Intravascular Endothelial Hyperplasia of the Foot.

Intravascular endothelial hyperplasia is a benign soft tissue mass rarely reported in the foot. Advanced imaging and confirming a benign diagnosis are critical for any soft tissue mass. This paper identifies 2 patients that developed intravascular endothelial hyperplasia tumors which required surgical excision. A 17-year-old male patient presented to clinic complaining of a painful bump to the arch of his right foot which he related to an injury 9 months prior. Magnetic resonance imaging of the right foot revealed a mass within the plantar subcutaneous fat that was serpiginous in nature similar to adjacent branching vessels favoring a low-flow vascular malformation. A 38-year-old female with Multiple Sclerosis presented with complaints of persistent symptoms of pain to the 1st interspace, difficult ambulation and neuritis. Ultrasound and MRI observed solid, multilobulated mass, with internal vascular malformation, MRI describing intrinsic involvement along the abductor musculature and flexor tendons. Both lesions were surgically excised and sent for pathology. Pathology report indicated a diagnosis of intravascular papillary endothelial hyperplasia or Masson's tumor in both cases. Pathology diagnosis of intravascular papillary endothelial hyperplasia is generally good with wide resection leading to low recurrence rates. Both patients in the current study have progressed postoperatively with resolution of symptoms and without recurrence.

Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature.

Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan-guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.

Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location.

Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Immunohistochemically, the tumor is positive for CD31, CD34 and negative for D2-40. Herein we present a rare case of intraoral Kaposiform hemangioendothelioma in a 10-year-old boy.

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.

[Primary cardiac hemangioendothelioma: early diagnosis and surgical resection]. / Hemangioendotelioma cardiaco primario: diagnóstico y resección quirúrgica temprana.

BACKGROUND: The term hemangioendothelioma encompasses all tumors that derive from the endothelium of blood vessels. It has an uncertain prognosis, and it is always considered as a low-grade malignancy. CLINICAL CASE: Male, 23, who was admitted to the emergency room with chest pain, intermittent paroxysmal nocturnal dyspnea and malaise. He denied having a history of degenerative diseases, and had a weight loss of 6 kg in two months. Chest X-rays suggested pericardial effusion. Patient presented a tendency to hypotension tamponade, which was solved with subxiphoid puncture, and drain 800 mL of liquid from ancient hematological parameters. He presented a tumor in the right atrium of 8x4 cm, attached to the anterior wall of the atrium without involucre of interatrial septum. When patient underwent surgery, it was identified a tumor lesion in the anterior wall of right appendage. It was done the resection of the tumor and of 70% of the right atrial appendage. The latter was replaced with bovine pericardium. Patient showed good outcome; it was discharged after 10 days of follow-up surgery for six months in the outpatient clinic. CONCLUSION: The hemangioendothelioma is a vascular tumor of unpredictable behavior, and whose origin rarely comes from the heart. A timely detection, and a radical surgical resection is, so far, the more acceptable management, given the worldwide little experience for handling this type of tumor. Our experience suggests an aggressive clinical approach and surgical removal within the first hours of the suspected diagnosis for the greatest chance of complete resection and reducing the risk of recurrence.

Introducción: el hemangioendotelioma engloba todos los tumores que derivan del endotelio de los vasos sanguíneos. Es de pronóstico incierto y siempre se considera como una lesión maligna de bajo grado. Caso clínico: paciente masculino de 23 años que ingresó a urgencias con dolor retroesternal intermitente, disnea paroxística nocturna y ataque al estado general. Negó antecedentes crónico-degenerativos y tuvo pérdida ponderal de 6 kg en dos meses. Las pruebas sugirieron derrame pericárdico. Presentó tendencia a la hipotensión por tamponade, resuelta con punción subxifoidea, y drenaje de 800 mL de líquido de características hemáticas antiguas. El paciente presentó un tumor de la aurícula derecha de 8 x 4 cm, dependiente de la pared anterior de la aurícula, sin involucro de septum interatrial. Al someter al paciente a cirugía, se identificó lesión tumoral en la pared anterior de orejuela derecha. Se hizo resección del tumor y del 70 % de la orejuela derecha, sustituida con parche de pericardio bovino. El paciente presentó buena evolución; egresó a los 10 días de la cirugía con seguimiento por seis meses en la consulta externa. Conclusión: estamos ante un tumor vascular oncológicamente impredecible y que rara vez se origina en el corazón. Una detección oportuna y la resección quirúrgica radical es el manejo más aceptado ante la poca experiencia que hay a nivel mundial para lidiar con esta entidad. Sugerimos un abordaje clínico agresivo y la extirpación quirúrgica dentro de las primeras horas de la sospecha diagnóstica para obtener la mayor posibilidad de resección total y la reducción del riesgo de recidiva.

