Hemangioma capilar en el hallux: presentación de un caso / Capillary hemangioma in the hallux: Case report

Introducción: Los hemangiomas son neoplasias benignas que se originan de células endoteliales; rara vez resultan malignos. El síntoma más común es el dolor, porque el hemangioma comprime un tronco nervioso cercano o un nervio directamente. Presentamos un caso de hemangioma en una región muy poco frecuente. Se trata de una mujer de 35 años que presentaba una tumoración friable, de coloración marrón, dolorosa, en la región distal del hallux, con aumento de tamaño en los últimos meses. Se realizó la exéresis completa de la tumoración. El diagnóstico anatomopatológico fue hemangioma capilar. No se observó recidiva luego de 36 meses de seguimiento. Conclusión: Frente a estas neoplasias, se recomienda la exéresis de la pieza y su posterior estudio. Nivel de Evidencia: IV

Introduction: Hemangiomas are benign neoplasms originating from endothelial cells and may rarely be malignant. The most common symptom is pain, due to the compression of a nerve or nerve trunk by the hemangioma. We present the case of a patient with this type of tumor in an infrequent location. The patient was a 35-year-old female with a painful, brown-colored and friable tumor in the distal region of the hallux which had increased in size in the last months before treatment. Complete surgical resection of the tumor was performed, with a histopathological diagnosis of capillary hemangioma. No recurrence was observed during the 36-month follow-up. Conclusion: We recommend a complete resection of these neoplasms and their posterior histopathology analysis. Level of Evidence: IV

A capillary angioma of the lips: an extremely rare situation in the oral cavity / Angioma capilar de los labios: una situación extremadamente rara en la cavidad oral

Hemangiomas are tumors identified by rapid endothelial cell proliferation in early infancy, followed mostly by involution over the time. However, 10-12 % of true hemangiomas don't involute and require surgical treatment. Hemangiomas are classified on the basis of their histological appearance as capillary, mixed and cavernous. We report a case of capillary angioma in a 13-year old boy who presented with a budding into the lower lip. The lesion was excised and histopathological report confirmed the diagnosis.

Los hemangiomas son tumores identificados por la rápida proliferación de células endoteliales en la primera infancia, seguidos principalmente por involución a lo largo del tiempo. Sin embargo, entre el 10 y el 12 % de los hemangiomas no intervienen y no requieren tratamiento quirúrgico. Los hemangiomas se clasifican en función de su aspecto histológico como capilares, mixtos y cavernosos. Reportamos un caso de angioma capilar en un niño de 13 años que presentó un brote en el labio inferior. La lesión fue extirpada y el informe histopatológico confirmó el diagnóstico.

Pulmonary capillary hemangiomatosis: a lesson learned

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. Clinical features are characterized by nonspecific findings such as shortness of breath, cough, chest pain, and fatigue. It can be clinically indistinguishable from pre-capillary pulmonary arterial hypertension disorders such as primary pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension. However, the diagnostic distinction, which usually requires a multidisciplinary approach, is crucial in order to avoid inappropriate treatment with vasodilator medications usually used for PAH treatment. Prognosis of PCH remains poor with lung transplant being the only definitive treatment. We report an autopsy case of pulmonary capillary hemangiomatosis unmasked at autopsy that was treated with a prostacyclin analog, usually contraindicated in such patients. We emphasize that this entity should always be on the differential diagnosis in a patient with pulmonary hypertension and requires great vigilance on the part of the clinician, radiologist and pathologist to make the diagnosis and guide appropriate management.

Intramedullary Spinal Capillary Hemangioma: Case Report

Capillary hemangiomas involving the neuraxis are very uncommon. In the spinal cord, they are located mainly intradural and extramedullary. To our knowledge, only four cases in conus medullaris have been previously described. In our case, a 46-year-old man was admitted with back pain, sphincter disturbances, as well as progressive weakness and numbness on the lower extremities. Magnetic resonance imaging revealed an undefined intramedullary lesion on the conus medullaris. The patient underwent microsurgery, which achieved complete removal. Histopathological diagnosis was compatible with capillary hemangioma. His postoperative course was uneventful and all symptoms, including bladder dysfunction clearly regressed. The treatment of intramedullary capillary hemangiomas is very critical in preventing unnecessary morbidity, providing accurate information with respect to prognosis, and establishes a regular outpatient follow-up. The natural history of this lesion involving the spinal cord is not well described, although they are common elsewhere in the body.

