Papillary intralymphatic angioendothelioma: Dabska tumor

Abstract Papillary intralymphatic angioendothelioma (Dabska tumor) is a rarely metastasizing lymphatic vascular neoplasm that usually affects children and young adults. The majority of these cases occur in soft tissues of extremities, and to date less than 40 cases have been described. Despite the generally indolent evolution, can be locally invasive with the potential to metastasize. We describe a case of a young woman presenting with a plantar lesion, for 9 months and histological diagnosis of Dabska tumor. This neoplasm should be considered in the differential diagnosis of vascular dermatoses, allowing early diagnosis and treatment. Long-term follow-up should be performed.

Hiperplasia endotelial papilar intravascular en la cavidad oral: reporte de un caso / Intravascular papillary endothelial hyperplasia: report of a case

La hiperplasia endotelial papilar intravascular (HEPI) es una lesiónbenigna muy poco común en la cavidad oral. Fue descrita por primera vez por Masson en 1923 como hemangioendotelioma vegetante intravascular; más adelante, se le dieron diferentes nombres como angiomatosis intravascular, seudoangiosarcoma de Masson y, por último, hiperplasia endotelial papilífera intravascular. Los hallazgos en bocason poco comunes, y cuando se presentan, generalmente es en labio.Se expone el caso clínico de un paciente de 78 años de edad con una lesión asintomática bilobular en encía, que refi ere una evolución lenta y progresiva de 10 años. Se emitió un diagnóstico clínico presuntivo degranuloma periférico de células gigantes y se procedió a realizar biopsiaescisional, así como su envío a estudio histopatológico para confirmar eldiagnóstico; se emitió el diagnóstico de HEPI y se confirmó por medio de inmunohistoquímica. Esta lesión puede ser fácilmente confundida con neoplasias malignas vasculares (angiosarcoma, sarcoma de Kaposi),tanto clínica como histopatológicamente. El objetivo de este trabajo es describir mediante un caso clínico la importancia de un correcto diagnóstico a través de una adecuada exploración clínica, así comouna observación microscópica a conciencia y la comunicación interdisciplinaria entre el cirujano maxilofacial y el patólogo bucal, para no confundir esta entidad benigna con otro tipo de patologías que requieren tratamientos más agresivos e invasivos, sin ser adecuados para la HEPI.

Intravascular papillary endothelial hyperplasia (IPEH) is a rarebenign lesion in the oral cavity. It was fi rst described by Massonin 1923 as intravascular vegetating hemangio endothelioma, andlater received diff erent names, like «intravascular angiomatosis¼,«Masson’s pseudoangiosarcoma¼ and «intravascular papillaryendothelial hyperplasia¼; fi ndings in the mouth are rare, and when present, they are generally in the lip. The clinical case presented is a78-year-old patient with a bilobular asymptomatic lesion in gingivawith a slow and progressive 10-year evolution. A presumptive clinicaldiagnosis of peripheral giant-cell granuloma was issued, followed byan excisional biopsy and histopathology study to confi rm the diagnosis;it had been correctly diagnosed as IPEH, which was confi rmed byimmuno histochemistry. This lesion can be easily confused with vascular malignancies (angiosarcoma, Kaposi sarcoma) both clinically andhistologically. The intention of this work is, through a clinical case,to describe the importance of a correct diagnosis by a proper clinical examination, detailed microscopic observation and interdisciplinary communication between the surgeon and the pathologist, in order toavoid a misdiagnosis of this benign entity with other pathologies tha trequire more aggressive and invasive treatments, inadequate for IPEH.

Extra‑vascular type of oral intravascular papillary endothelialhyperplasia (Masson’s tumor) of lower lip: A case report and review of the literature.

Intravascular papillary endothelial hyperplasia (IPEH) is an unusual reactive lesion of vascular origin, which rarely occurs in the oral cavity. Pathogenetically, is it divided into true, mixed and extra‑vascular types. We report a case of extra‑vascular IPEH of the lower lip in 54‑year‑old female patient. Patient gives history of trauma 4 months back with lesion developing at the site to trauma. The lesion was 3 cm × 4 cm in size with soft to firm in consistency. Histologically, it is characterized by an exuberant papillary endothelial cell proliferation toward the lumen of an enlarged blood vessel from the area of an organizing thrombus. The lesion was surgically excised under local anesthesia. The patient was followed for 1‑year with no evidence of recurrence. This paper discusses the various aspects of IPEH of the oral cavity such as pathogenesis, clinical features, histopathology treatment, and prognosis.

Coexistent hepatic and pulmonary epitheloid hemangioendothelioma.

