Angiosarcoma gastrointestinal multifocal, con compromiso extenso de estómago, intestino delgado y colon. Reporte de un caso / Multifocal gastrointestinal angiosarcoma, with extensive gastric, small intestine and colon involvement. A case report

Introducción. El angiosarcoma es una neoplasia vascular originada a nivel del endotelio, de baja frecuencia, conocido por su agresividad y crecimiento acelerado. Alcanza solo el 1 al 2 % del total de los sarcomas. La presentación gastrointestinal es infrecuente y su incidencia es poco conocida debido al escaso reporte de casos en la literatura. Suele identificarse en etapas avanzadas debido a la dificultad del diagnóstico histopatológico por sus características morfológicas, siendo necesario aplicar tinciones especiales o estudio inmunohistoquímico. Caso clínico. Por su interés y singularidad, presentamos el caso de un paciente masculino de 54 años, quien consultó con hemorragia digestiva profusa, anemización y requerimiento de terapia transfusional. Resultados. Durante el proceso diagnóstico, en la endoscopia encontraron diversas lesiones multifocales que se extendían por gran parte del tracto gastrointestinal. El estudio histopatológico mostró angiosarcoma gastrointestinal. Discusión. El angiosarcoma del tracto gastrointestinal es extremadamente infrecuente, de difícil diagnóstico y bajas posibilidades de manejo curativo, con opciones terapéuticas limitadas, lo que configura un mal pronóstico a corto plazo

Introduction. Angiosarcoma is a vascular neoplasm originating from endothelial cells, known for its aggressiveness, accelerated growth and reduced frequency. Reach only 1 to 2% of total sarcomas. Gastrointestinal presentation is extremely rare, the true incidence is poorly known, due to the limited reports of this entity in the literature. It is usually identified in advanced stages in view of the difficulty of the histopathological diagnosis, attributable to its morphological characteristics, being necessary to apply special stains or immunohistochemical study. Case report. Due to their interest and uniqueness, we present the case of a 54-year-old male patient, who presented with a profuse gastrointestinal bleeding, anemia, and requirement for transfusion therapy. Results. The endoscopy detected several multifocal lesions that extended most of the gastrointestinal tract. The histopathological study showed gastrointestinal angiosarcoma. Discussion. Angiosarcoma of the gastrointestinal tract is extremely uncommon, difficult to diagnose and has low possibilities of curative management, with limited therapeutic options, which configures a poor prognosis in the short term

Diagnosis of pulmonary artery sarcoma and differential diagnosis of pulmonary embolism with double-detector spectral CT: a case report / 中国医师杂志

Objective:The differential diagnosis of pulmonary artery sarcoma (PAS) and pulmonary embolism (PE) by double-detector spectral computerized tomography (CT) provides a new way to improve the detection rate of PAS and reduce the misdiagnosis rate.Methods:In the way of retrospective study, the Philips Nebula Workstation (ISP) was used to reconstruct electron density map, iodine density map and spectral curve in the spectral CT plain scan and enhancement of the PAS patient. In the plain scan image, the low density areas of the ascending aorta and the right pulmonary trunk were selected to measure their electronic density values. In the chest enhancement image, the iodine density of PAS area and PE area were measured respectively, and the spectral curves of PSA area and PE area were compared.Results:The electron density of the ascending aorta and the right pulmonary trunk in the low density area of the PAS patient during the plain scan of spectral CT were 104.4% EDW (relative to the percentage of the electron density of water) and 102.2% EDW, respectively, which were lower than those in the normal ascending aorta area. The fusion image of mixed energy and electron density clearly reflected the scope of the lesion. The iodine density in PAS area was 1.89 mg/ml, and the iodine density in PE area was 0.03 mg/ml during the enhancement phase. The iodine uptake in PAS area was significantly higher than that in PE area. The slope of PAS region was 2.08, and the slope of PE region was -1.86. The slopes of the two spectral curves were inconsistent.Conclusions:The electronic density, iodine density and spectral curve measured by double-detector spectral CT may provide powerful imaging basis for the diagnosis of PAS and the differentiation of PAS from PE, which is helpful for the early diagnosis of the lesions, and also provide basis for the biopsy location of the mixed lesions of PAS and PE.

