The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
John B Mulliken - One of the best experts on this subject based on the ideXlab platform.
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lumbar association between cutaneous infantile Hemangiomas of the lower body and regional congenital anomalies
The Journal of Pediatrics, 2010Co-Authors: Ionela Iacobas, Ilona J. Frieden, Anthony J Mancini, John B Mulliken, Patricia E Burrows, Marilyn G Liang, Daniela Kramer, Amy S Paller, Robert A Silverman, Annette WagnerAbstract:Objective To define the clinical spectrum of regional congenital anomalies associated with large cutaneous Hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of Hemangioma location, and provide imaging guidelines for evaluation. Study design We conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases. Results Hemangiomas in our series tended to be “segmental” and often “minimal growth” in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb Hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous Hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies. Conclusions We propose the acronym “LUMBAR” to describe the association of Lower body Hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.
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endothelial progenitor cells in infantile Hemangioma
Blood, 2004Co-Authors: Alan F Flint, John B Mulliken, Joyce BischoffAbstract:Infantile Hemangioma is an endothelial tumor that grows rapidly after birth but slowly regresses during early childhood. Initial proliferation of Hemangioma is characterized by clonal expansion of endothelial cells (ECs) and neovascularization. Here, we demonstrated mRNA encoding CD133-2, an important marker for endothelial progenitor cells (EPCs), predominantly in proliferating but not involuting or involuted Hemangioma. Progenitor cells coexpressing CD133 and CD34 were detected by flow cytometry in 11 of 12 proliferating Hemangioma specimens from children 3 to 24 months of age. Furthermore, in 4 proliferating Hemangiomas, we showed that 0.14% to 1.6% of CD45 nucleated cells were EPCs that coexpressed CD133 and the EC marker KDR. This finding is consistent with the presence of KDR immature ECs in proliferating Hemangioma. Our results suggest that EPCs contribute to the early growth of Hemangioma. To our knowledge, this is the first study to show direct evidence of EPCs in a human vascular tumor. (Blood. 2004;103:1373-1375)
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increased tie2 expression enhanced response to angiopoietin 1 and dysregulated angiopoietin 2 expression in Hemangioma derived endothelial cells
American Journal of Pathology, 2001Co-Authors: Ying Yu, Joyce Varughese, Lawrence F Brown, John B Mulliken, Joyce BischoffAbstract:Infantile Hemangiomas are endothelial tumors that grow rapidly in the first year of life and regress slowly during early childhood. Although Hemangiomas are well-known vascular lesions, little is known about the mechanisms that cause the excessive endothelial cell proliferation in these most common tumors of infancy. To investigate the molecular basis of Hemangioma, we isolated endothelial cells from several proliferative-phase lesions and showed that these cells are clonal and exhibit abnormal properties in vitro (E. Boye, Y. Yu, G. Paranya, J. B. Mulliken, B. R. Olsen, J. Bischoff: Clonality and altered behavior of endothelial cells from Hemangiomas. J Clin Invest 2001, 107:745–752). Here, we analyzed mRNA expression patterns of genes required for angiogenesis, including members of the vascular endothelial growth factor (VEGF)/VEGF receptor family and the angiopoietin/Tie family, in Hemangioma-derived and normal endothelial cells. KDR, Flt-1, Tie1, Tie2, and angiopoietin-2 (Ang2) were strongly expressed in cultured Hemangioma-derived endothelial cells and in Hemangioma tissue. In contrast, there was little expression of angiopoietin-1 (Ang1) or VEGF. We found Tie2 mRNA and protein up-regulated with a concomitant increase in cellular responsiveness to Ang1 in most Hemangioma-derived endothelial cells. Ang2 mRNA was down-regulated in response to serum in Hemangioma-derived endothelial cells, but not in normal endothelial cells, suggesting altered regulation. These findings implicate Tie2 and its ligands Ang1 and Ang2 in the pathogenesis of Hemangioma.
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soft tissue vascular anomalies utility of us for diagnosis
Radiology, 2000Co-Authors: Harriet J Paltiel, Patricia E Burrows, Harry P W Kozakewich, David Zurakowski, John B MullikenAbstract:PURPOSE: To determine the ultrasonographic (US) features that distinguish soft-tissue Hemangioma from vascular malformation and one type of malformation from another. MATERIALS AND METHODS: Eighty-seven vascular anomalies were evaluated by means of US. Lesions were assessed for the presence of solid tissue and abnormal arteries, veins, or cysts. Vessel density, peak flow velocities, and resistive indexes were compared. RESULTS: There were 49 Hemangiomas and 38 vascular malformations. A significantly greater proportion of Hemangiomas (48 of 49) compared with vascular malformations (zero of 38) consisted of a solid-tissue mass (P < .001). Vessel density was comparable for Hemangioma and arteriovenous malformation (AVM) but significantly greater compared with the other vascular malformations (P < .001 in each case). No differences in mean arterial peak velocity were detected between Hemangiomas and malformations. Mean venous peak velocity was significantly higher for AVM than for other vascular malformations...
