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

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

Abstract Vascular anomalies grouped into vascular tumors (hemangioma) (HI) and vascular malformation (VM) are benign vascular lesions that are difficult to distinguish from one another clinically and often confused with each other at histopathology. This confusing terminology leads to improper diagnosis, illogical treatment, and misdirected research. This study aimed to study GLUT 1, S-100, and nerve bundle to differentiate hemangioma and vascular malformation. Thirty two cases of vascular lesions (26 vascular malformations and 6 hemangiomas) were taken into the study. For histological evaluation and immunohistochemistry (IHC), samples of vascular lesions were collected in formalin. All the hematoxylin and eosin-stained slides were evaluated under light microscope for histology and nerve bundles. Immunohistochemical staining was performed by streptavidin–biotin method for GLUT 1 and S-100. GLUT 1 was positive in all 6 cases of hemangiomas (100%) and only in 1 case of vascular malformation. Nerve bundle was present in 24 cases of vascular malformation (92.3%) out of 26 cases but not in any cases of hemangioma and S-100 was found positive in all vascular malformation cases (100%) but not in hemangioma. So GLUT 1 expression, S-100, and presence of nerve bundle in vascular lesions can help to differentiate hemangioma and vascular malformation.

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

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

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

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

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

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

Hemangioma Versus Vascular Malformation: Presence of Nerve Bundle Is a Diagnostic Clue for Vascular Malformation

2005 ◽  
Vol 129 (6) ◽  
pp. 772-775 ◽  
Author(s):  
Patrick A. Adegboyega ◽  
Suimin Qiu
Keyword(s):  
Vascular Malformation ◽  
Detection Method ◽  
Vascular Malformations ◽  
Vascular Lesions ◽  
Stained Glass ◽  
Nerve Bundle ◽  
Glass Slides ◽  
Nerve Bundles ◽  
Diagnostic Clue ◽  
Hematoxylin Eosin

Abstract Context.—Arteriovenous vascular malformations and hemangiomas are benign vascular lesions that are difficult to distinguish from one another clinically. Also, they may be confused with each other at histopathology. Therefore, histochemical stains for the presence of an artery are frequently used to distinguish between the two. Objective.—Because it is clinically relevant to differentiate between arteriovenous vascular malformations and hemangiomas, this study was carried out to explore additional diagnostic clues that may help in the diagnosis and differentiation of these lesions. Design.—A total of 167 cases of benign extracranial vascular lesions were retrieved from the anatomic pathology file of our institution. These comprised 66 cases diagnosed as arteriovenous vascular malformations and 101 cases previously diagnosed as hemangiomas. The hematoxylin-eosin–stained glass slides were reviewed, Movat pentichrome histochemical stain was used to identify elastic vessels (arteries/arterioles), and S100 immunostain was used to identify nerves within these vascular lesions. For immunohistochemistry, the avidin-biotin detection method was used. Results.—With Movat stain, the presence of thick-walled elastic arteries was detected in 12 of the 101 cases previously diagnosed as hemangiomas, and these cases were therefore reclassified as vascular malformations. Using the same criterion, 2 of the 66 cases originally diagnosed as arteriovenous vascular malformations were reclassified as hemangiomas because they lacked arterial structures. Thus, with this strict criterion, we ended up with 91 cases of hemangiomas and 76 cases of arteriovenous vascular malformations. Intralesional nerves were identified in 91% (69/76) of cases of arteriovenous vascular malformations, including all the 12 arteriovenous vascular malformations previously diagnosed as hemangiomas. In contrast, no intralesional nerve was detected in any of the 91 hemangiomas. Conclusions.—These results show that nerve bundles are consistently present in vascular malformations and absent in hemangiomas and so can be used as a diagnostic clue to differentiate between these lesions. Also, in addition to describing a previously unreported component of vascular malformations, these data further confirm the hamartomatous nature of these lesions.

scholarly journals Cavernous Hemangioma of the Tongue

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

ABSTRACT Background Vascular anomalies are divided into vascular tumors, hemangiomas being the most common, and vascular malformations. Most vascular anomalies are noticed at birth or occur during infancy, and generally involve skin or subcutaneous soft tissues. Adult onset hemangiomas are rare, and intramuscular location is extremely rare. Surgical excision is recommended for hemangiomas in adults, if they are symptomatic, or manifest growth. Materials and methods We report a rare case of a 51-year-old woman, with an intramuscular hemangioma of the tongue, presenting as a submental mass. Preoperative imaging for assessment of tumor extent was followed by a successful surgical excision. Results Postoperative course was uneventful with primary healing of the wound, and with no functional deficit of tongue function. Conclusion Although a variety of treatment approaches are reported for childhood hemangiomas, surgical excision is the preferred treatment for adult onset symptomatic hemangiomas. Preoperative work up should include imaging preferably with contrast enhanced magnetic resonance imaging (MRI). Embolization may be considered for larger lesions. Intraoperative hypotension should be avoided to ensure identification of the entire lesion to ensure complete excision. How to cite this article Petrovic I, Roman B, Shah JP. Cavernous Hemangioma of the Tongue. Int J Head Neck Surg 2015;6(3):109-111.