Angiosarcoma óseo en el humero de un paciente pediátrico, un reto diagnóstico: reporte de caso / Bone angiosarcoma in the humerus of a pediatric patient, a whole diagnostic challenge: Case report

Resumen El angiosarcoma óseo es una neoplasia maligna rara, agresiva y de mal pronóstico, que es inusual en niños. Esta entidad afecta preferentemente a los huesos tubulares y se puede presentar como lesión solitaria o multicéntrica. Presentamos el caso de una niña de 7 años, sin antecedentes médico quirúrgicos, quien consultó al Instituto Nacional de Cancerología - Colombia, por presentar una lesión tumoral que comprometía la totalidad del húmero, destruyéndolo y deformando el brazo. Para orientar el diagnóstico de la lesión, se realizó un estudio histopatológico minucioso y marcadores de inmunohistoquímica, estos sugirieren una neoplasia vascular maligna. Por el extenso compromiso tumoral, la única opción terapéutica, fue la desarticulación escapulotorácica de la extremidad, seguida de manejo adyuvante con protocolo de quimioterapia. Durante los ciclos de quimioterapia, la paciente presentó lesiones metastásicas en pulmón, que fueron resecadas. En el momento, se encuentra sin lesiones tumorales y en seguimiento estricto.

Summary Angiosarcoma of the bone is a rare, aggressive and poor-prognosis malignancy, that is unusual in children. This entity preferentially affects the tubular bones, and can present as a solitary or multicentric lesion. We present the case of a 7-year-old girl, without medical surgical history, who consulted to the National Cancer Institute - Colombia, for presenting a tumor lesion that compromised the entire humerus, destroying it and deforming the arm. To guide the diagnosis of the lesion, a detailed histopathological study and immunohistochemical markers were performed, which suggested a malignant vascular neoplasm. Due to the extensive tumor involvement, the only therapeutic option was scapulothoracic disarticulation of the limb, followed by adjuvant management with a chemotherapy protocol. During the chemotherapy cycles, the patient presented metastatic lesions in the lung, which were resected. At the moment, she is without tumor lesions and under close follow-up.

Tumor filoides maligno con diferenciación angiosarcomatosa / Malignant phyllodes tumor with angiosarcomatous

Resumen Los tumores filoides (TF) malignos son infrecuentes. Poco se ha descrito en la literatura sobre la diferenciación angiosarcomatosa de un TF maligno (1). Presentamos el caso de una mujer de 25 años con TF maligno de alto grado con diferenciación angiosarcomatosa y compromiso axilar. Manejada con mastectomía radical modificada y adyuvancia con radioterapia (RT). Se hace una discusión del caso y revisión de la literatura.

Abstract Malignant phyllodes tumors (TF) are rare. Little has been described in the literature on angiosarcomatous differentiation of a malignant TF. We report the case of a 25 years old woman with a malignant phyllodes tumor of high grade with angiosarcomatous differentiation and axillary involvement. She was managed with modified radical mastectomy and adjuvant radiotherapy. A discussion of the case and review of the literature was made.

Rosetas em angiossarcoma cutâneo: uma nova pista dermatoscópica / Rosettes in cutaneous angiosarcoma: a new dermoscopic clue

O angiossarcoma cutâneo é um câncer raro, de mau prognóstico, que acomete principalmente idosos do sexo masculino. Está relacionado à exposição solar crônica, linfedema crônico e procedimentos com uso de radiação. Relatamos o caso de um homem de 62 anos com presença de tumoração violácea de crescimento progressivo na região temporal esquerda. A dermatoscopia demonstrou áreas eritematosas de diferentes tons, estruturas semelhantes à pseudo-aberturas foliculares e rosetas. A biópsia e a análise imunohistoquímica confirmaram o diagnóstico de angiossarcoma cutâneo. O paciente atualmente está em tratamento quimioterápico no serviço de oncologia com melhora significativa da lesão.

Cutaneous angiosarcoma is a rare cancer with a poor prognosis that affects mainly elderly men. It is related to chronic exposure to sunlight, chronic lymphedema, and procedures using radiation. We report a case of a 62-year-old man with a progressively growing violaceous tumor in the left temporal region. Dermoscopy showed erythematous areas of different shades, pseudo-follicular openings structures, and rosettes. The biopsy and the immunohistochemical study confirmed the diagnosis of cutaneous angiosarcoma. The patient is currently undergoing chemotherapy treatment in the oncology service with significant improvement of the lesion.