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progressive growth of infantile cutaneous Hemangiomas is directly correlated with hyperplasia and angiogenesis of adjacent epidermis and inversely correlated with expression of the endogenous angiogenesis inhibitor ifn β
International Journal of Oncology, 1999Co-Authors: Diane R Bielenberg, John B Mulliken, Corazon D Bucana, Ricardo Sanchez, Judah Folkman, Isaiah J FidlerAbstract:: Cutaneous infantile Hemangioma progresses through proliferation and involution phases. Since treatment with interferon, a negative regulator of angiogenesis, accelerates the involution phase, we hypothesized that cutaneous infantile Hemangioma is associated with an imbalance between endogenous positive and negative regulators of angiogenesis. We examined 30 specimens of cutaneous Hemangioma [proliferative phase (n=15), involuting phase (n=8), and involuted phase (n=7)] and control human skin (n=17), fixed in formalin and embedded in paraffin. Routine histology, immunohistochemistry, and an mRNA in situ hybridization technique were used to measure expression of the positive angiogenic molecules basic fibroblast growth factor (bFGF) and vascular endothelial growth factor/vascular permeability factor (VEGF/VPF), and an endogenous inhibitor of angiogenesis, interferon-beta (IFN-beta). Proliferative phase Hemangiomas expressed high levels of bFGF and VEGF/VPF but not IFN-beta (mRNA and protein). The epidermis directly overlying proliferating Hemangiomas was hyperplastic, contained numerous dividing cells, and expressed bFGF and VEGF/VPF but not IFN-beta. Epidermis from normal individuals and epidermis directly overlying involuted tumors or at sites distant to the proliferating Hemangioma was not hyperplastic and expressed normal levels of bFGF, VEGF/VPF, and IFN-beta. These data suggest that the proliferation of cutaneous Hemangiomas and adjacent epidermis is associated with an imbalance between positive and negative angiogenic factors expressed by the neoplasm and adjacent normal tissue.
Woo Sung Moon - One of the best experts on this subject based on the ideXlab platform.
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a sclerosing Hemangioma of the liver
Clinical and molecular hepatology, 2013Co-Authors: Ji Soo Song, Yo Na Kim, Woo Sung MoonAbstract:Sclerosing Hemangioma of the liver is an unusual tumor type. Because of its rarity and atypical radiologic findings, sclerosing Hemangiomas can be difficult to distinguish from other lesions such as hepatocellular carcinoma, cholangiocarcinoma, metastasis, and organized abscesses. In this issue, we present a case consisting of both hepatic sclerosing Hemangioma and cavernous Hemangioma in a 63-year-old woman and discuss the histopathologic findings.
Zhongfu Zhao - One of the best experts on this subject based on the ideXlab platform.
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cavernous Hemangiomas of the temporalis muscle with prominent formation of phleboliths case report and review of the literature
Medicine, 2017Co-Authors: Danhui Wang, Guanjun Wang, Peng Cheng, Feng Zhang, Xiaobing Duan, Zhongfu ZhaoAbstract:RATIONALE: Hemangiomas are benign tumors characterized by an abnormal proliferation of blood vessels, most often occur in the skin and subcutaneous tissue, intramuscular Hemangioma, a distinctive type of Hemangioma within the skeletal muscle, account for <1% of all Hemangiomas, temporalis muscle is a very uncommon site, cavernous Hemangioma of the temporalis muscle with prominent formation of phleboliths is rare reported. PATIENT CONCERNS: A 62-year-old man presented with a slowly increased mass in his right temporal fossa. DIAGNOSES: Computed tomography (CT) scan showed the lesion across the zygomatic arch, with many calcified nodules differ in sizes and no erosion to the bone, magnetic resonance imaging (MRI) showed an oval lesion with hypointense and isointense on T2-weighted imaging within the temporal muscle, and preoperation diagnosis was Hemangioma. INTERVENTIONS: The tumor was resected under general anesthesia. OUTCOMES: The mass was excised completely, and the histopathology examination confirmed the diagnosis of cavernous Hemangioma with prominent formation of phleboliths. The patient recovered very well without dysfunctions. LESSONS: Cavernous Hemangioma should be suspected when mass occurs in this region. CT and MRI are important for the early diagnosis of tumor, and resection the tumor completely is recommended.
