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.

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.

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.

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.

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.

Skin Tumours – Histopathological Review of 125 Cases.

The skin is the largest organ in the body. A wide variety of hyperplastic growths and tumours, both benign and malignant are encountered in the clinical practice. Any lesion, for which the diagnosis is uncertain, based on the history and clinical examination should be biopsied for histopathological examination to rule out malignancy. Objective: To analyze retrospectively tumours of skin with respect to age, sex, clinical features and histopathological features in a tertiary referral centre in Maharashtra, India. Material & Methods: The present study consisted of analysis of tumours of skin received in the histopathology section of department of pathology over a period of 5 years that is from August 2005 to July 2010. The material comprised of biopsies and excision specimens. The clinical and histopathological details were noted. The findings were compared with those reported by other authors. Results: One twenty five (125) tumours of skin were observed. The benign tumours were slightly more common (51.2%) than malignant tumours (48.8%). The maximum number of tumours was found in 7th decade (25.6%). Maximum number of tumours were found in third decade in benign tumours (20.3%) and seventh decade in malignant tumours (37.7%). Both benign and malignant tumours of skin were common in males than females. The equal numbers of skin tumours were seen in both the head and neck region (44.8%) and the extremities (44.8%). Face was the commonest site for skin tumours (35.2%). The keratinocytic tumours, both benign and malignant were common tumours of skin (62.4%) while neural tumours were rarely observed (1.6%). The Squamous Cell Carcinoma (SCC) was the commonest malignant tumour (45.9%) followed by Basal Cell Carcinoma (BCC) (34.4%). Verrucas (32.8%) were the commonest benign tumours followed by pyogenic granuloma (21.9%). Conclusion: SCC is the most common malignant skin tumour in India, unlike the Western countries. Histopathological study is a very important step in the diagnosis of skin tumours.

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.

Tumor intracardíaco como presentación de leiomiomatosis intravascular / Intracardiac tumor as presentation of intravascular leiomyomatosis

Presentamos un caso de leiomiomatosis intravascular que se extiende a cavidades cardiacas derechas, siendo tratado mediante cirugía en un tiempo.

We present a case of an intravenous leiomyomatosis extending into the right cardiac chambers, which was treated by one stage surgery.

Leiomiomatose endovenosa: abordagem anestésica a propósito de um caso clínico / Anesthetic approach for a clinical case of intravenous leiomyomatosis: case report / Leiomiomatosis endovenosa: abordaje anestésico a propósito de un caso clínico

JUSTIFICATIVA E OBJETIVOS: Descrevemos a abordagem anestésica a propósito de um caso clínico de leiomiomatose endovenosa, com invasão da veia cava inferior e extensão à aurícula direita, tratada com êxito por meio de abordagem cirúrgica. RELATO DE CASO: Doente do sexo feminino, 45 anos, aparentemente estável até duas semanas antes da ida à urgência, altura em que iniciou cansaço e dispneia. Fez ecocardiograma, no qual se constatou massa intracardíaca. Por isso, fez tumorectomia eletiva na transição veia cava-aurícula direita e ventrículo direito. À histologia, a peça operatória evidenciou sugestividade de leiomioma uterino. Posteriormente, para avaliar a extensão, fez-se tomografia computadorizada, que demonstrou extensão à veia cava inferior e ao ovário esquerdo. Foi então proposta para histerectomia total, anexetomia esquerda e salpingectomia direita, remoção de leiomioma intravenoso e colocação de filtro definitivo na veia cava inferior. Neste artigo descreve-se a abordagem anestésica com particular ênfase na correção das necessidades hídricas, bem como na evolução no pós-operatório, e salientam-se possíveis contributos à abordagem futura de casos semelhantes. CONCLUSÕES: O diagnóstico pré-operatório de leiomiomatose endovenosa é extremamente difícil. O tratamento consiste na extração cirúrgica. Esse procedimento envolve importantes perdas hemáticas e, por isso, condicionou a preparação pré-operatória. No intraoperatório a fluidoterapia foi fulcral e a monitoração invasiva se assumiu como preponderante no auxílio do equilíbrio hidroeletrolítico da doente. A existência de um laboratório de análises clínicas acessível e com resposta rápida e avaliação gasimétrica próxima foi determinante. A unidade de cuidados intensivos equipada com ventilador para os cuidados pós-operatórios revelou-se outra necessidade decorrente deste caso.