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.

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.

[Reversion of hypophosphatemia after the excision of a composite hemangioendothelioma in the great toe]. / Hipofosfatemia revertida al extirpar hemangioendotelioma compuesto del dedo mayor del pie.

We report a 43 years old male admitted to the hospital for progressive lumbar pain, lasting 20 years, that caused severe disability. On admission the patient had a serum phosphate of 2 mg/dl, an urine phosphate excretion over 300 mg/dl and serum alkaline phosphatases over 750 U/L. Serum intact parathormone was normal and tubular maximum phosphorus/glomerular filtration was 0.7 mg/dl. Bone scintigraphy showed an increased radionuclide uptake in condro-costal joints. Bone densitometry showed femoral osteoporosis. A violet colored mass was detected in a great toe. It was removed and the pathological diagnosis was a composite hemangioendothelioma. After tumor excision, serum phosphate levels returned to normal values and symptoms disappeared within 15 days.

Hipofosfatemia revertida al extirpar hemangioendotelioma compuesto del dedo mayor del pie / Reversion of hypophosphatemia after the excision of a composite hemangioendothelioma in the great toe

We report a 43 years old male admitted to the hospital for progressive lumbar pain, lasting 20 years, that caused severe disability. On admission the patient had a serum phosphate of 2 mg/dl, an urine phosphate excretion over 300 mg/dl and serum alkaline phosphatases over 750 U/L. Serum intact parathormone was normal and tubular maximum phosphorus/glomerular filtration was 0.7 mg/dl. Bone scintigraphy showed an increased radionuclide uptake in condro-costal joints. Bone densitometry showed femoral osteoporosis. A violet colored mass was detected in a great toe. It was removed and the pathological diagnosis was a composite hemangioendothelioma. After tumor excision, serum phosphate levels returned to normal values and symptoms disappeared within 15 days.

Intravascular papillary endothelial hyperplasia causing cauda equina compression: case report.

OBJECTIVE AND IMPORTANCE: Intravascular papillary endothelial hyperplasia (Masson's vegetant hemangioendothelioma) is a rare condition affecting the neuraxis. In the literature, only one case of this lesion involving the vertebral canal with spinal cord compression has been reported. We present a case of cauda equina compression due to this abnormality. CLINICAL PRESENTATION: A 17-year-old boy was admitted at our hospital with pain, numbness, paresis of the left lower extremity, and bladder dysfunction of approximately 1 month's duration. Computed tomography and magnetic resonance imaging of the spine revealed a tumor within the spinal canal at the T12-L1 level. INTERVENTION: The patient underwent a T12-L1 laminectomy. An epidural red nodular tumor was visualized and totally resected. The findings of the pathological examination were compatible with intravascular papillary endothelial hyperplasia. At follow-up examination 1 month after the operation, the patient had complete resolution of the pain, and the motor deficit and bladder dysfunction had improved significantly. CONCLUSION: This rare benign vascular lesion may be clinically and histopathologically mistaken for an angiosarcoma. Because the intravascular papillary endothelial hyperplasia can be cured by complete surgical resection, it is important to distinguish between these two lesions to avoid inappropriate aggressive treatment.

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.

[Hemangioendothelioma of the lip]. / Hemangioendotelioma do lábio.

Hemangioendothelioma is a relatively uncommon tumor of the oral cavity. This article reviews the literature on hemangioendothelioma and presents one additional case of this neoplasm found on the lip.

Hemangioendotelioma ósseo: relato de uma caso e revisäo de literatura / Bone hemangioendothelioma: report of a case and review of the literature

Os autores relatam um caso de hemangioendotelioma ósseo e tecem comentários sobre sua confusa nomenclatura, diagnóstico, evoluçäo e tratamento, comparando seu caso com os encontrados na literatura. Os autores lembram também a raridade de sua ocorrência