Hemangiomas capilares que envolvem o neuroeixo são raros. Quando localizados na coluna vertebral, geralmente são intradurais e extramedulares. Até a presente data somente 4 casos de hemangiomas no cone medular foram descritos. O caso refere-se a um homem de 46 anos com dor lombar, alterações esfincterianas, fraqueza e parestesias em membros inferiores de evolução progressiva. Ressonância magnética demonstrou lesão no cone medular de aspecto indefinido. Submetido a remoção microcirúrgica completa. Histopatológico compatível com hemangioma capilar. O pósoperatório ocorreu sem intercorrências e houve recuperação dos déficits, inclusive da disfunção esfincteriana. O tratamento dessa lesão não deve acrescentar morbidade. A história natural dessa lesão na medula espinhal ainda não está definida, apesar de ser uma lesão comum em outras partes do organismo.

Hemangiomatosis capilar pulmonar congénita en un recién nacido / Congenital pulmonary capillary hemangiomatosis in a newborn

La hemangiomatosis capilar pulmonar es una entidad poco frecuente, caracterizada por la proliferación de capilares que infiltran paredes alveolares, septos interlobulillares, pleura e intersticio pulmonar, sin características de malignidad, con asociación casi constante a hipertensión pulmonar. Hasta el momento, solo se han reportado en la literatura, dos casos de presentación congénita; este es el tercer caso en un recién nacido y que no se asocia a hipertensión pulmonar. Esta se encuentra en la mayoría de los pacientes con dicha patología, con mayor incidencia entre los 20 y los 40 años de edad. Se presenta a un recién nacido pretérmino de 36 semanas de gestación con dificultad respiratoria progresiva, que requirió asistencia ventilatoria mecánica por desaturaciones constantes en su evolución clínica, sin signos clínicos, radiológicos o ecográficos de hipertensión pulmonar.

Pulmonary capillary hemangiomatosis is a rare entity characterized by the proliferation of capillaries into alveolar walls, interlobular septa, pleura and pulmonary interstitium, without malignant characteristics, with almost constant association with pulmonary hypertension. Until now two cases of congenital presentation have been reported in the literature. This is the third case in a newborn; he has not followed the usual pattern associated with pulmonary hypertension as occurs in most patients with this pathology; the highest incidence is among 20-40 years old. We report a preterm newborn patient of 36 weeks of gestation with progressive respiratory distress requiring mechanical ventilation by constant desaturation during his clinical evolution without clinical, radiological or ultrasonographic signs of pulmonary hypertension.

Epidural Capillary Hemangioma of the Thoracic Spine / Hemangioma capilar extradural da coluna torácica

Background Hemangiomas are congenital vascular malformations pathologically considered as harmatomas and classified as capillary, cavernous, arteriovenous or venous, and usually located at soft tissue or bone, mainly in the spinal column. Pure epidural capillary hemangiomas are extremely rare lesions that should be included in the differential diagnosis of spinal epidural lesions; only three patients with epidural capillary hemangiomas have been reported to date. Case Report We report a case of a 57-year-oldman that complained of dorsal and back pain. The neurological examination revealed back tenderness and crural paraparesis. His reflexes were exaggeratedand Babinski signwaspresenton both sides.Amagnetic resonance imaging showed an epidural lesion at the level of T10­12 that demonstrated extension with intense postgadolinium enhancement. These lesions were different from more common lesions, mainly schwanommas, mainly due to the foraminal extension, which sets them apart from cavernous hemangiomas. The surgical ressection was performed. After laminectomy, a reddish epiduralmass that extended intothe right T11­12 foraminawas revealed. Thefeeding vessels had to be identified and divided. In such cases, the surgeonmust carefully dissect the lesion circumferentially away from the dura and employ judicious hemostasis. The patient́s histopathological examination revealed a vascular tumor composed of vessels of several calibers. The imagery obtained from the exams led to the diagnosis of a capillary hemangioma. Conclusions Pure epidural capillary hemangiomas should be included in the differential diagnosis of spinal epidural lesions, mainly schwanommas, especially due to the foraminal extension, which may differentiates them from cavernous hemangiomas. Surgical excision is mandatory and intervertebral foraminal extension may preclude gross total resection.