BACKGROUND AND OBJECTIVES: The aim of this study was to describe the anesthetic approach in a case of intravenous leiomyomatosis with invasion of the inferior vena cava and extension to the right atrium, successfully treated with surgical approach. CASE REPORT:Female patient, 45 years old, apparently stable until two weeks before the admission to the emergency department with complaints of fatigue and dyspnea. Echocardiogram was performed, which detected an intracardiac mass. Therefore, elective tumorectomy was performed in the vena cava-right atrium and right ventricle transition. Histological examination of the specimen suggested uterine leiomyoma. Subsequently, to assess the extent, computed tomography was done and showed extension to the inferior vena cava and left ovary. Hysterectomy, left adnexectomy and right salpingectomy, removal of intravenous leiomyoma, and permanent filter placement in the inferior vena cava were proposed. In this article, we describe the anesthetic approach with particular emphasis on the correction of fluid requirements, as well as postoperative evolution, and we highlight possible contributions to future approach of similar cases. CONCLUSIONS: Preoperative diagnosis of intravenous leiomyomatosis is extremely difficult. Treatment consists of surgical removal. This procedure involves major blood loss and, therefore, preoperative preparation was conditioned: intraoperative fluid therapy was central and invasive monitoring considered predominant to assist in fluid and electrolyte balance of the patient; the existence of a clinical laboratory accessible and with rapid response and blood gas assessment was crucial; the intensive care unit equipped with ventilator for postoperative care proved to be another requirement resulting from this case.

JUSTIFICATIVA Y OBJETIVOS: Describimos aquí el abordaje anestésico a propósito de un caso clínico de leiomiomatosis endovenosa, con invasión de la vena cava inferior y extensión a la aurícula derecha, que tuvo éxito por medio de un abordaje quirúrgico. RELATO DE CASO: Mujer enferma, de 45 años, aparentemente estable hasta dos semanas antes de su llegada a urgencias, en que debutó con cansancio y disnea. Hizo el ecocardiograma en que se comprobó masa intracardiaca. Por eso, se procedió a la tumorectomía electiva en la transición vena cava-aurícula derecha y ventrículo derecho. En la histología, la pieza operatoria mostró una sugestión de leiomioma uterino. Posteriormente, para evaluar la extensión, se realizó la tomografía computadorizada, que arrojó una extensión a la vena cava inferior y al ovario izquierdo. Entonces se propuso la histerectomía total, anexetomía izquierda y salpingectomía derecha, retirada de leiomioma intravenoso y colocación de filtro definitivo en la vena cava inferior. En este artículo, describimos el abordaje anestésico dándole un énfasis particular a la corrección de las necesidades hídricas, como también a la evolución en el posoperatorio, destacando posibles aportes al abordaje futuro de casos parecidos. CONCLUSIONES: El diagnóstico preoperatorio de leiomiomatosis endovenosa es extremadamente difícil. El tratamiento consiste en la extracción quirúrgica. Ese procedimiento involucra importantes pérdidas hemáticas y por eso, condicionó la preparación preoperatoria. En el intraoperatorio la fluidoterapia fue fundamental y la monitorización invasiva se asumió como preponderante en el auxilio del equilibrio hidroelectrolítico del enfermo. La existencia de un laboratorio de análisis clínicos accesible y con una respuesta rápida y una evaluación de la gasometría fue determinante. La unidad de cuidados intensivos equipada con ventilador para los cuidados postoperatorios fue otra necesidad originada por este caso.

Primary malignant fibrous histiocytoma involving the left pulmonary vein presenting as a left atrial tumor.

A 35‑year-old woman presented with 4 months history of progressively increasing intermittent dyspnea and hemoptysis. Transthoracic echocardiography revealed a loculated mass in the left atrium (LA). A provisional diagnosis of LA myxoma was made. Intraoperatively the tumor was found extending into and closely adherent to the left pulmonary vein and could not be completely cleared off from the pulmonary venous wall. The histopathological examination of the tumor revealed it to be a myxoid malignant fibrous histiocytoma.

Hemangiopericitoma: reporte de 3 casos / Hemangiopericytoma: a report of 3 cases and a literature review

Introduction: The hemangiopericytoma (HPC) is a rare vascular tumor that can be potentially malignant. It can be found in any part of the body but usually in the lower extremities or the retroperitoneum. Because its potential malign nature, it's necessary to perform oncological resections when are operating on. Methods: Three cases are presented. Results: One of them was located at the sacrococcigeal space, being the second reported case in the international literature. The other cases were in cervical and adrenal localization.

