Angiosarcoma gastrointestinal multifocal, con compromiso extenso de estómago, intestino delgado y colon. Reporte de un caso / Multifocal gastrointestinal angiosarcoma, with extensive gastric, small intestine and colon involvement. A case report

Introducción. El angiosarcoma es una neoplasia vascular originada a nivel del endotelio, de baja frecuencia, conocido por su agresividad y crecimiento acelerado. Alcanza solo el 1 al 2 % del total de los sarcomas. La presentación gastrointestinal es infrecuente y su incidencia es poco conocida debido al escaso reporte de casos en la literatura. Suele identificarse en etapas avanzadas debido a la dificultad del diagnóstico histopatológico por sus características morfológicas, siendo necesario aplicar tinciones especiales o estudio inmunohistoquímico. Caso clínico. Por su interés y singularidad, presentamos el caso de un paciente masculino de 54 años, quien consultó con hemorragia digestiva profusa, anemización y requerimiento de terapia transfusional. Resultados. Durante el proceso diagnóstico, en la endoscopia encontraron diversas lesiones multifocales que se extendían por gran parte del tracto gastrointestinal. El estudio histopatológico mostró angiosarcoma gastrointestinal. Discusión. El angiosarcoma del tracto gastrointestinal es extremadamente infrecuente, de difícil diagnóstico y bajas posibilidades de manejo curativo, con opciones terapéuticas limitadas, lo que configura un mal pronóstico a corto plazo

Introduction. Angiosarcoma is a vascular neoplasm originating from endothelial cells, known for its aggressiveness, accelerated growth and reduced frequency. Reach only 1 to 2% of total sarcomas. Gastrointestinal presentation is extremely rare, the true incidence is poorly known, due to the limited reports of this entity in the literature. It is usually identified in advanced stages in view of the difficulty of the histopathological diagnosis, attributable to its morphological characteristics, being necessary to apply special stains or immunohistochemical study. Case report. Due to their interest and uniqueness, we present the case of a 54-year-old male patient, who presented with a profuse gastrointestinal bleeding, anemia, and requirement for transfusion therapy. Results. The endoscopy detected several multifocal lesions that extended most of the gastrointestinal tract. The histopathological study showed gastrointestinal angiosarcoma. Discussion. Angiosarcoma of the gastrointestinal tract is extremely uncommon, difficult to diagnose and has low possibilities of curative management, with limited therapeutic options, which configures a poor prognosis in the short term

Endoscopic management of sinonasal vascular tumors without embolization: Our experience with vessel ligation

Objective@#To present our surgical experience and technique in performing endoscopic sinus surgery for vascular sinonasal tumors without pre-operative embolization using intraoperative ligation of the external carotid artery or its distal branches.@*Methods@#Design: Retrospective Series. Setting: Tertiary Private Teaching Hospital. Participants: Seven Patients. @*Results@#Out of 7 patients (5 males, 2 females, aged 12 to 64 years old) with non-embolized vascular sinonasal tumors, 2 had juvenile angiofibroma, 3 had a benign vascular tumor (hemangiopericytoma, hemangioma and a vasoformative solitary fibrous tumor), and 2 had a malignancy (rhabdomyosarcoma, squamous cell carcinoma). Four (57.1%) had external carotid artery ligation, two (28.6%) had internal maxillary artery ligation and one (14.2%) had sphenopalatine artery ligation. The mean intraoperative blood loss was 2447.1 mL (range 900mL to 5,000mL) and average operation duration was 7.6 hours (range 2.9 hours to 14.5 hours). The average amount of transfused blood products was 1785.7mL (zero to 3,000mL). The average hospital stay was 7 days (range 2 to 13 days) with one post-operative complication (ICU admission for hypotension from intraoperative blood loss). @*Conclusion@#Intraoperative ligation of the ECA or its distal branches to disrupt the vascular supply of sinonasal tumors may provide a viable means of preventing excessive intraoperative blood loss in patients with non-embolized vascular sinonasal tumors.

