Tratamento cirúrgico de hemangioma infantil nasal e labial na fase involuída: relato de caso / Surgical treatment of infantile nasal and labial hemangioma in the involuted phase: a case report

Hemangioma infantil (HI) é o tumor vascular mais comum e a neoplasia benigna mais frequente da infância, com maior incidência no sexo feminino e na população branca. Quase 60% dos casos ocorrem em cabeça e pescoço, sendo o tratamento ativo durante a fase proliferativa mais frequentemente indicado, em decorrência dos possíveis problemas funcionais e do potencial desfigurante. Relatamos um caso de paciente com hemangioma infantil involuído de ponta nasal e lábio superior, tratado de forma expectante durante a infância, submetida à correção da deformidade residual com técnicas de rinoplastia, associado à zetaplastia e lipoenxertia do lábio superior com bom resultado e satisfação do paciente.

Infantile hemangioma (IH) is the most common vascular tumor and the most frequent benign neoplasm in childhood, with the highest incidence in females and the white population. Almost 60% of cases occur in the head and neck, and active treatment during the proliferative phase is the most frequently indicated, due to possible functional problems and disfiguring potential. We report a case of a patient with involute infantile hemangioma of the nasal tip and upper lip, treated expectantly during childhood, submitted to residual deformity correction with rhinoplasty techniques, associated with zetaplasty and upper lip grafting with good results and patient satisfaction.

Anomalías Vasculares de la Cavidad Oral: Revisión de la Clasificación y Tratamiento Aplicado a dos Casos Clínicos / Vascular Anomalies of the Oral Cavity: Review of Classification and Treatment Applied to Two Clinical Cases

RESUMEN: Las anomalías vasculares de cabeza y cuello son un grupo de lesiones que afectan vasos sanguíneos y linfáticos donde el tratamiento sigue siendo un desafío. La clasificación actualizada de anomalías vasculares de cabeza y cuello es la clasificación de Mulliken modificada, que las subdivide en a) tumores vasculares y, b) malformaciones vasculares. En este reporte, presentamos dos casos clínicos de pacientes de sexo masculino, con diagnóstico de anomalías vasculares que afectan al labio y paladar duro, diagnosticados como malformación arteriovenosa y malformación venosa, respectivamente. Dichas lesiones remitieron completamente mediante tratamientos conservadores (agentes esclerosantes) y/o quirúrgicos (exéresis quirúrgica completa de la lesión) logrando una remisión completa. Consecutivamente, presentamos una revisión de la literatura enfocado a la clasificación actual, enfoques terapéuticos actuales y futuros.

ABSTRACT: Vascular anomalies of the head and neck are a group of lesions that affect blood and lymph vessels where treatment remains a challenge. The updated classification of head and neck vascular anomalies is the modified Mulliken classification, which subdivides them into a) vascular tumors and b) vascular malformations. In this report, we present two clinical cases of male patients, with diagnosis of vascular anomalies affecting the lip and hard palate, diagnosed as arteriovenous malformation and venous malformation, respectively. These lesions were completely treated with conservative (sclerosing agents) and/or surgical (complete surgical exeresis of the lesion) treatments, achieving a complete remission. Consequently, we present a review of the literature focused on the current classification, current and future therapeutic approaches.

Tratamiento multidisciplinar del leimiosarcoma de vena cava inferior / Multidisciplinary treatment of leiomyosarcoma of inferior cava

Resumen Introducción Los leiomiosarcomas de la vena cava inferior son tumores raros, sólo hay 300 casos descritos en la literatura. Su incidencia es mayor en mujeres, suelen aparecer entre los 50-60 años, y presentan una progresión lenta y mal pronóstico. Los síntomas son inespecíficos haciendo que el diagnóstico se realice de forma tardía, éste se realiza mediante pruebas de imagen y biopsia guiada. Caso clínico Se presenta el caso de un varón de 73 años con diagnóstico de leiomiosarcoma de la vena cava inferior, como hallazgo incidental en TC de control, tratado mediante radioterapia neoadyuvante, cirugía y radioterapia intraoperatoria. Discusión El único tratamiento que ha descrito modificaciones en la supervivencia es la cirugía. El papel de la adyuvancia y neoadyuvancia en estos tumores es muy controvertido. La elección de la actitud terapéutica dependerá de la localización del tumor, tamaño, la relación con estructuras adyacentes y la presencia de circulación colateral.