Robert Myles - One of the best experts on this subject based on the ideXlab platform.
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three cases of large retinal capillary Hemangiomas treated with verteporfin and photodynamic therapy
Archives of Ophthalmology, 2005Co-Authors: Thomas M Aaberg, Daniel F Martin, James Gilman, Robert MylesAbstract:Objective To investigate the efficacy of verteporfin and photodynamic therapy in the treatment of large retinal capillary Hemangiomas. Methods Case reports of 3 patients with large retinal capillary Hemangiomas treated with photodynamic therapy using verteporfin. Standard verteporfin dosages (6 mg/m 2 of body surface area) were given. Both standard and modified photodynamic protocols were followed. Modified protocols included shorter verteporfin infusion times and longer light exposure times. Results Pretreatment best-corrected Snellen visual acuity of the 3 affected eyes were 20/100, 20/50, and 2/200, respectively. All cases had associated exudative retinal detachments involving the macula. Cases 1 and 2 were classic endophytic retinal capillary Hemangiomas. Case 3 was a reactive retinal capillary Hemangioma. Case 1 had 2 photodynamic therapy treatments, and after 8 months, visual acuity improved to 20/40. Two years after initiating photodynamic therapy, the visual acuity was 20/30 and there was no reperfusion of the Hemangioma. Case 2 had 3 photodynamic therapy treatments. The Hemangioma was fibrotic, and 20 months after initiating photodynamic therapy visual acuity improved to 20/30. Case 3 had 1 treatment, 11 weeks later and visual acuity improved to 20/400. Four months after treatment, visual acuity returned to counting fingers because of tractional elevation of the macula as the capillary Hemangioma fibrosed. Vitrectomy surgery was performed, and choroidal and retinal neovascularization was discovered. Three months after vitrectomy visual acuity was 20/400. In cases 1 and 2, the capillary Hemangioma ultimately regressed, and the exudative detachment resolved. Conclusions Verteporfin and photodynamic therapy were effective in achieving closure of large retinal capillary Hemangiomas. In all cases, the Hemangioma underwent fibrosis with consequent macular puckering due to retinal traction. In all cases, the visual acuity improved.
Ilona J. Frieden - One of the best experts on this subject based on the ideXlab platform.
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measuring the severity of infantile Hemangiomas instrument development and reliability
Archives of Dermatology, 2012Co-Authors: Anita N. Haggstrom, Ilona J. Frieden, Beth A Drolet, Maria C Garzon, Kimberly A Horii, Jennifer L Beaumont, Jin She Lai, Denise M Adams, Kristen E Holland, Anne W LuckyAbstract:Objectives To develop instruments that measure the severity of infantile Hemangiomas (Hemangioma Severity Scale [HSS]) and the complications of infantile Hemangiomas for longitudinal use (Hemangioma Dynamic Complication Scale [HDCS]). Design Instrument development and reliability study. Setting Academic research. Participants The HSS and the HDCS were developed through the collaborative effort of members of the Hemangioma Investigator Group Research Core, an expert multi-institutional research group. After development of the scales, 13 pediatric dermatologists used the HSS to score 20 different Hemangiomas. In addition, 12 pediatric dermatologists used the HDCS to score Hemangioma-related complications for 24 clinical scenarios. Interrater and intrarater reliability was measured for both scales. Main Outcome Measures Interrater and intrarater reliability. Results For the HSS, interrater reliability and intrarater reliability exceeded 99%. Similarly, the HDCS had a high rate of interrater agreement; for individual items, agreement among raters was 67% to 100%, with most clinical scenarios demonstrating greater than 90% agreement. Intrarater reliability was excellent for all individual items of the HDCS. Conclusion The HSS and the HDCS are reliable scales that can be used to measure the severity of infantile Hemangiomas, including the severity of complications for longitudinal use.
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lumbar association between cutaneous infantile Hemangiomas of the lower body and regional congenital anomalies
The Journal of Pediatrics, 2010Co-Authors: Ionela Iacobas, Ilona J. Frieden, Anthony J Mancini, John B Mulliken, Patricia E Burrows, Marilyn G Liang, Daniela Kramer, Amy S Paller, Robert A Silverman, Annette WagnerAbstract:Objective To define the clinical spectrum of regional congenital anomalies associated with large cutaneous Hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of Hemangioma location, and provide imaging guidelines for evaluation. Study design We conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases. Results Hemangiomas in our series tended to be “segmental” and often “minimal growth” in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb Hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous Hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies. Conclusions We propose the acronym “LUMBAR” to describe the association of Lower body Hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.
