Multiple pulmonary infantile hemangiomas responsive to oral propranolol.

Intrathoracic infantile hemangiomas (IHs) are extremely rare. They may be located in the pericardium, trachea, bronchia, diaphragm, mediastinum, or the lungs and may be associated with cutaneous IHs. We present a newborn with multiple pulmonary IHs in the absence of skin lesions that showed a dramatic response to oral propranolol.

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.

SOLITARY RETINAL CAPILLARY HEMANGIOMA IN A PATIENT WITH BILATERAL CHORIORETINAL COLOBOMA.

PURPOSE: To report a case showing a very rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient. METHODS: Observational case report. RESULTS: A 21-year-old woman presented at our clinic for vision screening. She had a history of bilateral chorioretinal coloboma with amblyopia in her left eye. Her best-corrected visual acuity was 20/25 in her right eye and 20/200 in her left eye. On slit-lamp examination, iris coloboma was observed in the left eye. Fundus evaluation showed inferior chorioretinal coloboma in both eyes, and an elevated, round, and orange-red retinal lesion with feeder vessels localized in the midperipheral region of the right eye. The patient was diagnosed as having solitary retinal capillary hemangioma and underwent argon laser therapy for treating the tumoral lesion. Diagnostic studies were negative for von Hippel-Lindau disease. This is the first reported case of solitary retinal capillary hemangioma associated with bilateral chorioretinal coloboma. CONCLUSION: Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Retinal capillary hemangioma is a vascular retinal tumor that may occur sporadically or as part of the von Hippel-Lindau syndrome. We report a rare association of chorioretinal coloboma and retinal capillary hemangioma in a previously healthy patient.

An update of 77 cases diagnosed as oral hemangiomas based on GLUT-1 positivity.

OBJECTIVES: To evaluate cases diagnosed as "oral hemangiomas" based on the immunohistochemical expression of human glucose transporter protein (GLUT-1) and on histopathological features, and to investigate whether the classification proposed by the ISSVA was used correctly to classify these lesions. MATERIAL AND METHODS: All cases stored in the archives of an Oral Pathology Service and diagnosed as "oral hemangiomas" were reviewed. Seventy-seven cases were analyzed regarding the expression of GLUT-1. GLUT-1(+) specimens were classified as true infantile hemangioma (IH) and GLUT-1(-) specimens were reclassified based on their histopathological features. The nomenclature of these lesions was evaluated and some cases were reclassified. RESULTS: Only 26 (33.8%) of the specimens were indeed IHs. Among the GLUT-1(-) specimens, 20 (26.0%) were reclassified as pyogenic granulomas (PGs) and 31 (40.2%) as vascular malformations. Considering the previously applied nomenclature, only 47.5% of the cases initially diagnosed as "hemangiomas" were IHs. In the group of "capillary hemangiomas", most cases (56.2%) were PGs. Among the three "cellular hemangiomas", two were PGs and one was IH. Most (88.8%) "cavernous hemangiomas" were vascular malformations. CONCLUSION: Careful and parameterized review of cases of vascular anomalies is necessary using auxiliary tools such as GLUT-1, since the exclusive use of histopathological findings might be insufficient to differentiate some anomalies. CLINICAL RELEVANCE: Accurate clinical examination and the use of biomarkers such as GLUT-1 are essential for the diagnosis.

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.

Oral capillary hemangioma: a clinical protocol of diagnosis and treatment in adults.

PURPOSE: Hemangiomas are benign vascular anomalies characterized by benign proliferation of blood vessels. There are no well-defined criteria for the diagnosis and treatment of oral capillary hemangioma (OCH). The objective of this study is to report a safe, effective, and low-cost protocol for diagnosis and treatment of OCH. MATERIALS AND METHODS: Eight patients were diagnosed with OCH, through two physical examination maneuvers-the diascopy and head lowering maneuver with abdominal compression (HLMAC). The treatment performed was sclerotherapy with ethanolamine oleate, weekly, until the disappearance of the lesion. No local anesthetics were administered prior to the sclerosing agent injection. RESULTS: All cases showed a complete remission of the lesions after sclerotherapy. There were no visible scars or compromise esthetics or normal function, and there was no evidence of recurrence. Only one patient had superficial ulceration, which remained asymptomatic and healed without specific treatment. CONCLUSIONS: This protocol was effective in the diagnosis of OCH through two physical examination maneuvers with low-cost and easy implementation, the diascopy and the HLMAC. The low concentration associated with a reduced amount of the sclerosing agent was responsible for the excellent results in the cases treated.

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.

[Infantile hemangiomas]. / Hemangiomas infantiles.

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.

Intratesticular kaposiform haemangioendothelioma in adults: a report of two cases.

Kaposiform haemangioendothelioma is a very rare vascular tumour of intermediate (borderline) malignancy, typically occurring in the skin and soft tissues of the extremities in infants and children. We report two morphologically and immunophenotypically classical cases occurring in the testicular parenchyma of old adults, review the literature on vascular tumours of the testis and discuss the differential diagnosis of these unusual cases.

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

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

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.

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.

Hemangioma capilar em língua: relato de caso / Capillary hemangioma of tongue: a case report

É reportado um caso de hemangioma capilar em um paciente leucoderma de 32 anos, com queixa de nódulo assintomático em dorso lingual anterior. O paciente relatou início há aproximadamente cinco meses após queimadura por alimento quente. Ao exame físico intrabucal se constatou lesão nodular arredondada medindo em torno de 2,5 cm em seu maior diâmetro, superfície ulcerada, coloração avermelhada e consistência firme à palpação. Foi estabelecido diagnóstico diferencial de fibroma, hemangioma capilar ulcerado, granuloma piogênico e hiperplasia fibrosa inflamatória. Foi realizada biópsia excisional, identificando-se ao exame histopatológico inúmeros capilares, revestidos por camada única de células endoteliais, sustentadas por um estroma de tecido conjuntivo, definindo o diagnóstico como hemangioma capilar. Nos controles pós-operatórios realizados desde a primeira semana até dois meses, foi observada cicatrização favorável da área operada, sem indícios de recidiva.

Actualización en el diagnóstico y tratamiento de los hemangiomas / Update on diagnosis and treatment of hemangiomas

Enfatizar en la correcta clasificación es aún una alta prioridad en la literatura ya persiste una gran desinformación al respecto. Nuevos progresos en la ciencia han permitido una mejor comprensión de estas lesiones. El avance en el diagnóstico precoz y la experiencia terapéutica han mejorado nuestra habilidad para tratar lesiones extensas y mejorar la calidad de vida de los pacientes.

Emphasis on correct classification is still a high priority in the literature and yet there remains a great deal of misinformation. Many new developments in the basic science of these lesions are allowing better understanding of why these lesions occur while improving our management in these patients. Advances in early diagnosis and therapeutic experience have improved our ability to treat extensive lesions and also improve patients' quality of life.

[Capillary hemangioma of the spinal cord: case report]. / Hemangioma capilar da medula: relato de caso.

We report a rare case of spinal cord capillary hemangioma in a 79-year-old woman, presented with paraparesia that had progressed within 8 months. Radiologically, the lesion resemble other vascular spinal cord tumors. The patient underwent surgery and the outcome was good. Histologically, the lesion resembled capillary hemangioma of skin or soft tissue, composed of lobules of small capillaries with associated feeding vessels, all enveloped by a delicate fibrous capsule. A review of the published cases in the literature is provided as well as a discussion of the clinical, radiological and histological aspects of the lesion and the differential diagnosis. Knowledge of its existence may avoid misdiagnosis of this benign lesion.