the equilibrium and hepatobiliary phases. The apparent diffusion coefficient (ADC) values ranged from 2.0 to 2.4x10-3 mm2/sec. Microscopically, the tumors were composed of numerous capillary-like small vessels lined with plump endothelial cells, arranged in a single layer without mitoses, and small bile ducts were trapped and scattered within the tumor. These findings were considered to be characteristic of IH. To the best of our knowledge, this case is the third report on IH in adults. [ABSTRACT FROM AUTHOR]

Aims The pathogenesis of infantile haemangiomas (IHs) is still far from clear despite the fact that they are common vascular tumours distinctive for their perinatal presentation, rapid growth during the first year of life and subsequent slow involution. Aims To determine the role of M2-polarised macrophages in IHs. Methods M2-polarised macrophages were initially identified in 20 specimens of IHs by both immunochemistry and immunofluorescence for CD68 and CD163. The immunopositive M2-polarised macrophages in different phases of IHs were quantified, and further analysed for their correlations with the expression levels of Ki67, vascular endothelial growth factor (VEGF) and macrophage colony-stimulating factor (M-CSF). Results The infiltrating macrophages in proliferative IHs were predominantly CD68/CD163, thus of the M2-polarised phenotype, whereas the density of these cells was significantly decreased in the involuting IHs. The high density of M2-polarised macrophages in proliferative IHs was closely correlated with overexpression of M-CSF, one of the cytokines considered to induce macrophages to polarise towards an M2 phenotype. The infiltrating M2-polarised macrophages probably contributed to the proliferation and angiogenesis of haemangioma endothelial cells, as evidenced by their close correlations with the immunoreactivities of Ki67 and VEGF. Conclusions Results indicate that the infiltrating M2-polarised macrophages may contribute to the progression of IHs by promoting the angiogenic process. [ABSTRACT FROM AUTHOR]

Pyogenic granuloma is a benign vascular tumor of unknown etiology, though multiple factors play a role in its onset, e.g., trauma, chronic irritation, drugs etc., It is commonly seen in children and adolescents. Giant pyogenic granuloma is its atypical variant. We are presenting two cases of giant pyogenic granuloma, one, in a 28-year-old adult, presenting as a giant fluffy swelling of scalp and the other in a 11-year-old child, presenting as a giant ulcerated globular swelling of the scalp. [ABSTRACT FROM AUTHOR]

Hemangioma is the most common benign oral soft tissue tumor in children. In adults, the occurrence of hemangioma with its primary location on gingival tissues is very rare. Two main forms of hemangiomas are recognized: capillary and cavernous. Surgical treatment of this lesion may produce a severe bleeding, for this reason; it may be managed by specialized practitioner. This article reports a case of hemangioma localized in the mandibular gingiva in a 19-year-old man, the adopted treatment was excision of the tumor with a narrow margin of normal mucosa. The histopathological examination shows that it consisted of a mixed capillary-cavernous hemangioma. The pathogenesis, differential diagnosis and the treatment of hemangioma of the oral cavity are discussed. According to our research, this is the first described mixed capillarycavernous gingival hemangioma in the literature. [ABSTRACT FROM AUTHOR]

Vascular anomalies are a common finding in children. Although most of these lesions are benign, they can be a severe cosmetic problem and cause structural and functional damage to nearby tissues. As a result, physicians are tasked with developing effective treatment options with superior safety profiles. Vascular anomalies may be divided into tumors and malformations. Vascular tumors, such as infantile hemangiomas, typically appear a few months after birth, whereas the majority of vascular malformations, such as port-wine stains, are present at birth. Although these lesions vary in appearance, etiology, and disease course, many are treated in a similar fashion. In this review, we focus on treatment modalities for some of the more-prevalent childhood vascular lesions, including port-wine stains, primary telangiectasias, infantile hemangiomas, pyogenic granulomas, and angiomas. [ABSTRACT FROM AUTHOR]