Tratamiento de malformaciones y tumores vasculares, en un centro de referencia en Bogotá / Treatment of vascular malformations and tumors in a reference center in Bogotá

Introducción. Las malformaciones vasculares son anomalías que están presentes desde el nacimiento, no desaparecen y pueden crecer a lo largo de la vida. Se ha demostrado en estudios retrospectivos que la terminología para clasificar las anomalías vasculares es inexacta en un 69 % de casos, por lo que se hace un diagnostico inadecuado y en un 53 % de casos se brinda al paciente y su familia una información incorrecta del tratamiento y el curso clínico. Métodos. Estudio prospectivo longitudinal, realizado entre 2016 y 2019, donde se incluyeron pacientes con anomalías vasculares, que consultaron a nuestra institución, fueron valorados por el servicio de cirugía vascular, se hizo un plan diagnóstico y manejo integral, vía endovascular, quirúrgica o mixta, de acuerdo con cada caso. Resultados. La malformación más común fue la de tipo venoso, en el 40,3 % de los casos. Se realizó manejo endovascular en el 93,1 % de casos de malformaciones vasculares y quirúrgico en el 6,9 %. La mejoría de los síntomas que motivaron la consulta fue del 100 % para los tumores vasculares y del 70,8 % para las malformaciones. Sin embargo, los resultados son heterogéneos. Discusión. Es necesario realizar un adecuado diagnóstico de las anomalías vasculares, para alcanzar un trata-miento eficaz, con mejoría de los síntomas

Introduction. Vascular malformations are abnormalities that are present from birth, do not disappear and can grow throughout life. It has been shown in retrospective studies that the terminology to classify vascular anomalies is inaccurate in 69% of cases, an inappropriate diagnosis is made and in 53% of cases the patient and their family are given incorrect information on the treatment and the clinical course.Methods. Longitudinal prospective study conducted between 2016 and 2019. Patients with vascular anomalies and consulted to our institution were included. They were evaluated by the vascular surgery service, and a comprehensive diagnosis and management plan was made, including endovascular, surgical or mixed, according to each case. Results. The most common malformation was the venous type in 40.3% of the cases. Endovascular management was performed in 93.1% of cases and surgery in 6.9%. The improvement in the symptoms that led to the con-sultation was 100% for vascular tumors and 70.8% for malformations. However, the results are heterogeneous.Conclusions. It is necessary to obtain an adequate diagnosis of vascular anomalies, to achieve an effective treatment, with improvement of the symptoms

Malignant glomus tumor of the index finger

Glomus tumor (GT) is a benign mesenchymal tumor with an estimated incidence of 1.5 to 2% of soft tissue tumors. The majority of glomus tumors are benign and are mostly seen in the superficial skin & soft tissue of upper and lower distal extremity. The malignant variant of the glomus tumor is scarce. We report a case of a recurrent glomus tumor diagnosed in a 28-year-old male patient, who complained of painful swelling in the proximal phalanx of the right index finger. The magnetic resonance imaging of the hand revealed a well-defined multilobulated soft tissue mass at the palmar aspect of the 2nd digit along the shaft of the proximal phalanx. Histopathology revealed a well-circumscribed tumor arranged in solid sheets, nests and cords interconnect by vessels of varying size. The tumor cells were round to oval, showed moderate nuclear pleomorphism, eosinophilic cytoplasm, atypical mitoses (>5/10HPF), and necrosis. Immunohistochemically tumor cells reveal diffuse and strong cytoplasmic positivity with smooth muscle actin (SMA). Based on histomorphology and immunohistochemistry, a final diagnosis of malignant glomus tumor was made. We report this case due to its rarity, and it to be included among the differential if the lesion is painful and recurrent.

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.

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.

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)

Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review

Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.

Angiossarcoma do couro cabeludo: relato de caso / Angiosarcoma of the scalp: case report

O angiossarcoma é uma rara neoplasia vascular originada das células endoteliais. É muito agressivo com alta taxa de recorrência, metástases precoces e mau prognóstico. O diagnóstico precoce do angiossarcoma é um desafio, pois muitas vezes apresenta-se como uma equimose ou pápula violácea que pode ser confundida com lesão benigna como um hemangioma. O tamanho do tumor é um fator prognóstico importante e o diagnóstico precoce e tratamento combinado são fundamentais para aumentar a sobrevida desses pacientes. Esse estudo relata o caso de um homem de 58 anos de idade com angiossarcoma de couro cabeludo altamente agressivo e prognóstico reservado. (AU)