scholarly journals Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies

2018 ◽  
Vol 190 (09) ◽  
pp. 825-835 ◽  
Author(s):  
Maliha Sadick ◽  
René Müller-Wille ◽  
Moritz Wildgruber ◽  
Walter Wohlgemuth
Keyword(s):  
Classification System ◽  
Vascular Malformations ◽  
Color Doppler ◽  
Vascular Anomalies ◽  
Endothelial Cell Proliferation ◽  
Vascular Tumors ◽  
Interdisciplinary Management ◽  
Venous Malformations ◽  
Medical Evaluation ◽  
Malformation Syndromes

Background Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities. Method A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed. Results Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient’s age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %). Conclusion The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies. Key Points  Citation Format

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

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

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

Vascular Anomalies

2019 ◽  
pp. 241-260
Author(s):  
Lara Wine Lee ◽  
Cameron C. Trenor
Keyword(s):  
Functional Impairment ◽  
Vascular Tissue ◽  
Vascular Malformations ◽  
Diagnostic Procedures ◽  
Vascular Anomalies ◽  
Surgical Approaches ◽  
Vascular Tumors ◽  
Vascular Abnormalities ◽  
The Aesthetic ◽  
Excessive Proliferation

The chapter provides a description of the main anomalies characterized by excessive proliferation of the vascular tissue and the major syndromes associated with them. The predominant types of vascular anomalies are vascular tumors and vascular malformations. Vascular tumors are further subdivided in benign, locally aggressive, and malignant, according to the clinical course of the lesions, while vascular abnormalities are classified as simple (capillary, lymphatic, venous, arteriovenous, and arteriovenous fistula) or combined. For each subgroup of vascular anomalies, clinical presentation and diagnostic procedures are discussed, as well as pathogenic mechanisms and potential comorbidities. The management of vascular anomalies may be oriented to treat the aesthetic discomfort as well as the functional impairment and may involve both pharmaceutical and surgical approaches.

scholarly journals Peripheral Vascular Anomalies – Essentials in Periinterventional Imaging

2019 ◽  
Vol 192 (02) ◽  
pp. 150-162 ◽  
Author(s):  
Maliha Sadick ◽  
Daniel Overhoff ◽  
Bettina Baessler ◽  
Naema von Spangenberg ◽  
Lena Krebs ◽  
...  
Keyword(s):  
Therapeutic Approach ◽  
Vascular Malformations ◽  
Vascular Anomaly ◽  
Flow Dynamics ◽  
Vascular Anomalies ◽  
Vascular Tumors ◽  
Slow Flow ◽  
Peripheral Vascular ◽  
Fast Flow ◽  
Selection Of

Background Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning. Method In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools. Results Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics. Conclusion Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies. Key Points:  Citation Format

scholarly journals Sirolimus for the treatment of vascular anomalies in children

2020 ◽  
Vol 7 (3) ◽  
pp. 22-31
Author(s):  
E. K. Donyush ◽  
Z. A. Kondrashova ◽  
Yu. A. Polyaev ◽  
R. V. Garbuzov
Keyword(s):  
Vascular Malformations ◽  
Venous Malformation ◽  
Antiangiogenic Therapy ◽  
Pneumocystis Pneumonia ◽  
Response To Therapy ◽  
Vascular Anomalies ◽  
Vascular Tumors ◽  
Treatment Regimens ◽  
Transplant Patients ◽  
Results Of Treatment

Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.

Venous-predominant parenchymal arteriovenous malformation: a rare subtype with a venous drainage pattern mimicking developmental venous anomaly

2008 ◽  
Vol 108 (6) ◽  
pp. 1142-1147 ◽  
Author(s):  
So-Hyang Im ◽  
Moon Hee Han ◽  
Bae Ju Kwon ◽  
Jung Yong Ahn ◽  
Cheolkyu Jung ◽  
...  
Keyword(s):  
Arteriovenous Malformation ◽  
Vascular Malformation ◽  
Vascular Malformations ◽  
Gamma Knife Radiosurgery ◽  
Vascular Lesions ◽  
Arterial Phase ◽  
Developmental Venous Anomaly ◽  
Venous Anomaly ◽  
Arteriovenous Shunts

Object Considerable confusion exists in the literature regarding the classification of cerebrovascular malformations and their clinical significance. One example is provided by the atypical developmental venous anomaly (DVA) with arteriovenous shunt, because it remains controversial whether these lesions should be classified as DVAs or as atypical cases of other subtypes of cerebrovascular malformations. The purpose of this study was to clarify the classification of these challenging vascular lesions in an effort to suggest an appropriate diagnosis and management strategy. Methods The authors present a series of 15 patients with intracranial vascular malformations that were angiographically classified as atypical DVAs with arteriovenous shunts. This type of vascular malformation shows a fine arterial blush without a distinct nidus and early filling of dilated medullary veins that drain these arterial components during the arterial phase on angiography. Those prominent medullary veins converge toward an enlarged main draining vein, which together form the caput medusae appearance of a typical DVA. Results Based on clinical, angiographic, surgical, and histological findings, the authors propose classifying these vascular malformations as a subtype of an arteriovenous malformation (AVM), rather than as a variant of DVA or as a combined vascular malformation. Conclusions Correct recognition of this AVM subtype is required for its proper management, and its clinical behavior appears to follow that of a typical AVM. Gamma Knife radiosurgery appears to be a good alternative to resection, although long-term follow-up results require verification.

Sign in / Sign up

Export Citation Format

Share Document