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propranolol in the management of airway infantile Hemangiomas
Archives of Otolaryngology-head & Neck Surgery, 2010Co-Authors: Kristina W Rosbe, Ki Young Suh, Anna K Meyer, Sheilagh Maguiness, Ilona J. FriedenAbstract:Objective To report our experience with propranolol in managing airway infantile Hemangiomas. Design Case series of 3 consecutive patients who had extensive, symptomatic airway infantile Hemangiomas treated with propranolol. Setting Tertiary academic medical center. Patients Three infants with facial cutaneous Hemangiomas who developed stridor that progressed to respiratory distress, which according to laryngoscopic examination results was confirmed to be caused by extensive subglottic Hemangiomas. These patients underwent follow-up during their course of therapy, ranging from 3 weeks to 15 months. Results Patient 1 failed to respond to systemic corticosteroids, laser ablation, and intravenous vincristine for her airway Hemangioma and had to undergo tracheotomy. She was given propranolol after her tracheotomy and had a significant reduction in her subglottic airway obstruction. Patient 2 developed progressive stridor secondary to airway Hemangioma at age 6½ months following tapering of systemic corticosteroids prescribed for her periorbital Hemangioma. Systemic corticosteroids were restarted with the addition of propranolol. The stridor improved within 24 hours, and she was able to be weaned off corticosteroids. Patient 3 was also treated with initial combined therapy of systemic corticosteroids and propranolol. He had a significant reduction in stridor within 24 hours and was weaned off corticosteroids. Conclusions Our 3 patients had severe respiratory symptoms related to their airway infantile Hemangiomas. In the first patient, propranolol was used when other treatments were ineffective or associated with intolerable adverse effects. In the second and third patients, propranolol was part of a dual regimen that resulted in rapid resolution of airway symptoms and allowed for quicker weaning of corticosteroids.
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phace syndrome with intracerebral Hemangiomas heterotopia and endocrine dysfunction
Pediatric Neurology, 2007Co-Authors: Gabriele Poindexter, Denise W Metry, James A Barkovich, Ilona J. FriedenAbstract:PHACE is an acronym to describe the association of posterior fossa brain malformations, Hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, and eye abnormalities. More than 200 cases have been reported. The present report presents the cases of two female infants with PHACE syndrome, both of whom had additional congenital defects of subependymal gray matter heterotopia, craniofacial arterial anomalies, and pituitary dysfunction. One had an extensive segmental facial Hemangioma with ipsilateral intracranial Hemangiomas. The other had multiple cutaneous Hemangiomas, but no segmental facial Hemangioma. These two cases suggest a further expansion of the spectrum of PHACE to include other forms of disordered cerebral development and endocrine dysfunction.
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Patterns of Infantile Hemangiomas: New Clues to Hemangioma Pathogenesis and Embryonic Facial Development
Pediatrics, 2006Co-Authors: Anita N. Haggstrom, Edward J. Lammer, Richard A. Schneider, Ralph S. Marcucio, Ilona J. FriedenAbstract:OBJECTIVES. Large facial infantile Hemangiomas have higher rates of complications than small localized Hemangiomas, more often require treatment, and can be associated with neurological, ophthalmologic, and cardiac anomalies (PHACE syndrome). The anatomic patterns of these Hemangiomas are often referred to as "segmental" despite a lack of precise anatomic definitions. Our study aims to define "segmental" Hemangiomas based on clinically observed patterns. Our secondary goal is to relate the observed patterns to currently accepted developmental patterns to gain insight into Hemangioma pathogenesis and craniofacial development. METHODS. Photographic data were extracted from a large cohort of patients with infantile Hemangiomas. We mapped 294 Hemangiomas and recorded common morphologic patterns. Anatomic descriptions of the most common patterns were described and compared with accepted concepts of craniofacial development. RESULTS. Four primary segments were identified (Segl-Seg4). Seg2 and Seg3 correspond with the previously recognized maxillary and mandibular prominences. Segl and Seg4 differ from standard human embryology texts. The frontotemporal segment, Segl, encompasses the lateral forehead, anterior temporal scalp, and lateral frontal scalp. The segment Seg4, encompassing the medial frontal scalp, nasal bridge, nasal tip, ala, and philtrum, is substantially narrower on the forehead than the previously described frontonasal prominence. CONCLUSIONS. The patterns provide new clues regarding facial development. The observed patterns resemble previously described facial developmental units on the lower face but are distinctly different on the upper face. The patterns suggest that neural crest derivatives may play a role in the development of facial Hemangiomas. Finally, these patterns (Segl-Seg4) help standardize the nomenclature of facial segmental Hemangiomas to analyze more effectively Hemangioma risks and behavior.
