Hipertensão Arterial Pulmonar com Características de Envolvimento Venoso: Um Trabalho Investigativo / Pulmonary Arterial Hypertension with Features of Venous Involvement: A Detective's Task

Resumo A doença veno-oclusiva pulmonar (DVOP) e a hemangiomatose capilar pulmonar são tipos raros de substratos histopatológicos dentro do espectro da hipertensão arterial pulmonar (HAP) com prognóstico muito ruim. Caracterizam-se por um processo fibroproliferativo generalizado das veias e/ou capilares de pequeno calibre com preservação das veias maiores, resultando em um fenótipo de hipertensão pulmonar pré-capilar. A apresentação clínica é inespecífica e semelhante a outras etiologias de HAP. O diagnóstico definitivo é obtido por meio de análise histológica, embora a biópsia pulmonar não seja aconselhada devido ao maior risco de complicações. No entanto, alguns achados adicionais podem permitir um diagnóstico clínico presuntivo de DVOP, especialmente história de tabagismo, uso de drogas quimioterápicas, exposição a solventes orgânicos (particularmente tricloroetileno), baixa capacidade de difusão do monóxido de carbono (DLCO), dessaturação ao esforço e evidências de doença venosa sem doença cardíaca esquerda no exame de imagem, manifestada por uma tríade clássica de opacidades em vidro fosco, linhas septais, e linfadenopatias. O transplante pulmonar é o único tratamento eficaz e os pacientes devem ser encaminhados no momento do diagnóstico, devido à rápida progressão da doença e ao prognóstico ruim. Apresentamos o caso de um homem de 58 anos com HAP com características de envolvimento venoso/capilar em que a suspeita clínica, o pronto diagnóstico e o encaminhamento precoce para transplante pulmonar foram determinantes para um bom desfecho.

Abstract Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.

Giant mediastinal capillary hemangioma in a fetus: a case report / 中华围产医学杂志

We describe a rare case of fetal mediastinal capillary hemangioma presenting as pleural effusion and a huge pleural occupying lesion during late pregnancy. The patient was admitted at 36 +3 weeks of gestation, with a fetal chest occupying lesion for 11 days. Routine prenatal ultrasound and MRI indicated right pleural effusion and a huge chest occupying lesion in the fetus. The woman was administered oxytocin and delivered a live baby boy at 36 +5 weeks of gestation. The baby was diagnosed as mediastinal hemangioma by postnatal CT, angiography and 3D reconstruction and was discharged after oral propranolol treatment. However, he was readmitted one month after birth due to "pneumonia and tachypnea". After multidisciplinary consultation, the baby underwent a right-side thoracic mediastinal mass resection, and a mediastinal capillary hemangioma was confirmed by pathology. The child continued taking propranolol orally and received regular follow-up and rehabilitation after the operation up to 7 months old, by which time no obvious abnormalities were found.

Lobular capillary hemangioma in a patient with ankylosing spondylitis using adalimumab: an exuberant presentation

Abstract Lobular capillary hemangioma or pyogenic granuloma is a benign vascular tumor of the skin or mucous membranes. Most patients present a single lesion. It manifests clinically as an erythematous, friable, and fast-growing tumor. This report details a case with exuberant presentation in a patient with ankylosing spondylitis, using adalimumab. Factors triggering pyogenic granuloma are not well known. They may spontaneously regress, but most require treatment.

Hemangioma piógeno ótico: a propósito de un caso / Otical pyogenic hemangioma: about a case

Fundamento: el hemangioma piógeno o granuloma piógeno, incluye neoplasias que se caracterizan por un gran número de vasos sanguíneos normales o anormales que resultan difíciles de diferenciar de malformaciones o hematomas. Es una tumoración de tejidos blandos, excepcionalmente en el tejido óseo, siendo relativamente frecuente, resulta de un desarrollo reactivo focal de tejido fibrovascular con proliferación endotelial y se les relaciona con traumas, irritación, cambios hormonales, el embarazo. Objetivo: describir la localización infrecuente y la evolución de la enfermedad. Caso clínico: paciente blanco masculino antecedentes de notar una lesión de aspecto granulomatoso en cara interna del trago, por cuyo motivo se incindió con anterioridad y por el crecimiento rápido se decidió exceresis total y biopsia, se informó la presencia de un hemangioma piógeno. Conclusiones: el hemangioma o granuloma piógeno, es una tumoración benigna de inicio brusco y de curso rápido. La irritación local de los tejidos es la causa más frecuente y el tratamiento quirúrgico es la terapéutica adecuada para la curación de la enfermedad.

Background: pyogenic hemangioma or pyogenic granuloma includes neoplasms characterized by a large number of normal or abnormal blood vessels that are difficult to differentiate from malformations or hematomas. It is a soft tissue tumor, exceptionally in bone tissue, being relatively frequent, results from a focal reactive development of fibrovascular tissue with endothelial proliferation, and is associated with trauma, irritation, hormonal changes, pregnancy. Objective: to describe the infrequent location and the evolution of the disease. Clinical case: a white male patient had a history of a granulomatous lesion on the internal side of the swallow, for which reason it was previously incinerated and for rapid growth it was decided total exceresis and biopsy reporting the presence of a pyogenic hemangioma. Conclusions: pyogenic hemangioma or granuloma is a benign tumor of rapid onset and development. Local tissue irritation is the most common cause and surgical treatment is the appropriate therapy for the cure of the disease.