Introdução Os hemangiomas são malformações vasculares congênitas patologicamente consideradas como hamartomas. Podem ser classificadas como capilar, cavernoso, arteriovenoso ou venoso, e são geralmente localizadas em tecidos moles ou ossos, principalmente na coluna vertebral. Hemangioma capilar epidural puro é uma lesão extremamente rara que deve ser incluída no diagnóstico diferencial das lesões espinais epidurais, foram relatados casos de apenas três pacientes com hemangiomas capilares epidurais. Relato de Caso Relatamos o caso de um homem de 57 anos de idade com queixa de dorsalgia. Ao exame neurológico, paraparesia crural, com hiperreflexia e sinal de Babinski bilateral. A ressonância magnética mostrou uma lesão epidural no nível de T10­12 com intenso realce pós-gadolíneo. Hemangioma capilar deve ser diferenciado de lesões mais comuns, principalmente schwannomas, devido à extensão foraminal. A ressecção cirúrgica foi realizada. Um processo expansivo epidural avermelhado, se estendendo para o forâmen direito de T11­12, tornou-se evidente após a laminectomia. Os vasos que o irrigavam foram identificados e adequadamente separados. A lesão foi cuidadosamente dissecada circunferencialmente e uma hemostasia criteriosa foi realizada. O exame histopatológico revelou um tumor vascular composto por vasos de vários calibres. Exames de imagem corroboraram com a hipótese de um hemangioma capilar. Conclusões Hemangiomas capilares epidurais puros devem ser incluídos no diagnóstico diferencial das lesões da coluna vertebral epidural, principalmente schwanommas, especialmente devido à extensão foraminal. A excisão cirúrgica é obrigatória e a extensão para o forame intervertebral pode impossibilitar a ressecção total.

Kasabach-Merritt syndrome: clinical vs. surgical treatment / Síndrome de Kasabach-Merritt: tratamento clínico versus cirúrgico

Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient's clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.

A síndrome de Kassabach-Merritt é uma associação de hemangioma capilar e trombocitopenia, que promove sangramentos com petéquias, equimoses e hematomas espontâneos. A conduta é tratar com corticoide, interferon alfa ou quimioterápicos. Apresentamos um caso de criança (com 1 ano e 9 meses anos de idade) com um hemangioma gigante desde a raiz da coxa até altura do joelho, e plaquetopenia. O tratamento foi iniciado com corticoterapia sem melhora do quadro, surgindo de forma espontânea pontos de sangramento intratumoral e cutâneo. Sem condições clínicas de receber vincristina e o interferon, foi feita a ressecção tumoral de urgência por extrema plaquetopenia e sangramento. A criança começou então a desenvolver um quadro séptico com hipotensão e isquemia do tecido residual. Diante deste quadro, configurou-se um desafio terapêutico que será objeto deste artigo.

Angioma capilar espinal intradural: reporte de un caso y revisión de la literatura / Spinal cord capillary hemangioma: a case report and literature review

INTRODUCTION: Capillary angiomas are extremely rare in the spinal intradural space (being even less frequent in the intramedullary location)10-24. We analyze the characteristics of these lesions. METHODS: We present a case report of a patient with a symptomatic spinal-intradural capillary hemangioma at the thoracic level, followed by a review of the literature. RESULTS: We found a total of 41 patients reported with single angiomas, and three more patients with multiple lesions. Most patients were male (33/44 patients), and the mean age was 53,5 years. The localization of these lesions was mainly in the dorsal spine (24), cauda equina (15) and conus medullaris (6), with one case reported in the cervical spine 11. Most patients presented with back pain, mielopathy or radiculopathy. Contrary to the case of cavernous angiomas, we did not find cases of capillary angiomas presenting with subarachnoid or intramedullary hemorrhage. The imaging characteristics (isointense at T1WI, hyperintense at T2WI and with intense contrast enhancement) were constant in almost all the reports. CONCLUSIONS: Spinal intradural capillary angiomas are rare vascular lesions, frequently mistaken for intradural tumors. It affects mostly males in the fifth or sixth decade of life, and is preferentially located in the thoracolumbar spine. It can be occasionally associated with marked spinal cord edema, specially when there is a intramedullary component. These lesions have a good prognosis after surgical treatment, and must be taken into account in the differential diagnosis of intradural tumors of thoracolumbar spine and cauda equina.

Hemangiomas infantiles / [Infantile hemangiomas].

Infantile hemangiomas are the most common benign tumors of infancy. While most of them have an uncomplicated course, between 10 and 15

are at risk for complications, especially during the proliferative phase, and can cause impairment of vital functions or produce permanent cosmetic disfigurement. In this update we delineate the natural history of infantile hemangiomas, with special emphasis on identifying those that require special diagnostic evaluations and multidisciplinary and closer follow-up, reviewing the study and treatment indications and current treatment modalities.