Angiosarcoma of the head and neck is a rare vascular sarcoma associated with high rates of local recurrence and distant metastasis and a poor prognosis. The diagnosis of cutaneous angiosarcoma can be challenging because it often presents insidiously as a bruiselike lesion or a purplish papule thatmay be mistaken for a benign lesion such as hemangioma. The tumor size is a important prognostic factor and a multimodality treatment approach is imperative for successful outcomes. This study reports the case of 58-years-man with angiosarcoma of the scalp aggressive and a poor prognosis. (AU)

Anorectal hemangioma - differential diagnosis of anal bleeding / Hemangioma anorretal - diagnóstico diferencial de sangramento anal

ABSTRACT Anorectal hemangioma is one of the rarest causes of lower gastrointestinal bleeding, but is often neglected and confused in the differential diagnosis. The clinical examination is a turning point for a correct diagnosis and management of patients, thus avoiding unnecessary procedures. The treatment of choice for this condition is surgical and intraoperative bleeding is the main complication of this therapy. The present case reports a 25-year old patient with a history of bleeding from the age of 13, being diagnosed with anorectal hemangioma, and surgically treated with resection of the affected segment and with wound synthesis by marsupialization, with a good progression postoperatively.

RESUMO O Hemangioma Anorretal é uma das mais raras causas de Hemorragia digestiva baixa, sendo muitas vezes negligenciada e confundida no diagnóstico diferencial. O exame clínico representa um ponto decisivo para correto diagnóstico e manejo do paciente, evitando realização de exames desnecessários, e o tratamento de escolha dessa patologia é cirúrgico, sendo o sangramento intraoperatorio a principal complicação dessa terapêutica. O relato de caso a seguir reporta a história de paciente com 25 anos, que apresentava sangramento desde os 13, sendo diagnosticado com Hemangioma Anorretal e tratado cirurgicamente com ressecção do segmento afetado e síntese de ferida com marsupialização, evoluindo bem no pós-operatório.

Prognostic value of Sox2 expression in digestive tract cancers: A meta-analysis / 华中科技大学学报（医学）（英德文版）

The aim of the present study was to accurately evaluate the association of Sox2 expression with the survival of patients with digestive tract cancers. Relevant literatures were identified by comprehensively searching databases including the Pubmed, Embase, CBMdisc, and Wanfang (up to October 2014). A meta-analysis was performed to clarify the association between Sox2 expression and overall survival or clinicopathological parameters of patients with digestive tract cancers (esophageal, gastric, and colorectal cancers). The results showed a significant association between high Sox2 expression and poor overall survival in patients with digestive tract carcinomas (HR=1.55, 95% CI=1.04-2.31), especially for patients with esophageal cancer (HR=2.04, 95%CI=1.30-3.22), colorectal cancer (HR=1.40, 95% CI=1.04-1.89), and digestive tract adenocarcinoma (HR=1.80, 95% CI=1.12-2.89), for Europeans (HR=1.98, 95% CI=1.44-2.71) or patients who did not receive neoadjuvant treatment (HR=1.73, 95% CI=1.10-2.72). Furthermore, Sox2 over-expression was highly correlated with vascular invasion (OR=1.86, 95% CI=1.25-2.77) and poor differentiation (OR=1.88, 95% CI=1.14-3.08), especially in esophageal and colorectal cancers. In conclusion, Sox2 expression may serve as a novel prognostic factor for patients with digestive tract cancers. Over-expression of Sox2 that is correlated with vascular invasion and poor differentiation suggests poor outcomes of patients with digestive tract cancers.

Angioma en penacho / Tufted angioma

El Angioma en Penacho es un raro tumor vascular de la infancia, que comparte características con el hemangioendotelioma kaposiforme, teniendo ambos posibilidad de desarrollar Síndrome de Kasabach-Merrit. Presentamos una paciente portadora de un angioma en penacho desde el nacimiento, que hemos seguido por 13 años: clínicamente, con estudios de imágenes y biopsias. El caso presenta similitudes con la literatura, con algunas interesantes peculiaridades en su evolución. Consideramos que por el comportamiento clínico e histológico, el Angioma en Penacho es parte de la misma enfermedad que el Hemangioendotelioma Kaposiforme.

Tufted angioma is a rare vascular tumor of childhood that shares features with the Kaposiform hemangioendothelioma, having both tumors the possibility of developing Kasabach-Merrit phenomenon. We report the case of a patient with a tufted angioma since birth, which we have followed for 13 years: clinically, with imaging studies and biopsies. The case presents similarities with literature, with some interesting peculiarities in its evolution. We consider that because of the clinical and histological behavior, Tufted angioma is a part of the same disease that Kaposiform hemangioendothelioma.