Cutaneous New World Leishmaniasis on a Port-wine stain birthmark

We present an interesting case report of two sarcoid-like lesions on a port-wine stain (PWS) birthmark in a Brazilian patient which on investigation proved to be cutaneous leishmaniasis.

Clinical and pathological analysis of lobular capillary hemangioma / 中国医师进修杂志

Objective To explore the chnical manifestation,pathological features and characteristics of lobular capillary hemangioma (LCH),so as to raise awareness.Methods Sixty-nine cases of LCH were studied by routine histopathologic and immunohistochemical examinations.Results The age range of the 69 patients including 34 males and 35 females with LCH was 4-80 years,with an average age of 42 years.The lesions of 50 cases were located in head and neck region,and the other 19 cases were in other parts of the body,with 1 case in the left main bronchus and intracalvarium.The average diameter of the tumors was about 1 cm,and parts of the tumors showed polypoid masses.Most of the tumors were in the skin and mucosa.Granulomatous lesions showed the proliferation of thin-walled and thick-walled capillaries in a lobular pattern.The vascular interstitium was edematous and was infiltrated by the inflammatory cells.Immunohistochemical examination showed CD34 positive in the endothelial cells and smooth muscle actin positive in the peri-vascular spindle cells.Conclusions Studies of LCH show the obvious features on age,gender and diseased location,and very few have a relapse.Its pathogenesis is still unclear.LCH is rarely found in the trachea,and is more rarely in the central nervous system.So far,there is no case report to show LCH occurs both in the bronchus and intracalvarium.In the special case,the early lesion is found in the left main bronchus.Although the patient is treated,LCH still recurs for many times in a short period.Finally the tumor is also found in the intracalvarium.Therefore,it's critical to alert whether LCH also occurs in the important organs at the early stage.Complete surgical resection and / or adjuvant radiotherapy can avoid delaying the treatment and the subsequent serious outcomes.

A Case of Capillary Hemangioma of Lingular Segmental Bronchus in Adult / 결핵및호흡기질환

Capillary hemangioma of the tracheobronchial tree is an extremely rare benign tumor in adults, especially those located in the bronchus. Characteristics and treatment of capillary hemangiomas of adult tracheobronchial trees have not been well known. We present a 61-year-old man with hemoptysis, which was caused by a small tiny nodule in the left lingular segmental bronchus. The nodule was removed by a forcep biopsy, via flexible bronchoscopy, and it was revealed to be capillary hemangioma. A small isolated endobronchial capillary hemangioma can be treated with excisional forcep biopsy, but a risk of massive bleeding should not be overlooked.

Hemangiomas periorbitários: necessidade de conduta ativa - relato de dois casos / Periorbital hemangiomas: the need for active management - report of two cases

Hemangioma é o tumor mais comum da infância, frequentemente situado na cabeça e pescoço. A órbita é frequentemente acometida e indica intervenção precoce e agressiva para evitar sérias complicações visuais. Reportam-se dois casos, nos quais há impedimento da visão no primeiro e, no segundo, um hemangioma profundo acomete áreas adjacentes, confirmado por exame radiológico. Demonstra-se sucesso terapêutico após corticoterapia sistêmica agressiva, evitando sequelas visuais permanentes, além do resultado estético satisfatório. O tratamento de escolha é o corticosteroide oral, devendo ser conduta individualizada e com bom seguimento clínico dos possíveis efeitos adversos.

Hemangioma is the most common tumor of childhood and is commonly located on the head or neck. The orbit is often affected and early and aggressive intervention is required to prevent serious visual complications. This paper reports on two cases. In the first case, the patient's vision was impaired, while in the second case a deep hemangioma affecting adjacent areas was confirmed radiologically. Treatment with aggressive systemic corticotherapy was successful, thus avoiding permanent damage to the patients' vision. Furthermore, esthetic outcome was satisfactory. The treatment of choice is oral corticosteroids and management should be individualized and should include careful follow-up to monitor possible adverse effects.

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.

A Case of Pulmonary Capillary Hemangiomatosis-Like Lesion Followed for Seven Years / 결핵및호흡기질환

Pulmonary capillary hemangiomatosis (PCH) is a rare disease of unknown etiology that is characterized by nodules composed of infiltrating capillary blood vessels. Herein, we describe a case of a PCH-like lesion that was detected by chest computed tomography. Transthoracic needle aspiration resulted in life-threatening hemorrhage. The patient was followed for seven years. He remained in good health and a follow up image showed little interval change.

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.

A Case of Tracheal Capillary Hemangioma in an Adult / 결핵및호흡기질환

Capillary hemangioma of the trachea is an extremely rare benign tumor in adults. We present the case of a 28-year-old woman with hemoptysis owing to a tumor of the proximal trachea. The tumor was removed by rigid bronchoscopy. The resulting specimens showed capillary hemangioma without any signs of malignant transformation. This presentation is the first case in Korea. Although tracheal capillary hemangioma is a rare disease, we should consider it as a possible cause of hemoptysis in the young adult.