Epitheloid hemangioendothelioma (EHE) is a rare neoplasm of vascular origin known to arise in soft tissue, liver and lung. We describe a case of coexistent hepatic and pulmonary epitheloid hemangioendothelioma, proven on autopsy, and review the histological and radiological features of epitheloid hemangioendothelioma. The coexistence of hepatic with pulmonary EHE has been reported in only a few cases. Large confluent masses, peripheral location with capsular retraction, hypertrophy of uninvolved liver, invasion of portal and hepatic veins, enhancing margins and delayed enhancement and dense calcification are the typical features which provide a clue to diagnosis of hepatic EHE. In patients with both hepatic and pulmonary EHE it is difficult to say whether the tumor arose primarily in the lung or liver, or began simultaneously in both organs.

Infantile hepatic hemangioendothelioma: an 8-month old infant successfully treated with a corticosteroid

Infantile hemangioendothelioma is a rare benign vascular tumor of the liver. We report a case of hepatic hemangioendothelioma in an 8- month-old female infant who presented with hepatomegaly and respiratory distress, which was successfully treated with oral prednisolone for six months

Hemangioendotelioma hepático infantil: revisión bibliográfica / Infantile hepatic hemangioendothelioma: bibliographical revision

Se presenta el caso de un recién nacido que, a las pocas horas de vida, comienza con dificultad respiratoria y signos y síntomas de insuficiencia cardíaca congestiva. Se le realiza una ecografía abdominal, la cual presenta imágenes sugestivas de hemangioendotelioma hepático infantil. El diagnóstico es corroborado con otros estudios imagenológicos. Se efectúa una actualización bibliográfica del tema.

Symptomatic hepatic hemangioendothelioma in a newborn.

A case of hepatic hemangioendothelioma presenting as congestive cardiac failure in a neonate is reported. There was also evidence of platelet consumption. The case was managed successfully with oral prednisolone, resulting in improvement of symptoms and tumor regression over 3 months.

Endovascular papillary angioendothelioma in an elderly female.

Endovascular papillary angioendothelioma (EPA) is a rare vascular tumor of low grade malignancy, encountered primarily in children. However some cases have also been reported in adults. A sixty year old female with EPA is described here. She presented with a swelling in the neck. Total surgical excision was performed. Histomorphology revealed anastomosing lymphatic channels showing intraluminal papillary fronds with hyaline vascular cores and hobnailing of endothelial cells. A diagnosis of EPA was made based on this picture. This tumor is recurrent and nodal metastasis has been reported in some cases.

Hemangioendotelioma: tumor raro de mediastino / Hemangioendothelioma: a rare tumor of the mediastinum

Apresentou-se para atendimento um homem de 30 anos, branco, natural e procedente de São Paulo, com quadro de dor em hemitórax esquerdo, na região anterior e lateral, constante e de leve intensidade havia três meses, associado a dispnéia aos grandes esforços havia um mês. Durante a investigação foi visualizada em radiograma, tomografia e ressonância magnética de tórax grande tumoração em mediastino anterior e médio, com possível invasão dos vasos da base. Ele foi submetido à mediastinotomia paraesternal esquerda com biópsia da massa mediastinal, a qual complicou por sangramento intenso. Optou-se pela esternotomia mediana total e toracotomia ântero-lateral esquerda de urgência, com controle do sangramento e ressecção completa do tumor. Houve boa evolução, com alta hospitalar no nono dia pós-operatório. O exame anatomopatológico mostrou tratar-se de hemangioendotelioma de mediastino.

High Grade Hemangioendothelioma of the Temporal Bone in a Child: A Case Report

Hemangioendothelioma is a rare vascular tumor characterized by endothelial tumor cells and variable malignant behavior, and it's not common for this lesion to involve the bone. Although there are a few reports of cranial involvement by hemangioendothelioma, only rare cases arising in temporal bone have been published. We present the radiologic findings of a 7-year-old boy who had a high grade hemangioendothelioma involving the temporal bone with intracranial extension. Evidence of flow voids on MR images suggested a tumor of vascular origin, and the ill-defined margins, cortical destruction and intracranial extension on the CT and MR images were correlated with the tumor's high histologic grade.