Leiomyosarcoma of the great saphenous vein / Leiomiossarcoma de veia safena magna

A 56-year-old male patient presented with a complaint of two painful, hard, palpable nodules in the right lower limb. A Doppler ultrasound scan revealed the presence of nodules, likely to be neoplastic. Computed angiography showed two solid hypervascular nodules in the right great saphenous vein, fed by branches of the posterior tibial artery. Embolization of the nodules using surgical cyanoacrylate was performed, followed by an excisional biopsy. Anatomical pathology and immunohistochemical analysis identified the nodule as a high-grade leiomyosarcoma, characterized by ten mitotic figures per ten high-power fields, necrosis and cell pleomorphism. Immunohistochemical analysis results were positive for caldesmon and desmin labeling. A second surgical procedure was performed to enlarge the free margins.

Paciente do sexo masculino, 56 anos, com queixa de dois nódulos palpáveis, dolorosos e rígidos, em membro inferior direito. O eco-Doppler colorido evidenciou nódulos de provável natureza neoplásica na veia safena magna direita distal, com fluxo de baixa velocidade no seu interior. A angiotomografia evidenciou dois nódulos sólidos hipervascularizados no trajeto da veia safena magna direita, possuindo ramos nutridores provenientes da artéria tibial posterior. Para realizar a biópsia excisional dos nódulos, optou-se inicialmente pela embolização do tumor com cola cirúrgica de cianoacrilato, devido à sua alta vascularização. Os exames anatomopatológico e imuno-histoquímico evidenciaram leiomiossarcoma de alto grau, com dez mitoses por dez campos de grande aumento, necrose tumoral e pleomorfismo celular. A imuno-histoquímica demonstrou positividade para os marcadores Caldesmon (anticorpo hCD) e Desmina (anticorpo D33). O paciente realizou uma nova cirurgia para ampliação de margens comprometidas e está em acompanhamento clínico com a Oncologia.

Sindrome de Maffucci / Maffucci’s syndrome

El síndrome de Maffucci es un raro trastorno congénito de etiología desconocida. Se caracterizapor encondromas, deformidades óseas, hemangiomas y con menor frecuencia linfangiomas. Presentamos un paciente con síndrome de Maffucci y hacemos una revisión de la patología...

Hemangiopericitoma de órbita / Orbital hemangiopericytoma

Hemangiopericitomas são tumores raros originados a partir da proliferação de pericitos, ou seja, células que envolvem os capilares. São encontrados em ossos, pulmões, crânio, partes moles profundas ou membros inferiores, principalmente na coxa. É considerado um tumor com potencial de malignidade incerto e quando afeta a região orbitária pode apresentar um comportamento biológico agressivo, com grande chance de recidiva. O objetivo deste trabalho é relatar um caso de hemangiopericitoma orbital e destacar suas características clínicas, cirúrgicas e histopatológicas. Usualmente, os hemangiopericitomas da órbita são tumores sólidos, únicos e de crescimento lento. Devem ser lembrados no diagnóstico diferencial dos tumores orbitários bem delimitados, como cistos epidermoides, schwannomas, neurofibromas, fibro-histiocitomas, lipomas e malformações vasculares. A confirmação diagnóstica é realizada pelo exame anatomopatológico e, por vezes, complementada pelo estudo imuno-histoquímico. O tratamento deve ser realizado com exérese completa do tumor, com margens amplas, sendo a radioterapia e a quimioterapia reservadas para casos de lesões reincidentes.

Hemangiopericytomas are rare tumors arising from the proliferation of pericytes. They may be found in the lungs, bones, skull, deep soft tissue or limbs. The tumor has an unpredictable prognosis and when it affects the orbital region, may have an aggressive behavior, with high incidence of recurrence. We report a case of orbital hemangiopericytoma and highlight clinical, surgical, and histopathological features of these tumors. Orbital hemangiopericytomas usually are solid, slow-growing tumors. They should be considered in the differential diagnosis of well-defined orbital masses along with epidermoid cysts, schwannomas, neurofibromas, fibrous histiocytomas, lipomas, and vascular malformations. The diagnosis is confirmed by anatomopathologic examination and sometimes complemented by immunohistochemistry. Complete excision of the tumor with wide margins is usually curative; however, radiotherapy and chemotherapy may be required for recurrent lesions.