Pingyangmycin Treating Infantile Proliferating Capillary Hemangiomas in 30 Children / 实用儿科临床杂志

Objective To explore the microcosmic mechanism of pingyangmycin treating infantile proliferating capillary hemangiomas so as to find the optimal method for hemangiomas′ treatment.Methods Sixty samples of infantile proliferating hemangiomas were divided into control group(30 cases,aged from 2 days to 6 months) and experimental group(30 cases,aged from 2 to 6 months).Pingyangmycin was made into emulsion and smeared on the surfaces of the leision in experimental group with 3 times every day as well as only matrix in control group.The specimens were resected on d7,then made into pathological slices and electron microscope slices in order to observe the cells microcosmic structure changes and ultrastructure changes.Furthermore,the apoptotic indexes of two groups were detected by the molecular biology method(TUNEL test).Results The number of apoptotic cells were lower in control group(apoptotic index 18.87?13.67)but higher apparently in experimental group(apoptotic index 29.52?15.33).The difference between two groups was significant(t=2.842 P

Capillary Hemangioma of the Cauda Equina: Case Report

Capillary hemangioma of the cauda equina is extremely rare. We present the MR imaging and histologic findings of a case of this disease. The tumor was well demarcated, 0.9x1.3 cm in diameter and 1.2 cm in length. On the MR images, the tumor showed isointensity relative to the spinal cord on the T1-weighted images, hyperintensity on the T2-weighted images, and strong homogeneous enhancement on the contrast-enhanced T1-weighted images. Capillary hemangioma should be included in the differential diagnosis of a spinal tumor of the cauda equina.

Clinical features of 18 cases of juxtapapillary capillary hemangioma / 中华眼底病杂志

Objective To evaluate the clinical features of 18 cases of juxtapapillary capillary hemangioma. Methods In 18 cases of juxtapapillary capillary hemangioma, the clinical data of 19 eyes, the results of fundus fluorescein angiography (FFA) of 16 eyes, and the follow-up observation of 7 eyes were retrospectively analyzed. Results In 18 cases, the sex ratio of male and female is 1∶2 with the average age of 28.9 years. Peripheral retinal angioma was found in 3 cases (4 eyes). In 19 eyes, the center of hemangioma was located beyond the rim of optic, most of which was in inferotemporal quadrant of the optic (7/19) with the size of 1-2.5 disc diameter (DD). Most of the tumors were red in color (12/19). In the result of FFA of 16 eyes, wash-out at the late phase was found in 13 eyes. Decreased acuity was found in all 7 cases that were followed up more than 12 months (including 5 cases treated by laser photocoagulation). Retinal detachment didn′t occur in 6 cases without exudative retinal detachment after being followed-up for 54.5 months of the average term. Conclusions Most of the juxtapapillary capillary hemangioma are orange or red oval tumors. The center of hemangioma are located beyond the rim of the optic. The patients often complain gradually decreased visual acuity when they are in prime of their life with exudation and edema in different degree around the tumor. FFA is helpful for the diagnosis and differential diagnosis of this disease.

The clinical and pathological aspects of digestive tract bleeding caused by angiodeformity of the bile duct:report of 21 cases / 中国普通外科杂志

From 1989 to Apr. 1997, 21 patients were admitted for gastrointestinal bleeding caused by binary angiodeformity. Diagnosis was made in 16 out of 18 undergoing selective celiac angiography with the occuracy rate of 88. 9%. Endoscopy found the deformity in 6 out of 14 (42. 8%). Bleeding stopped in 9 of 11 treated conservatively. Surgery was successful in all 9 cases,and endoscopic cautery successfully stopped the bleeding in the remaining one. The authors came to the conclusion that selective celiac angiography was most useful for the diagnosis. Drug therapy and endoscopic cautery effect only temporary hematostasis.

Expression of p63 and p73 Protein in Capillary Hemangioma / 中华皮肤科杂志

Objective To investigate the relationship between the expression of p63, p73 proteins and the development of hemangioma. Methods The immunohistochemical technique and quantitative image analysis were used to detect the expression of p63 and p73 proteins in 40 cases of capillary hemangioma and 20 specimens of normal skin. Results The absorbance value (mean ? SD) of p63 and p73 expression in normal skin tissue, proliferative phase of hemangioma and involuting phase of hemangioma were 0.923 ? 0.191 and 0.953 ? 0.120, 8.271 ? 1.953 and 6.408 ? 2.151, 0.920 ? 0.187 and 1.073 ? 0.516, respectively. The expression of p63 and p73 in proliferative phase of hemangioma was significantly increased as compared with those in involuting phase of hemangioma and normal skin tissue (P 0.05). Conclusions It is suggested that p63 gene is not a tumor suppressor gene but an oncogene in hemangioma and may contribute to the proliferation of endothelial cell and be associated with angiogenesis, and p73 may play an important role in the proliferation of hemangioma.