Brief Description of ISSVA Classification for Radiologists.

Ongoing discovery regarding the origin and treatment of vascular anomalies requires standardized nomenclature which itself must undergo iterative updating. This article introduces the 2018 International Society for the Study of Vascular Anomalies (ISSVA) classification, emphasizing the biologic basis of vascular anomalies, summarizing the key features of commonly encountered entities, and serving as a foundation for subsequent articles presented herein. Vascular tumors are discussed to highlight their distinction from vascular malformations which will receive greater attention with respect to management and technical considerations within the issue.

Reconstrução de couro cabeludo após ampla ressecção de angiossarcoma / Scalp reconstruction after wide resection of an angiosarcoma

Introdução: O couro cabeludo é uma área de difícil reconstrução devido à sua pouca elasticidade e por sobrepor-se a uma estrutura rígida e convexa. Existem diferentes técnicas cirúrgicas para reparação dos defeitos, que podem ser produto de diversas etiologias, como: traumas, deformidades e consequência de doenças, principalmente oncológicas, como é o caso do paciente apresentada neste trabalho. O angiossarcoma cutâneo é um tumor vascular maligno raro e extremamente agressivo, que afeta principalmente idosos. É caracterizado clinicamente pelo aparecimento de placas eritêmato-violáceas e de rápida evolução. O tratamento depende da extensão da doença. A maioria dos casos são tratados com ampla ressecção cirúrgica e reconstrução. O objetivo é relatar um caso de reconstrução de grande defeito do couro cabeludo depois de uma dissecção oncológica, realizado no Hospital Central do Exército (HCE) - RJ. Métodos: O caso foi tratado com enxerto autólogo e uso de matriz dérmica, em 2 tempos cirúrgicos, até a total cobertura da área lesionada. Resultados: Obteve-se resultado satisfatório após as cirurgias de enxertia de pele na área ressecada. Conclusão: O enxerto autólogo, juntamente a matriz dérmica mostrou-se uma opção viável na reconstrução do couro cabeludo.

Introduction: It is difficult to reconstruct the scalp due to its poor elasticity and presence of layers over a rigid convex structure. Different surgical techniques are used to repair defects that may develop due to several etiologies, such as trauma, deformities, and disease sequelae, especially cancer, as noted in the present case. Cutaneous angiosarcoma, a rare and extremely aggressive malignant vascular tumor that mainly develops in elderly individuals, is clinically characterized by the onset of rapidly evolving erythematous purple plaques. The treatment depends on disease extent. Most patients are treated with wide surgical resection and reconstruction. The objective is to report a case of reconstruction of a major scalp defect after an oncologic dissection performed at the Hospital Central do Exército in Rio de Janeiro. Methods: An autologous graft and dermal matrices were applied during two surgical periods till the damaged area was fully covered. Results: Satisfactory results were obtained after performing skin grafting surgery in the resected area. Conclusion: The autologous graft and dermal matrix proved to be a viable option for scalp reconstruction.

Vascular anomalies of the upper limb.

Vascular anomalies are common in the upper extremities, but there continues to be a relative paucity of information about them in publications dealing with surgery in the hands and upper limbs. The wide spectrum of pathology and an inconsistent use of terminology make vascular anomalies susceptible to incorrect diagnosis and as a result, to misdirected management. This article aims to provide an update on vascular anomalies relevant to the upper limbs, focusing on significant advances in pathogenesis and genetics, classification systems, diagnosis and treatment.

Concomitant subcutaneous intravascular lymphoma lesions in a patient with cardiac diffuse large B-cell lymphoma: is intravascular lymphoma a distinct clinical entity?

We describe a patient who presented with large cardiac diffuse large B-cell lymphoma (DLBCL) and adrenal masses. The patient also had subcutaneous intravascular lymphoma lesions which were detected by random skin biopsy. Although ambiguous, minimal extravascular location of lymphoma cells is permitted for the diagnosis of intravascular large B-cell lymphoma (IVLBCL) in the WHO definition, a number of rare cases have been reported as having concomitant tumours in other organs, such as the adrenal gland, brain, and penis. We assume that IVLBCL might be a peculiar feature of DLBCL characterised by preferential localisation of lymphoma cells within the capillaries rather than a distinct disease entity of DLBCL.

[Benign aggressive vascular anomalies in children]. / Anomalies vasculaires bénignes agressives de l'enfant et de l'adolescent.

Superficial vascular anomalies constitute a large group of malformative and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are constituted of dysplastic vessels. The classification from International Society for the Study of Vascular Anomalies (ISSVA) is based on this pathogenic difference. The most common vascular tumor is infantile hemangioma, which treatment, when necessary, is propranolol. Congenital hemangiomas and tumors that might be complicated with Kasabach-Merritt phenomenon, i.e. deep thrombocytopenia, are much rarer. Management of Kasabach-Merritt phenomenon is now largely based on sirolimus. Low-flow VMs include capillary, venous and lymphatic malformations; arteriovenous malformations are high-flow malformations. These different types of VMs might be combined. Currently, there is an increasing work in delineating the different entities based on molecular findings. Treatment of VMs depends on the impairment linked to them, and is decided case by case, in pluridisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially efficient, and management of patients with VMs increasingly involves medical drugs. First-line treatment of coagulation disorders associated with venous malformations is based on low molecular weight heparin; sirolimus seems efficient in hemorrhagic complications refractory to usual treatment. Sirolimus is about to become the standard treatment in painful inflammatory manifestations of mixed and/or complicated lymphatic malformations.