Tumor glômico: um diagnóstico fácil ou difícil? / Glomus tumor: an easy or a difficult diagnosis?

Introdução: O tumor glômico é uma lesão neoplásica benigna, geralmente única, quese forma no glomo terminal. Esta estrutura é uma anastomose arteriovenosa subcutâneadistribuída por toda a superfície corporal, em particular nos leitos ungueais, polpas digitais,palmas das mãos e plantas dos pés. Apesar da sua raridade, recebe atenção especialpor seus sintomas caracterizados por dor paroxística, sensibilidade à pressão do local, aofrio e por sua localização típica na ponta do dedo. O diagnóstico inicial é raramente feito,levando os pacientes a apresentarem os sintomas por meses e até anos, sem o diagnósticoe tratamento adequados. Objetivo: O objetivo desse trabalho é evidenciar o método diagnósticoclínico e histopatológico do tumor glômico, e a utilização da microscopia em seutratamento cirúrgico. Resultados: Observou-se grande diminuição nos casos de recidiva,com retorno funcional do órgão acometido e melhora da saúde física, funcional e psicossocialdo paciente. Conclusão: A exérese do tumor mostrou-se eficaz e os resultados foramplenamente satisfatórios, não havendo complicações pós-operatórias significativas, nemrecidivas até o momento.

Introduction: The glomus tumor is rare subungueal pathology that consists of a neuromyoarterial.Glomus bodies sensible to temperature oscillations, that regulate arteriolarflow, located at the arteriolar-venula anastomosis. The main symptom of this pathology isan excruciating pain leading to functional limitation of the Lyme. Objective: The purposethis study is to highlight its clinical and histopathological diagnosis, as well as the use ofmicrocopy on its surgical treatment. Results: The treatment resulted in a great reduction ofrecurrence, with functional recovery of the limb and improvement of apaticutp’s physical,social and psycosocial health. Conclusion: The surgical resection of the tumor was efficientand the results were very satisfactory, with no significant post operative complications andno recurrence.

Diagnóstico e tratamento do hemangioma infantil: [revisão] / Diagnosis and treatment of infantile hemangioma: [review]

O hemangioma infantil é o tumor vascular benigno mais frequente nas crianças. Apresenta incidência de 2,5 a 5% em recém-nascidos vivos caucasianos, com predominância no sexo feminino. Durante um longo período não havia uma classificação diagnóstica aceita internacionalmente, dificultando a criação de condutas, bem como comparações entre os diversos tratamentos. Em 1996, a International Society for the Study of Vascular Anomalies dividiu as anomalias vasculares em duas categorias: tumores vasculares e malformações vasculares. O hemangioma a partir deste momento passou a significar um tumor benigno com características peculiares. A sua origem ainda é incerta e diversas hipóteses são formuladas para explicá-la. O quadro clínico apresenta três fases bem definidas: proliferativa, involutiva e involuída. O diagnóstico é realizado por meio da anamnese e do exame físico; quando indicados, ressonância magnética, ultrassom e métodos angiográficos podem ser auxiliares. A maioria dos hemangiomas infantis tem resolução favorável para regressão completa, sem complicações. No entanto, alguns deles podem levar ao comprometimento funcional ou apresentar complicações como ulcerações, infecções, sangramentos ou complicações sistêmicas durante sua evolução. Estes são os casos em que há indicação terapêutica. Entretanto, outras indicações relativas de tratamento podem ser consideradas, devido à localização e às dimensões do tumor. Diversas opções de tratamentos estão disponíveis além do cirúrgico: os tratamentos clínicos, com corticoesteróides (o mais utilizado), o alfa-interferon, com suas indicações um pouco restritas pelos seus efeitos colaterais e, mais recentemente, os beta-bloqueadores, com boa resposta e poucos efeitos colaterais, mas ainda em fase de pesquisa em protocolos clínicos.