Angiossarcoma hepático primário: uma trágica evolução ­ relato de caso e revisão de literatura / Primary hepatic angiosarcoma: a tragic evolution ­ case report and literature review / Angiosarcoma hepático primario: una evolución trágica ­ reporte de caso y revisión de la literatura

O angiossarcoma primário hepático é o tumor mesenquimal mais comum do fígado, representando cerca de 2% das neoplasias malignas primárias do órgão. Esse raro tumor tem sintomas inespecíficos, evolução agressiva e diagnóstico usualmente tardio, com prognóstico reservado mesmo quando tratado. Este trabalho consiste em um relato de caso de um paciente do sexo masculino, de 44 anos, que foi encaminhado à emergência do Hospital Geral Roberto Santos para investigação de quadro de anemia grave sintomática, síndrome consumptiva e hepatoesplenomegalia. Durante investigação laboratorial, evidenciou-se anemia com provável componente microangiopático associado à anemia da doença crônica. As sorologias para doenças virais e baciloscopia do escarro foram negativas. Foram detectados em exames de imagem dois nódulos hepáticos de grandes dimensões, adenomegalias retroperitonais, esplenomegalia de grande monta, volumoso derrame pleural à direita, além de alterações do esqueleto axial e apendicular. Evoluiu com síndrome da lise tumoral após tratamento clínico com corticoterapia por suspeita de linfoma, com óbito. A biópsia guiada por uma tomografia realizada previamente teve como conclusão perfil imuno-histoquímico compatível com angiossarcoma hepático. O angiossarcoma é um raro tumor, de difícil diagnóstico e tratamento, com evolução agressiva e achados clínico-laboratoriais pouco elucidativos, devendo a hipótese desta doença ser considerada no diagnóstico diferencial das neoplasias hepáticas. As opções terapêuticas são limitadas. Relatos de casos como este são de suma importância para o aumento do grau de suspeição clínica e um diagnóstico mais precoce dessa entidade de costumeira evolução catastrófica.

Primary hepatic angiosarcoma is the most common mesenchymal tumor of the liver, representing about 2% of primary hepatic malignancies. This rare tumor has nonspecific symptoms, delayed diagnosis, and aggressive evolution, with a poor prognosis even when treated. This study reports the case of a 44-year-old male patient referred to the emergency department of the Hospital Geral Roberto Santos with symptomatic severe anemia, consumptive syndrome, and hepatosplenomegaly. Laboratory investigation indicated anemia with a probable microangiopathic component and chronic disease anemia. Serology tests for viral diseases returned negative results, as well as sputum smear microscopy for tuberculosis. Imaging exams revealed two large hepatic nodules, retroperitoneal adenomegaly, large splenomegaly, large pleural effusion in the right lung, and bone involvement. After clinical treatment with corticosteroids for suspected lymphoma, the patient evolved with tumor lysis syndrome and died. Tomography-guided liver biopsy was previously performed, indicating an immunohistochemical profile compatible with hepatic angiosarcoma ­ a rare tumor of difficult diagnosis and treatments due to its aggressive evolution and poor clinical and laboratory findings. Considering the nonspecificity of imaging exams, this disease should be considered in the differential diagnosis of liver neoplasms investigation. Case reports such as the one described in this study are important for increasing the degree of clinical suspicion and earlier diagnosis of this malignancy.

El angiosarcoma hepático primario es el tumor mesenquimatoso del hígado más común y representa el 2% de las neoplasias malignas primarias del hígado. Este raro tumor presenta una sintomatología inespecífica, diagnóstico tardío y evolución agresiva, con mal pronóstico incluso en tratamiento. Este es un reporte de caso de un hombre de 44 años de edad, que fue remitido al servicio de urgencias del Hospital Geral Roberto Santos para investigar anemia severa sintomática, síndrome de consunción y hepatoesplenomegalia. Durante la investigación de laboratorio, se evidenció anemia con un probable componente microangiopático asociado a anemia por enfermedad crónica. La serología para enfermedades virales resultó negativa, así como la microscopía de frotis de esputo para tuberculosis. Las imágenes revelaron dos grandes nódulos hepáticos, adenomegalia retroperitoneal, gran esplenomegalia, gran derrame pleural en el pulmón derecho, así como afectación del esqueleto axial y apendicular. El paciente evolucionó con síndrome de lisis tumoral tras el tratamiento clínico con corticoides por sospecha de linfoma, y no se resistió. Previamente se realizó biopsia hepática guiada por tomografía con perfil inmunohistoquímico compatible con angiosarcoma hepático. El angiosarcoma es un tumor raro, de difícil diagnóstico y tratamiento por su evolución agresiva y deficientes hallazgos clínicos y de laboratorio. Los exámenes por imágenes son inespecíficos y la posibilidad de esta enfermedad debe considerarse en el diagnóstico diferencial de la investigación de neoplasias hepáticas. Las opciones terapéuticas son limitadas. Reportes de casos como este son importantes para incrementar el grado de sospecha clínica y el diagnóstico precoz de este tipo de evolución catastrófica habitual.