Hemangioma capilar como causa de colestasis extrahepática: reporte de caso / Capillary hemangioma as cause of extrahepatic cholestasis: case report

El hemangioma capilar es una patología epitelial benigna que se presenta usualmente en la piel, sin embargo también puede presentarse en mucosas, hasta el momento en la literatura revisada no hay reportes de hemangiomas capilares como causa de colestasis extrahepáticas. Se reporta el caso de un paciente femenino de 35 años con clínica de colestasis extrahepática sin evidencias de lesiones ocupantes de la luz de las vías biliares, se le realiza duodenoscopia donde se evidencia lesión de aspecto adenomatoso a nivel de la papila de Vater, la cual es resecada. El reporte anatomopatológico concluye la lesión como un hemangioma capilar. En vista de que en la literatura no se encuentran reportes de hemangiomas capilares como etiologías de colestasis se decide reportar dicho hallazgo

The capillary hemangioma is a benign epithelial disease usually occurs in the skin, but can also occur in mucous membranes, so far in the literature there are no reports of capillary hemangiomas as a cause of extrahepatic cholestasis. We report the case of a female patient 35 years old, with clinical signs of extrahepatic cholestasis and no evidence of occupying lesions of the bile ducts, duodenoscopy was performed which revealed a lesion of adenomatous aspect of the papilla of Vater, which is resected and sent to pathology. The report from pathology was of a capillary hemangioma. Since in the literature are no reports of capillary hemangiomas as etiologies of cholestasis is decided to report this finding

Hemangioma de mama simulando metástase no PET-CT / Breast hemangioma mimicking metastasis at PET-CT

Hemangioma de mama é um tumor benigno raro que apresenta pouca ou nenhuma captação de 18F-flúor-2-deoxi-Dglicose (FDG) na tomografia por emissão de pósitrons (PET). Relatamos um nódulo mamário compatível, patologicamente, com hemangioma, em uma mulher cuja PET scan demonstrou captação elevada de FDG (simulando tumor maligno). Também fizemos breve revisão das causas que levam a resultados falso-positivos e falso-negativos pela PET.

Breast hemangioma is a rare benign tumor that presents either absent or low 18F-fluoro-2-deoxy-D-glucose (FDG) uptake at positron emission tomography (PET). The authors report the case of a breast nodule pathologically compatible with hemangioma in a woman whose PET-scan has demonstrated increased FDG uptake (simulating a malignant tumor). A brief review of factors leading to false positive and false negative PET results is also undertaken.

Oral propranolol as a new treatment for facial infantile hemangioma: case report / Propranolol oral: novo tratamento para hemangioma facial em bebês: relato de caso

Hemangiomas are the most common benign tumors of infancy. Despite their self-limited course, infantile capillary hemangiomas can impair vital or sensory functions as vision and cause cosmetic deformity. The usual treatments include oral/intralesional steroids, alpha interferon, cytotoxins, pulsed dye laser and cosmetic surgery resection. These treatments are not free of multiple complications and toxic side effects. This report describes the case of a 3-month-old female baby with progressively increasing hemangioma of the left upper eyelid impinging over the visual field. The hemangioma promptly responded to low-dose oral propranolol. A clinical response was noticed few days after the beginning of the treatment, with regression to 1/4 of its original size in 45 days of treatment, and to less than 1/10 after 8 months, free of any major side effects.

Hemangiomas são os tumores benignos mais comuns durante o primeiro ano de vida. Apesar do seu curso autolimitado, os hemangiomas capilares podem prejudicar funções vitais ou sensoriais como a visão e causar alteração estética. O tratamento usual inclui esteróides orais ou intralesionais, interferon alfa, citotoxinas, laser e ressecção cirúrgica. Entretanto estes tratamentos não estão livres de complicações e efeitos adversos. Este relato descreve o caso de um bebê feminino de 3 meses com um hemangioma rapidamente progressivo na pálpebra superior esquerda, causando obstrução no eixo visual. O hemangioma respondeu rapidamente a uma baixa dose oral de propranolol. A resposta clínica foi notada poucos dias após o início do tratamento, com regressão a 1/4 do seu tamanho original após 45 dias de tratamento, e a menos de 1/10 após 8 meses, sem ter apresentado nenhum efeito adverso.