Introduction Leiomyosarcomas of the inferior vena cava are rare tumors, with fewer than 300 cases reported. Its incidence is higher in females, usually appear in the sixth decade and they have a slow-growing and poor prognosis. Symptoms are generally non-specific. Diagnosis is made with imaging studies and guided biopsy. Clinical Case We report a case of a 73-year-old male patient with leimyosarcoma of the inferior vena cava treated by neoadjuvant radiotherapy, surgery and intraoperative radiotherapy. Discussion Surgery is the only treatment that can improve the survival. The role of the adjuvancy and neoadjuvancy is very controversial. Surgical management is determined by the location of the tumour, the relationship with adjacent structures and the presence of collateral veins.

Rapidly involuting congenital hemangioma

Abstract: Rapidly involuting congenital hemangioma is a rare vascular tumor that generally has a good prognosis. The authors describe a case of a newborn girl with a left cervical vascular lesion. Image exams were performed, and the lesion slowly decreased, leaving redundant skin. Considering all of the findings, a final diagnosis of a rapidly involuting congenital hemangiomas was suspected.

Angiolymphatic invasion as a prognostic fator in resected N0 pancreatic adenocarcinoma / Invasão angiolinfática como um fator prognóstico no adenocarcinoma pancreático ressecado N0

ABSTRACT Background: Pancreatic adenocarcinoma remains one of the worst digestive cancers. Surgical resection is the main target when treating a patient with curative intent. Aim: To assess angiolymphatic invasion as a prognostic factor in resected pN0 pancreatic cancer. Methods: Thirty-eight patients were submitted to pancreatoduodenectomy due to head pancreatic cancer. Tumor size, margins, lymph nodes, pTNM staging, angiolymphatic and perineural invasion were described in the pathologists' reports. Results: Most patients were female. Overall median survival was 13 months. Gemcitabine was the regimen of choice for chemotherapy in selected patients; however, it did not improve overall survival. pR0 resection had better survival compared with pR1. Within the pN0 group, survival was significantly better in patients without angiolymphatic invasion. Conclusion: Angiolymphatic invasion in N0 pancreatoduodenectomy can be demonstrated by the Hematoxylin-Eosin stain and may predict a poor prognosis factor for those patients.

RESUMO Racional: Adenocarcinoma pancreático continua sendo um dos piores cânceres do aparelho digestivo. A ressecção cirúrgica é o principal objetivo quando se trata de intenção curativa. Objetivo: Avaliar a invasão angiolinfática como um fator prognóstico no câncer da cabeça do pâncreas ressecado pN0. Método: Trinta e oito pacientes foram submetidos a duodenopancreatectomia por câncer da cabeça do pâncreas. Tamanho do tumor, margens, linfonodos, estadiamento pTNM, invasão angiolinfática e perineural foram descritos nos laudos anatomopatológicos. Resultados: A maioria foi de mulheres. A sobrevida mediana global foi de 13 meses. Gencitabina foi a droga de escolha para quimioterapia nos pacientes selecionados, entretanto não aumentou a sobrevida global. Pacientes com ressecção pR0 tiveram sobrevida global superior quando comparados com ressecção pR1. Dentro do grupo de pacientes com pN0, a sobrevida foi significativamente melhor no grupo de pacientes que não apresentavam invasão angiolinfática. Conclusão: A invasão angiolinfática da duodenopancreatectomia N0 pode ser demonstrada utilizando apenas a hematoxilina-eosina e pode predizer prognóstico ruim para estes pacientes.

Hemangiosarcoma at the base of the left atrium of a dog with mitral and tricuspid valve degeneration ­ case report / Hemangiossarcoma na base do átrio esquerdo de um cão com degeneração da válvula mitral e tricúspide ­ relato de caso

Hemangiosarcoma (HSA) is a malignant neoplasm originating from the vascular endothelium, with high metastatic power and commonly described in the spleen, liver, and right atrium. Thus, the objective of the present study is to report the occurrence of HSA at the base of the left atrium of a male, elderly, Lhasa Apso dog, treated with cyanosis, orthopedic position, cough. In the physical examination, the presence of crackling was observed in pulmonary lobes, murmur in focus of mitral and tricuspid grade IV\VI. The recommended emergency therapy was performed until the clinical improvement of the animal. After echocardiography, mitral and tricuspid valvular degeneration was confirmed, as well as the presence of an irregular, mixed echocardiogram, extra-cardiac, immobile at the base of the left atrium, measuring 2.9 x 1.44 cm in diameter. The clinical therapy established stabilized the clinical picture for 128 days, with recurrence of pulmonary edema and severe hemorrhagic pleural effusion, with the owners opting for euthanasia of the animal and necropsy confirming the definitive diagnosis of extrahepatic SAH at the base of the left atrium.(AU)