Diagnosis and treatment of primary splenic angiosarcoma / 中华肝胆外科杂志

Objective:To analyze the clinical and imaging features, and treatment and curative effects of primary splenic angiosarcoma.Methods:A retrospective study was conducted on 6 patients with primary splenic angiosarcoma diagnosed histopathologically at the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019. The clinical characteristics, CT and ultrasound imaging findings, treatment methods and survival outcomes of these patients were analyzed.Results:There were 4 males and 2 females, with an average age of 52.6 years. These 6 patients with primary splenic angiosarcoma had no specific clinical manifestations, and the diagnoses were all confirmed histopathologically after surgery. CT examination of these 6 patients showed that the spleen volume was increased, with splenic space-occupying lesions. The lesions were single in 1 patient and multiple in 5 patients. Some lesions fused with each other. There were multiple intrahepatic metastases in 1 patient. Ultrasound in 3 patients showed enlarged solid hypoechoic spleens, and rich blood flow at the edges. Five patients underwent splenectomy with complete removal of tumors. For the patient with multiple lesions in the spleen and multiple space-occupying lesions in the liver who could not be treated radically, only splenectomy was done. This patient developed significantly more liver lesions 2 months after surgery, and survived for 3 months. Two patients who underwent postoperative adjuvant chemotherapy relapsed at 9 months and 13 months after surgery. They survived for 16 months and 19 months, respectively. A patient who received postoperative adjuvant chemotherapy survived for 27 months without any obvious signs of recurrence. A patient who underwent targeted therapy relapsed after 10 months, and survived for 14 months after surgery. The remaining patient who underwent simple surgery relapsed 4 months after surgery and died 6 months later.Conclusions:Primary splenic angiosarcoma is a highly malignant tumor. In this study, it lacked specific clinical manifestations and imaging features and its diagnosis required histopathological examination. Surgical resection is the main treatment for this disease. Whether surgery combined with postoperative adjuvant therapy is a more effective treatment than surgery alone for splenic angiosarcoma requires more studies in the future to determine.

Angiossarcoma cutâneo e suas peculiaridades / Peculiarities of cutaneous angiosarcoma

O angiossarcoma cutâneo é um sarcoma raro de tecido mole com prognóstico ruim, tendo a incidência em torno de 2,0% entre os sarcomas. Esta entidade pode se apresentar de várias formas clínicas, quais sejam, como lesão com aspecto de local contundido, nódulo, placa violácea e áreas hemorrágicas infiltrativas planas. Relatamos um caso de um homem leucoderma de 80 anos, cuja história se iniciou há mais de um ano com o surgimento de lesão nodular, rugosa e escura em terço superior da orelha direita. O tratamento do angiossarcoma cutâneo é multidisciplinar, sendo a cirurgia isolada ou associada à radioterapia (RT) usada para lesões iniciais e quimioterapia (QT) recomendada em lesões disseminadas. Os sarcomas cutâneos são tumores raros na rotina do cirurgião plástico, sendo crucial que, mediante suspeita, seja realizado tratamento e seguimento de maneira adequada.

Cutaneous angiosarcoma is a rare soft tissue sarcoma with a poor prognosis and an incidence of approximately 2.0%. This entity manifests as bruises, violaceous nodules and plaques, and diffuse hemorrhagic lesions with infiltrative growth. Here we report a case of an 80-year-old Caucasian man who presented with a nodular, hard, and dark lesion present in the upper third of the right ear for more than 1 year. The treatment of cutaneous angiosarcoma is multidisciplinary, including surgery alone or combined with radiotherapy for early lesions and chemotherapy for disseminated lesions. Cutaneous sarcomas are rare, and their appropriate treatment and follow-up are critical.