Uso de timolol en hemangiomas de la infancia / Using timolol in hemangiomas of infancy

Los hemangiomas de la infancia (HI) son los tumores vasculares benignos más frecuentes de la niñez. Ocasionalmente requieren tratamiento y, en aquellos en que esté indicada la intervención, se utilizan como terapias sistémicas los corticoides y, más recientemente, el propranolol. En el año 2010, se publicó por primera vez un trabajo sobre el uso de timolol tópico para HI no complicados, superficiales y de tamaño pequeño. Se presentan tres pacientes con HI que fueron tratados con timolol tópico, en los cuales se obtuvo un resultado satisfactorio

Infantile hemangiomas (IH) are the most common vascular benign tumors of childhood. Occasionally, treatment is required and in those in which intervention is indicated, the use of oral steroids and -most recently- propranolol, are established systemic therapies. In 2010 the use of topical timolol for the treatment of uncomplicated, superficial and small sized IH, was first published. We report 3 patients with IH treated with topical timolol showing satisfactory results

Capillary hemangioma of the sphenoid sinus / Hemangioma capilar do seio esfenoidal

Hemangiomas of upper respiratory tract are uncommonly seen in clinical practice, and there are only a few reports of cases arising in the nasal sinuses. Specifically concerning capillary hemangiomas of the sphenoid sinus, as far as we know, there is only one case reported in the literature. This case report describes also a capillary hemangioma of the sphenoid sinus. A 26 year- old man complained of an intense headache and a retronasal bloody drainage. After some days in conservative treatment without any improvement, and after having an imaging study showing partial opacification of the sphenoid sinus, he underwent a left sphenoidectomy. The lesion identified in the region was ressected and sent to histopathological analysis, which revealed a capillary hemangioma. The patient is on follow-up for 12 months now and still asymptomatic. This case report emphasizes the importance of also considering this diagnosis when evaluating paranasal sinuses related complaints, even when faced with a clinical history and ancillary exams indicative of a common inflammatory disease. Despite the good result seen with the surgical management of this patient, this alternative should be carefully chosen, taking into account possible hemorrhagic complications of different hemangiomas.

Hemangiomas da via aérea superior são vistos raramente na prática clínica e há apenas alguns relatos de casos nos seios paranasais. Falando especificamente do hemangioma capilar do seio esfenoidal, pela nossa busca na literatura, há apenas um relato. Descrevemos aqui mais um caso de hemangioma capilar do seio esfenoidal. Paciente de 26 anos com cefaleia intensa e drenagem pós-nasal que, após alguns dias de tratamento conservador sem melhora, e tendo feito exames de imagem mostrando opacificação parcial do seio esfenóide, foi submetido a esfenoidectomia esquerda. A lesão identificada na região foi ressecada e enviada para análise histopatológica. A mesma revelou um hemangioma capilar. O paciente, com seguimento de 12 meses, permanece assintomático. Esse relato enfatiza a importância de considerar esse diagnóstico quando avaliadas queixas relacionadas aos seios paranasais, mesmo quando a história e exames indiquem uma patologia inflamatória comum. Apesar do bom resultado cirúrgico desse paciente, essa alternativa terapêutica deve ser considerada com cuidado, levando em conta as possíveis complicações hemorrágicas dos diferentes hemangiomas.

Hallazgos imaginológicos en el síndrome de phaces: reporte de caso / Imaging findings in phaces syndrome: case report

Los hemangiomas de tipo capilar son los tumores de la infancia más frecuentes, principalmente en niños menores de un año de edad y usualmente afectan la cabeza y el cuello. Por lo general, están solitarios; sin embargo, aproximadamente un 20% de los niños con grandes hemangiomas cervicofaciales tendrán una de las anomalías asociadas en el síndrome de PHACES, un raro síndrome neurocutáneo de predominio en el sexo femenino, con características mayores, como malformaciones cerebrales en la fosa posterior, hemangiomas de tipo capilar, anomalías arteriales, coartación de aorta, defectos cardiacos y anormalidades oculares. Cuando se asocia con hendidura esternal o rafe supraumbilical, es referido como síndrome de PHACES. Se presenta el caso de una niña de cuatro años de edad con hemangioma facial congénito asociado con anomalías vasculares cerebrales y de la fosa posterior.