Hemangioendotelioma hepático: aspectos radiológicos e evolução clínica de um caso / Hepatic hemangioendothelioma: radiological findings and clinical evolution of a case

Os tumores vasculares constituem um espectro de lesões que variam de benignas, os hemangiomas, a altamente malignas, os angiossarcomas, passando por um estágio intermediário de malignidade, no qual se incluem os hemangioendoteliomas. Relatamos um caso de hemangioendotelioma hepático de evolução benigna em um recém-nato do sexo feminino, e mediante revisão da literatura descrevemos os principais aspectos desta afecção

Vascular tumors encompass a spectrum of lesions, which may vary from benign hemangiomas to malignant angiosarcomas. Hemangioendotheliomas can be considered intermediary between these two types of lesions. We report a case of hepatic hemangioendothelioma in a newborn female and review the most important aspects of this disease.

Infantile hemangioendothelioma.

Primary hepatic tumors are uncommon in children and account for only three per cent of the tumors in children. Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present before six months. An unusual presentation and progression of infantile hemangioendothelioma is reported in a 19-month-old female child. The diagnosis was arrived at by radiological and histopathological examination. The patient underwent excision surgery, following which made an uneventful recovery. On follow-up at six months, patient was asymptomatic with no evidence of recurrence.

Hemangioendotelioma intravascular vegetante de Masson na regiäo lombar: relato de caso / Masson's vegetant intravascular hemangioendotelioma in the lumbar region: case report

Os autores relatam o caso de um paciente do sexo masculino, adulto jovem portador de hemangioendotelioma intravascular vegetante de Masson localizado na regiäo lombar apresentando-se clinicamente através de lombociatalgia esquerda. Patologia rara, sendo encontrado apenas um caso descrito na literatura com localizaçäo semelhante. Ressaltamos a importância do diagnóstico preciso para näo submeter os pacientes a tratamentos desnecessários.

Hemangioendotelioma maligno primário da tiróide: caso 05/95 (RG 763.043 - Hospital Säo Paulo/EPM) / Primary malignant hemangioendothelioma of the thyroid gland: case 05/95 (RG 763.043 - Hospital Säo Paulo/EPM)

Apresentamos um caso de paciente com nódulo na glândula tiróide com 10 meses de evoluçao, de crescimento rápido e indolor. A cintilografia tiroidiana evidenciou a existência de nódulo frio. O resultado da punçao aspirativa por agulha fina (PAAF) foi suspeito (classe III). O exame de congelaçao diagnosticou nódulo benigno. No exame anatomopatológico observou-se neoplasia de origem vascular. Foi realizado a impregnaçao pela prata (reticulina) e exames imunohistoquímicos. Os antígenos pesquisados foram a vimentina, a tiroglobulina, a calcitonina, o antígeno carcinoembrionário, a enolase específica, o marcador neuroendócrino, a cromogranina, a citoqueratina AEl AE3 e o fator VIII. O diagnóstico foi definido pela imunodemonstraçao de vimentina, compatível com hemangioendotelioma maligno. O paciente foi novamente operado para complementaçao da tiroidectomia, sendo encaminhado a quimioterapia pós operatória, a qual abandonou no 3( ciclo. Encontra-se no 4( ano de pós operatório livre de doença, com PCI negativa e níveis baixos de tiroglobulina.

Tumores vasculares do ângulo pontocerebelar: relato de 2 casos e revisäo da literatura / Vascular tumors of the cerebellopontine angle: report of 2 cases and review of the literature

Os autores relatam 2 pacientes portadores de tumores de origem vascular situados na regiao do ângulo pontocerebelar. O primeiro paciente era portador de hemangioma do cavo de Meckel com extensao para o ângulo ponto cerebelar, cuja sintomatologia predominante era hipoestesia em território do nervo trigêmeo. O segundo tratava-se de um paciente portador de hemangioendotelioma localizado próximo ao meato acústico interno, cujos sintomas relacionavam-se à disfunçao dos nervos coclear e facial. Ambos foram totalmente removidos através de acesso retromastóideo e técnica microneurocirúrgica. Os tumores vasculares sao raros nesta regiao e constituem importante diagnóstico diferencial com os tumores mais comuns como o neurinoma e o meningioma. É apresentada revisao da literatura sobre o tema, com ênfase maior nos aspectos clínicos e diagnósticos.

Hemangioendotelioma vegetante intravascular de localización orbitaria / Intravascular vegetant hemangio-endothelioma in right orbit

Se presenta un caso de hemangioendiotelioma vegetante intravascular en la órbita, en un paciente masculino de 54 años de edad. Es una rara neoformación no neoplásica, originada como parte de un proceso de reparación tisular intravenoso con formación de papilas intraluminales y canales vasculares. El tratamiento es exclusivamente quirúrgico y el diagnóstico diferencial debe hacerse con el hemangiopericitoma o el histiocitoma fibroso benigno angioblástico.