Estado actual del tratamiento del angiofibroma nasal juvenil / Current status of the nasopharyngeal angiofibroma treatment

El angiofibroma nasal juvenil es un tumor benigno de presentación infrecuente, que afecta a varones prepuberes y púberes predominantemente, altamente vascularizado lo que explica el sangrado nasal importante que acompaña la sintomatología de estos pacientes, y que puede devenir en complicaciones fatales durante el acto operatorio. La localización anatómica donde se origina este tumor y la relación que establece durante su crecimiento con otras estructuras en la base de cráneo explican la complejidad que implica tratar a este tipo de pacientes. La presente revisión tiene por objeto presentar el estado actual del tratamiento de estos tumores. El tratamiento de elección es la cirugía, con una tendencia cada vez mayor a realizar abordajes menos invasivos, dejando otras modalidades como la radioterapia para aquellos casos inoperables. (AU)

The nasopharyngeal angiofibroma is an infrequent benign tumor that presents in male patients, predominantly preadolescents, highly vascularized which explains the high volume nasal bleeding during the course of the disease, that can even cause fatal outcomes during surgery. The anatomic location where this tumor originates and the following compromise of adyacent structures in the skull base explains the complexity of the treatment of this tumor. The following article review the state of the art of the treatment offered to the patients published in the literature. The election treatment is surgery, with a growing tendency to offer minimally invasive techniques, and recommending other modalities, such as radiotherapy, for advanced tumors not amenable to surgical resection. (AU)

Algunas especificidades sobre los hemangiomas externos en niños / Some specificities on external hemangiomas in children

Los hemangiomas son tumores vasculares benignos con características biológicas muy específicas en la infancia; estos se pueden localizar en órganos, y reciben el nombre de internos, o en cabeza, cuello, tronco y extremidades, que son los denominados externos, los cuales crecen a una velocidad variable. Con este trabajo se buscó exponer algunas particulares clinicoepidemiológicas de los hemangiomas externos, su clasificación, diagnóstico y evolución. Asimismo, se refieren aspectos relacionados con la efectividad del propranolol como propuesta terapéutica de primera elección en niños y se presentan los resultados de algunos autores al respecto, que en su mayoría convergen en cuanto a la aplicación de este medicamento como de primera línea.

Hemangiomas are benign vascular tumors with very specific biological characteristics during childhood; these tumors can be located in organs, and receive the name of internal, or in head, neck, trunk and extremities that are denominated external, which grow to a variable speed. Some clinical epidemiological particularities of external hemangiomas were exposed with this work, such as their classification, diagnosis and clinical course. Also, there is a reference to some aspects related to the effectiveness of propranolol as therapeutic proposal of first election in children and the results of some authors in this respect are presented, most of which coincide as for its use as first line medication.

Congenital Vascular Tumors.

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

Multifocal vascular lesions.

Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac compromise, and neurologic sequelae. This review provides a synopsis of the epidemiology, pathogenesis, presentation, workup, and treatment of several well-recognized multifocal vascular tumors and malformations.

Overgrowth syndromes with vascular malformations.

This review provides a clinically-oriented summary of the most commonly encountered overgrowth syndromes associated with vascular malformations. This manuscript will outline morphologic features, clinical evaluation and management of this complex group of patients. Recent genetic advances have aided in classification and help to explain overlapping clinical features in many cases.

Vascular Lesions.

Vascular lesions in childhood are comprised of vascular tumors and vascular malformations. Vascular tumors encompass neoplasms of the vascular system, of which infantile hemangiomas (IHs) are the most common. Vascular malformations, on the other hand, consist of lesions due to anomalous development of the vascular system, including the capillary, venous, arterial, and lymphatic systems. Capillary malformations represent the most frequent type of vascular malformation. IHs and vascular malformations tend to follow relatively predictable growth patterns in that IHs grow then involute during early childhood, whereas vascular malformations tend to exhibit little change. Both vascular tumors and vascular malformations can demonstrate a wide range of severity and potential associated complications necessitating specialist intervention when appropriate. Evaluation and treatment of the most common types of vascular lesions are discussed in this article. [Pediatr Ann. 2016;45(8):e299-e305.].

Preoperative tumor embolization.

In this article, the authors review general principles and technical details of preoperative embolization of various hypervascular head, neck, and spinal tumors encountered in contemporary neuroendovascular practice. Indications, treatment goals, techniques, outcomes, and complications are discussed, and illustrative case examples are presented.

Cryotherapy-induced release of epiretinal membrane associated with retinal vasoproliferative tumor: analysis of 16 cases.