Capillary hemangiomas of infancy are the most common childhood tumors, mainly in children under 1 year old, and they usually involve the head and neck. They are usually solitary, but about 20% of the children with large cervicofacial hemangiomas will have one of the anomalies associated with PHACES syndrome. PHACES is a rare neurocutaneous syndrome with female predominance and features such as: brain malformations in the posteriorfossa, hemangiomas, arterial anomalies, coarctation of the aorta, heart defects, and ocular abnormalities. When associated with sternal slit and/or supraumbilical Raphe, it is referredto as PHACES syndrome. The case of a 4-year-old child with congenital facial hemangioma associated to the posterior fossa and with cerebral vascular anomalies is presented.

Malformações capilares: resultados preliminares do tratamento associando laser ND:Yag 1064 nm e luz intensa pulsada / Capillary malformations: preliminary results of treatment associating ND:Yag 1064 nm laser and intense pulsed light

Introdução: As malformações vasculares congênitas do tipo capilar têm seus melhores resultados até o momento com o laser de corante. Nós associamos, em um único equipamento, o laser ND:Yag 1064 nm de pulso longo e a luz intensa pulsada, para o tratamento de lesões superficiais e profundas. Método: Dezessete pacientes foram tratados com a associação descrita em regime ambulatorial, com anestesia tópica, apenas em intervalos mensais, pelo mesmo cirurgião. O equipamento utilizado foi a plataforma Synchro (DEKA), com a ponteira de 4 mm de diâmetro e fluência de 110 J/cm2 para o laser ND:Yag 1064nm. Para a luz intensa pulsada foi utilizado um filtro de corte de 550 nm, a ponteira de 46 x 10 mm e 21 J/cm2 de fluência, divididos em dois disparos. Os resultados foram avaliados clinicamente e por meio de fotografias, em conjunto com os pacientes. Resultados: Dentre os dezessete pacientes tratados, quatro resultados foram considerados excelentes, nove, bons, quatro, regulares e nenhum insuficiente. Conclusão: O tratamento pode ser realizado adequadamente com anestesia tópica em regime ambulatorial. A associação das duas tecnologias possibilitou a obtenção de resultados satisfatórios e, provavelmente, é mais uma técnica no tratamento destas lesões.

Introduction: Congenital vascular malformations up to know, have their best results with dye laser. We associate, in single equipment, long pulse Nd:Yag 1064 nm laser and intense pulsed light, for simultaneous treatment of the superficial and deep lesions. Methods: Seventeen patients were treated with the association described in an office basis with topical anesthesia only at monthly intervals by the same surgeon. The equipment utilized was the Synchro Platform (DEKA) with a 4 mm diameter spot size and fluency of 110 J/cm2 for the ND:Yag 1064 nm laser. For the intense pulsed light was utilized a 550 nm cutoff filter with a 46 x 10 mm spot size and 21 J/cm2 as fluency, divided in two shots. Results were evaluated through clinical and photographical analysis together with the patient. Results: Among the seventeen patients treated, four results were considered excellent, nine, good,four, regular and none insufficient. Conclusion: The treatment can adequately be done in an office basis with topical anesthesia. Association of the two different technologies got a satisfactory result and it is probably one more technique for treating such lesions.

Importância do exame oftalmológico na doença de von Hippel-Lindau / The importance of the ophthalmological examination in von Hippel-Lindau disease

Von Hippel-Lindau (VHL) é uma síndrome tumoral autossômica dominante. Esses tumores incluem hemangioblastoma da retina e sistema nervoso central (CSN), carcinoma de células renais, feocromocitoma, tumores de pâncreas, cistoadenoma de rins, pâncreas e epidídimo. Os sintomas mais comuns são perda da visão, aumento da pressão intracraniana, déficits neurológicos, aumento da pressão arterial sistêmica paradoxal e dor local. Relatamos o caso de um paciente com perda de visão e história de hemangiomas cerebelares cujo diagnóstico de VHL foi feito após exame oftalmológico.

Von Hippel-Lindau (VHL) disease is an autossomical, dominant inherited tumour syndrom. These tumours may include haemangioblastoma in the retina and central nervous system (CNS), renal cell carcinoma, phaeochromocytoma, islet cell tumours of the pancreas, cystadenoma in the kidney, pancreas, and epididymis. The most common symptoms include: loss of vision, raised intracranial pressure, neurological deficits, paroxysmal raised blood pressure and local pain. We report herein a 29-year-old man with visual loss and cerebellar haemangioblastoma that despite neurological manifestations the diagnosis of VHL was established after the ophthalmological examanination.

Síndrome de POEMS con lesión lítica aislada y hemangiomas glomeruloides: caso clínico / POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: report of one case

POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.