O hemangiossarcoma (HSA) é uma neoplasia maligna originada no endotélio vascular, com alto poder metastático e comumente descrito no baço, fígado e átrio direito. Assim, o objetivo deste trabalho é relatar a ocorrência do HSA na base do átrio esquerdo de um cão, macho, idoso, da raça Lhasa Apso, atendido apresentando cianose, posição ortopneica e tosse. No exame físico foi observado presença de crepitação em lobos pulmonares e sopro em foco de mitral e tricúspide grau IV\VI. Foi realizada terapia emergencial preconizada até a melhora clínica do animal, e após a ecocardiografia confirmou-se a degeneração valvular mitral e tricúspide, bem como a presença de uma imagem irregular, de ecogenicidade mista, extracardíaca e imóvel na base do átrio esquerdo, medindo 2,9 x 1,44 cm de diâmetro. A terapia clínica instituída estabilizou o quadro clínico por 128 dias, havendo recidiva do edema pulmonar e efusão pleural hemorrágica grave, com os proprietários optando pela eutanásia do animal e a necropsia, confirmando o diagnóstico definitivo do HSA extracardíaco na base do átrio esquerdo.(AU)

Pelvic Intravenous Leiomyomatosis - Case Report / Leiomiomatose intravenosa pélvica - caso clínico

Abstract Introduction Intravenous leiomyomatosis is a benign and rare condition that can result in cardiac events with fatal outcomes when left untreated. Intravenous leiomyomatosis is probably underestimated because the diagnosis is easily missed. We present a case of an intravenous leiomyomatosis without extra-pelvic involvement, with a brief review of this pathology. Case Report 46-year-old woman submitted to hysterectomy and bilateral adnexectomy because of a pelvic mass detected in ultrasound. During the surgery, intravenous leiomyomatosis diagnosis was suspected. Pathological analysis confirmed this suspicion. Further imaging exams were performed without detecting any anomalies related to this condition. The patient remained with no evidence of disease after one year of follow-up. Conclusion Intravenous leiomyomatosis is a rare condition that can lead to serious complications. Early diagnosis followed by an appropriate treatment is very important to patient outcome, and underdiagnoses can be counteracted if the gynecologist is aware of this entity.

Resumo Introdução A leiomiomatose intravenosa é uma condição benigna, rara, que pode resultar em eventos cardíacos, podendo ser fatal quando não tratada. Esta patologia está provavelmente subestimada, uma vez que facilmente não é diagnosticada. Neste artigo, apresentamos um caso de leiomiomatose intravenosa sem envolvimento extrapélvico, com uma breve revisão da patologia. Relato de Caso Mulher de 46 anos de idade, submetida a histerectomia e anexectomia bilateral após detecção ecográfica de massa pélvica. Durante a cirurgia, houve a suspeita de leiomiomatose intravenosa, e o exame anátomo-patológico confirmou o diagnóstico. A paciente foi submetida a outros exames de imagem, não sendo detectada qualquer anomalia relacionada com a patologia. Após um ano de followup, a paciente manteve-se sem evidência de doença. Conclusão A leiomiomatose intravenosa é uma condição rara que pode levar a complicações graves. O diagnóstico precoce e o tratamento adequado são muito importantes para o prognóstico da paciente, e os subdiagnósticos podem ser evitados se o ginecologista estiver ciente dessa entidade.

Hemangiossarcoma ocular em um equino: relato de caso / Equine ocular hemangiosarcoma: case report

O hemangiossarcoma ocular na espécie equina é um tumor maligno, raro e agressivo, de origem vascular endotelial. No presente trabalho, descreve-se um caso de hemangiossarcoma ocular em uma égua de 10 anos que apresentava secreção serossanguinolenta advinda de uma massa, acometendo a conjuntiva bulbar e a terceira pálpebra do olho direito. O diagnóstico foi realizado com base na avaliação histopatológica e na imuno-histoquímica. Foi realizada a enucleação, assim como a completa excisão cirúrgica do tecido acometido, não sendo observada, após seis meses da terapia, a recidiva ou a metástase da lesão.