Introducción: El hemangiopericitoma (HPC) es un tumor vascular raro y potencialmente maligno que puede localizarse en cualquier parte del cuerpo, con mayor frecuencia en extremidades inferiores y retrope-ritoneo. Su potencial malignidad da la necesidad de realizar resecciones oncológicas al operarlos. Material y Método: Se presentan tres casos tratados por nuestro grupo. Resultados: Uno de ellos tiene localización sacra siendo, hasta donde sabemos, el segundo en ser publicado. Otro es de localización suprarrenal y el tercero es cervical.

Hemangioma sinovial: presentación de un caso y revisión bibliográfica / Synovial hemangioma: case report and bibliographic revision

El hemangioma sinovial es un tumor benigno vascular de partes blandas mayormente visto en niños y adolescentes. La patogenia de esta lesión es incierta y se encuentra en debate. Presentamos el caso de un paciente de sexo masculino de 41 años de edad, futbolista y tenista amateur, que concurre a nuestro centro con antecedente de dolor de rodilla izquierda de 3 años de evolución. Al examen físico la rodilla era estable, las maniobras de Pívot y Lachman fueron negativas, tampoco presentaba semiología meniscal ni efusión. Se constató un déficit de extensión de 15º, con una flexión completa. Las radiografías de la rodilla afectada no presentaban lesiones óseas y en la resonancia magnética no se visualizaban alteraciones. Luego de realizar tratamiento kinesiológico sin resultados satisfactorios se realizo cirugía artroscópica. Constatándose en la región anterior a la inserción tibial del ligamento cruzado anterior la presencia de una masa de aspecto quístico, similar a la observada en los casos de Ciclops Síndrome. El informe de anatomía patológica informo hemangioma arteriovenoso. En aquellos casos donde se trata de hemangiomas focalizados o pediculados y de tamaño apropiado, la resección artroscópica es la alternativa de tratamiento, y en los hemangiomas puramente sinoviales la embolización selectiva del vaso principal es una alternativa a la cirugía.

Leiomiomatosis intravenosa de origen pelviano con extensión intracardiaca: Reporte de dos casos / Pelvic intravenous leiomyomatosis with intracardiac extension: Report of two cases

Background: symptoms predominate. Diagnosis is based on clinical findings and appropriate imaging. We report two females, aged 35 and 51 years. One of them presented with a pelvic mass and dyspnea, the other patient had severe cardiac failure on admission. Computed axial tomography scan allowed an accurate preoperative diagnosis on both patients. Successful one stage resection of the tumor was performed under cardiopulmonary bypass. Both patients are asymptomatic on follow up at 6 months and 25 years.

Linfoma intravascular de células B grandes: hallazgos clínicos y morfológicos en un caso con desenlace fatal / Intravascular large B cell lymphoma: Report of one case

Intravascular large B cell lymphoma is a rare subtype of large cell lymphoma that is characterized by the proliferation of lymphoid cells within the lumina of small blood vessels. We report a 61-year-old male presenting paresis of both lower limbs, confusion and a history of weight loss. Magnetic resonance and CAT imaging studies showed multiple images of brain and cerebellar infarctions. Twenty days after admission, the patient died and the postmortem study demonstrated a multisystem intravascular large B cell lymphoma.

Tomografia por emissão de pósitrons com 18F fluordesoxiglicose como exame não invasivo para o diagnóstico de sarcomas primários de artéria pulmonar / 18F-fluorodeoxyglucose positron emission tomography as a noninvasive method for the diagnosis of primary pulmonary artery sarcoma

Os sarcomas de artéria pulmonar são tumores raros e de difícil diagnóstico, simulando frequentemente o tromboembolismo pulmonar crônico. Relatamos dois casos de pacientes do sexo feminino com quadro clínico de dispneia e massas pulmonares associadas a falhas de enchimento na artéria pulmonar em angiotomografia de tórax. A tomografia por emissão de pósitrons com 18F fluordesoxiglicose mostrou hipercaptação das respectivas lesões. O sarcoma de artéria pulmonar foi confirmado posteriormente por exame anatomopatológico. Ressaltamos a importância do uso desse tipo de tomografia como exame não invasivo no auxílio diagnóstico desses tumores.

Pulmonary artery sarcomas are rare, difficult-to-diagnose tumors that frequently mimic chronic pulmonary thromboembolism. We report the cases of two female patients with clinical signs of dyspnea and lung masses associated with pulmonary artery filling defects on chest CT angiography. We performed 18F-fluorodeoxyglucose positron emission tomography, which revealed increased radiotracer uptake in those lesions. Pulmonary artery sarcoma was subsequently confirmed by anatomopathological examination. We emphasize the importance of this type of tomography as a noninvasive method for the diagnosis of these tumors.