PURPOSE: To evaluate epiretinal membrane (ERM) response after cryotherapy for retinal vasoproliferative tumors (VPTs). METHOD: Retrospective interventional case series. RESULTS: Of 16 eyes with VPT and ERM, the tumor was classified as primary in 12 (75%) eyes or secondary in 4 (25%) eyes. The median patient age was 44 years (mean, 43 years; range, 9-70 years). The tumor was located in extramacular zone (n = 16, 100%) and inferotemporal quadrant (n = 12, 75%). The mean tumor base was 6 mm, and thickness was 3 mm. The ERM involved the macula in 12 (75%) eyes and extramacular zone in 4 (25%) eyes, with best-corrected visual acuity of 20/40 or better in 6 (38%) eyes. Associated features included cystoid macular edema (n = 8, 50%), subretinal fluid (n = 10, 63%), vitreous cells (n = 9, 56%), and vitreous hemorrhage (n = 7, 44%). Single-session cryotherapy (double freeze-thaw) to the VPT was performed in each case. Over mean follow-up of 68 months (median, 54 months; range, 8-252 months), tumor regression was documented in 16 (100%) cases, with ERM release in 10 (63%) cases. After ERM release, the foveal anatomy was normal in 12 (75%) eyes. Final visual acuity improved (n = 5, 31%), remained stable (n = 9, 56%), or worsened (n = 2, 13%). Posttreatment best-corrected visual acuity was 20/40 or better in 10 (63%) eyes. CONCLUSION: Cryotherapy is remarkably effective for VPT of 6 mm or less in basal dimension. After cryotherapy, VPT-related ERM spontaneously released in 63% of the cases, without the need for surgical intervention.

Congenital and infantile skin lesions affecting the hand and upper extremity, part 1: vascular neoplasms and malformations.

Many dermatologic conditions may be present on a newborn infant's upper extremity that can evoke concern for parents and/or primary caregivers. Although the pediatrician typically remains the first care provider, often these children are referred to specialists to diagnose and treat these lesions. Hand surgeons should be familiar with different infantile skin lesions on an upper extremity. Some lesions are best observed, whereas others require treatment with nonoperative measures, lasers, or surgical interventions. A 2-part series is presented to aid the hand surgeon in becoming familiar with these lesions. This part 1 article focuses on vascular neoplasms and malformations. Particular attention is paid to the multiple types of hemangiomas and hemangioendotheliomas, telangiectasias, angiokeratomas, as well as capillary, venous, and lymphatic malformations. Diagnostic tips and clinical photographs are provided to help differentiate among these lesions. In addition, the recommended treatment for each is discussed.

Cutaneous vascular lesions.

In 1982, vascular anomalies were classified as either vascular tumors or vascular malformations. Hemangiomas were identified as benign tumors that undergo a phase of active growth characterized by endothelial proliferation and hypercellularity, followed by gradual tumor regression over the first decade. Vascular malformations were described as structural congenital anomalies derived from capillaries, veins, lymphatic vessels, arteries, or a combination of these. Unlike vascular tumors, vascular malformations were shown to have normal levels of endothelial turnover and to grow proportionately with the child. This article describes the most common types of vascular anomalies and available treatment modalities.

Vascular tumors of the conjunctiva in 140 cases.

OBJECTIVE: To report clinical and histopathologic features of vascular tumors of the conjunctiva. DESIGN: Retrospective, noninterventional case series. PARTICIPANTS: A total of 140 patients. INTERVENTION: None. MAIN OUTCOME MEASURES: Tumor diagnosis, anatomic location, clinical features, management, and histopathology. RESULTS: There were 140 vascular tumors of the conjunctiva with 93% benign and 7% malignant. The specific diagnoses included lymphangioma/lymphangiectasis (n = 54, 36%), pyogenic granuloma (n = 31, 22%), capillary hemangioma (n = 20, 14%), Kaposi's sarcoma (n = 10, 7%), acquired sessile hemangioma (n = 10, 7%), racemose hemangioma (n = 7, 5%), varix (n = 4, 3%), cavernous hemangioma (n = 3, 2%), and glomangioma (n = 1, <1%). The lesions were unilateral in 89% and discovered at median age of 41 years. Bilateral lesions included lymphangiectasia, Kaposi's sarcoma, and racemose hemangioma. All tumors were typically found in adults with the exception of capillary hemangioma (<1 year) and glomangioma (16 years). The median tumor diameter was 6 mm, with the largest median diameter (15 mm) with Kaposi's sarcoma and (12 mm) glomangioma and capillary hemangioma and the smallest median diameter (3 mm) with cavernous hemangioma. The most common anatomic sites included bulbar conjunctiva, which was extralimbal in 80 patients (57%), limbal in 19 patients (14%), tarsal conjunctiva in 27 patients (19%), and forniceal conjunctival in 24 patients (17%). Clinical features included cystic component in 24% of patients (seen with lymphangioma, glomangioma, and varix), well-defined margins in 64% of patients, and feeder vessels in 39% of patients. CONCLUSIONS: A variety of vascular tumors can occur in both children and adults at various anatomic locations in the conjunctiva. They tend to occur unilaterally on the bulbar conjunctiva, and the majority of tumors are benign.