Equine ocular hemangiosarcoma is a rare and aggressive malignant tumor of vascular endothelial origin. We describe a case of ocular hemangiosarcoma in a 10-year-old mare with serosanguineous secretion arising from a mass involving the bulbar conjunctiva and third eyelid of the right eye. The diagnosis was based on histopathological evaluation and immunohistochemistry. Enucleation was performed as complete surgical excision of the affected tissue, with no recurrence or metastasis of the lesion being observed after six months of.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

Angiosarcoma of the head and neck

Introduction 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. Objective We describe our experience treating patients with angiosarcoma of the head and neck to evaluate the outcomes, patterns of failure, and current treatments. Methods We identified six patients with angiosarcoma of the head and neck and treated at our institution between 2000 and 2013. We compared our results to the literature from 1979 to 2013. Results Mean follow-up was 42 months. Local recurrence rate was 50% with diseasespecific survival and 2-year disease-free survival rates of 33.3 and 20%, respectively. Prognostic factors included tumor size > 5 cm and surgical margin status, with no correlation between histologic grade and survival. Combined-modality therapy was only used for aggressive tumors with positive surgical margins but is suggested to improve local control and overall survival. Conclusions Our data series supports that angiosarcoma of the head and neck has a high rate of recurrence and is associated with a poor prognosis, despite current combined-modality therapy. The study highlights the importance of attaining negative margins during surgical resection, the utility of adjuvant therapies, as well as the need for continued research in developing new management strategies.

Linfoma intravascular, un desafio diagnóstico: Caso clínico / Intravascular lymphoma: Report of one case

Intravascular lymphoma is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by clonal proliferation of lymphocytes inside of small and medium caliber vessels. Its incidence is estimated at one case per million. The clinical picture is very variable, but frequently has skin and central nervous system involvement. It is diagnosed by demonstrating pathological blood vessel infiltration by lymphoma cells. We report a 44 years old male presenting with fever, malaise and erythematous lesions in the abdominal wall. An abdominal wall biopsy showed dilated vascular vessels with atypical cells in their lumen, compatible with large B-cell intravascular lymphoma. He was treated with rituximab, cyclophosphamide, adriamycin, vincristine and prednisone and an autologous hematopoietic stem cell transplantation, achieving a complete remission that has lasted two years.

A case of acquired tufted angioma in adulthood

Tufted angioma is a rare vascular tumor whose name derives from its histopathological appearance, characterized by tufts of capillaries within the dermis. Its etiology and pathogenesis are uncertain. Tufted angioma typically occurs during infancy or early childhood and displays various clinical patterns. It may present as a subtle stain-like area that later thickens as a large plaque, infi ltrated or dusky blue-purple lesion, or as an exophytic, fi rm, violaceous, cutaneous nodule. Medical treatment is not necessary for tufted angioma, given its benign nature and slow progression. Only clinical follow-up is therefore recommended.

Targetoid hemosiderotic hemangioma - Case report

Targetoid Hemosiderotic Hemangioma, also known as Hobnail Hemangioma, is a lesion of vascular origin, probably lymphatic. The most common clinical feature is a solitary violaceous papule surrounded by a pale, thin area and a peripheral ecchymotic ring, simulating a target. Histopathologically, there is a biphasic pattern, with dilated vessels in the superficial dermis and pseudoangiosarcomatous pattern in the deep dermis, and endothelial cells with hobnail morphology. A simple excision is curative. We report a rare case of Targetoid Hemosiderotic Hemangioma.

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin / Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case

Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

Case for diagnosis

Intravascular papillary endothelial hyperplasia is a benign vascular lesion caused by proliferation of endothelium. It is reactive to thrombotic or inflammatory stimuli in the vessel wall.We report the case of a 14-yearold male patient with a violet-colored erythematous tumoral lesion of progressive growth in the occipital region. The diagnosis of intravascular papillary endothelial hyperplasia (IPEH) was confirmed by clinical and histopathological findings. Total lesion exeresis was performed with no recurrence up to date. IPEH presents clinical importance due to its clinical and histological resemblance to angiosarcoma. In order to differentiate it from angiosarcoma, distinguishing features of the benign disease should be considered, such as lack of cellular atypia and rare mitotic activity.Prognosis is good.