Infantile hemangioma is the most common benign tumor of infancy. Incidence is around 2.5 to 5% in caucasian newborns, with predominance in females. For a long period of time, the lack of a single classification accepted worlwide impaired treatment, management and comparisons between treatment groups. In 1996, The International Society for the Study of Vascular Anomalies proposed a classification for vascular anomalies, dividing vascular anomalies in two main groups: vascular tumors and vascular malformations. Infantile hemangioma is the most frequent vascular tumor. There are still several hypothesis for the explanation hemangioma development. Clinically there are three distinct phases, named proliferative, involutive and involuted. Diagnosis is clinically based and imaging methods like magnetic ressonance, ultra sound and interventional radiology may be useful in some cases. The majority of infantile hemangiomas have favorable resolution due to spontaneous regression. However, in some cases complications are present, like ulceration, bleeding, infection or systemic complications. In these cases, active treatment is indicated, as well as in cases of relative indication, depending on size and tumor location. Surgical resection and medical treatment are used. Medications more frequently used are oral corticosteroids, alfa-interferon - with more side effects and now a days beta-blockers, with good response, less side effects, but still under research protocols.

Tratamento de hemangioma ulcerado: relato de caso / Treating an ulcerated hemangioma: a case report

O hemangioma é o tumor vascular mais presente em crianças até 1 ano de idade. Geralmente, sua involução é espontânea, sendo por isso recomendada uma conduta expectante. Ulceração e infecção secundária, seguidas de hemorragia, são as complicações mais frequentes dos hemangiomas. Estima-se que apenas 10 a 20 por cento dos hemangiomas precisem ser tratados. A cirurgia é indicada nos casos de emergência, naqueles em que não há resposta aos tratamentos sistêmicos ou por razões estéticas. O objetivo deste estudo é relatar um caso de hemangioma combinado ulcerado do pavilhão auricular, em paciente de 8 meses de idade submetida a exérese total.

Hemangioma is the most common vascular tumor in children younger than 12 months. Because its regression is usually spontaneous, an expectant management is recommended in most cases. Ulceration and secondary infection followed by bleeding are the most frequent complications of hemangiomas. It is estimated that only 10 to 20 percent of hemangiomas require treatment. Surgery is usually indicated in emergency cases, when there is no response to systemic treatments, or for cosmetic reasons. The objective of this study was to report a case of ulcerated compound hemangioma on the external ear of an 8-month-old patient who underwent a complete surgical excision.

Parotid swelling in children; our experience at department of paediatric surgery B.V.

To describe the clinical and pathological presentation as well as treatment options of parotid swelling in children. Descriptive case series study. Department of Paediatric Surgery Bahawal Victoria Hospital Bahawalpur. From Nov 2005 to Jul 2007. All patients of either sex below the age of 13 years presenting with parotid swelling were included in the study. Clinical presentations, preoperative investigations, operative procedures, histopathology reports, postoperative complications and further management [radiotherapy and chemotherapy] were recorded. Twelve patients presented with parotid swelling. Commonest presentation was a lump over the parotid region [100%] and pain [25%]. Majority of tumours were benign [50%]. Vascular lesions outnumbered solid tumours. 4 patients [33.33%] had haemangioma 1 patient [8.33%] had cystic hygroma, one patient each of pleomorphic adenoma, mucoepidermoid carcinoma and Adenocarcinoma. Salivary gland lesions are most likely inflammatory in origin. Vascular tumours are common benign tumours than epithelial tumours in children. Superficial parotidectomy is the operation for benign tumours and total conservative parotidectomy for malignant tumours

Hemangioendotelioma kaposiforme / Hemangioendothelioma Kaposiform

El hemangioendotelioma kaposiforme es un tumor vascular agresivo y raro que ocurre predominantemente en la infancia. Suele tener localización cutánea o retroperitoneal. Frecuentemente se asocia con el fenómeno de Kasabach-Merritt y ocasionalmente con linfangiomatosis. Las lesiones cutáneas deben ser diferenciadas clínicamente del hemangioma de la infancia, de los hemangiomas congénitos (RICH y NICH) y de otros tumores vasculares. Describimos un caso de hemangioendotelioma kaposiforme cutáneo en una niña de 10 años.

Kaposiform hemangioendothelioma is a rare aggressive vascular tumor that predominantly occurs at infancy and early childhood. It usually presents cutaneous or retroperitoneal location. It is frequently associated with Kasabach-Merritt phenomenon and occasionally with lymphangiomatosis. Cutaneous lesions must be clinically differentiated from infantile hemangiomas, congenital hemangiomas (RICH and NICH) and other vascular tumors. We present a case of cutaneous kaposiform hemangioendothelioma in a 10-year-old girl.