Pathological and immunohistochemical aspects of primary hepatobiliary neoplasms in cats / Aspectos patológicos e imuno-histoquímicos de neoplasias hepatobiliares primárias em gatos

Primary hepatobiliary neoplasms (PHN) are uncommon in cats, and originate in hepatocytes, intra- and extrahepatic bile ducts, mesenchymal cells, and cells of neuroendocrine origin. The aim of this study was to determine the frequency of PHN in cats diagnosed in the metropolitan region of Porto Alegre (RS), Brazil, for a period of 17 years, determining their epidemiological, anatomopathological and immunohistochemical aspects. Necropsy reports of 2.090 cats were analyzed, 125 were diagnosed with primary hepatobiliary diseases, of which 15 were cases of PHN, representing 12% of the specific hepatobiliary conditions and 0.7% of the necropsies. All PHN were malignant, of which 93.3% had epithelial origin and 6.7% presented mesenchymal origin. Cholangiocarcinoma was the most commonly diagnosed neoplasm, followed by hepatocellular carcinoma and hemangiosarcoma. In general, cats with no defined breed were the most affected. Concerning sex, 60% were females and 40% males. Age ranged from five to 18 years, with a mean age of 10.5 years (median of ten years). Grossly, cholangiocarcinoma and hemangiosarcoma were multinodular and hepatocellular carcinoma was massive. Microscopically, cholangiocarcinomas were arranged in acini and ducts, whereas hepatocellular carcinomas were arranged in solid sheets or trabeculae. On immunohistochemistry, cholangiocarcinomas, hepatocellular carcinomas, and hemangiosarcomas were positive for the antibodies CK 7, Hep Par-1, and vimentin and von Willebrand factor, respectively.(AU)

Neoplasias hepatobiliares primárias (NHP) são incomuns em gatos e se originam de hepatócitos, células dos ductos biliares intra e extra-hepáticos, células mesenquimais e ainda células de origem neuroendócrina. O objetivo do trabalho foi determinar a frequência das NHP em gatos diagnosticados na Região Metropolitana de Porto Alegre, no período de 17 anos, abordando seus aspectos epidemiológicos, anatomopatológicos e imuno-histoquímicos (IHQ). Foram analisados os laudos de necropsia de 2.090 gatos sendo que 125 foram diagnosticados com doenças hepatobiliares primárias, destes 15 foram casos de NHP, representando 12% das condições hepatobiliares específicas e 0,7% do total de necropsias. Todos os diagnósticos de NHP eram malignos, destes 93,3% apresentaram origem epitelial e 6,7% mesenquimal. Colangiocarcinoma foi a neoplasia mais diagnosticada, seguido do carcinoma hepatocelular e hemangiossarcoma. De uma maneira geral, os gatos sem raça definida foram os mais acometidos. Em relação ao sexo 60% eram fêmeas e 40% machos. A idade variou de cinco a 18 anos, com a idade média de 10,5 anos (mediana de 10 anos). Macroscopicamente o colangiocarcinoma e hemangiossarcoma eram multinodulares, e o carcinoma hepatocelular, maciço. À histologia, houve predomínio do arranjo acinar e ductal nos colangiocarcinomas e sólido, no carcinoma hepatocelular. Na IHQ os colangiocarcinomas foram reativos para CK 7, carcinoma hepatocelular para Hep Par-1 e hemangiossarcoma para vimentina e fator de von Willebrand.(AU)

Radiation-induced angiosarcoma: case report / Angiossarcoma induzido por radiação: relato de caso

ABSTRACT Angiosarcoma of the breast accounts for less than 1% of breast tumors. This tumor may be primary or secondary to previous radiation therapy and it is also named "radiogenic angiosarcoma of the breast", which is still a rare entity with a poor prognosis. So far, there are only 307 cases reported about these tumors in the literature. We present a case of a 73-year-old woman with a prior history of breast-conserving treatment of right breast cancer, exhibiting mild pinkish skin changes in the ipsilateral breast. Her mammography was consistent with benign alterations (BI-RADS 2). On incisional biopsy specimens, hematoxylin-eosin showed atypical vascular lesion and suggested immunohistochemisty for diagnostic elucidation. Resection of the lesions was performed and histology showed radiogenic angiosarcoma. The patient underwent simple mastectomy. Immunohistochemistry was positive for antigens related to CD31 and CD34, and C-MYC oncogene amplification, confirming the diagnosis of angiosarcoma induced by breast irradiation. A delayed diagnosis is an important concern. Initial skin changes in radiogenic angiosarcoma are subtle, therefore, these alterations may be confused with other benign skin conditions such as telangiectasia. We highlight this case clinical aspects with the intention of alerting to the possibility of angiosarcoma of the breast in patients with a previous history of adjuvant radiation therapy for breast cancer treatment. Sixteen months after the surgery the patient remains asymptomatic.

RESUMO Os angiossarcomas de mama representam menos de 1% dos tumores da mama e podem ser primários ou secundários à radioterapia prévia. Tais tumores são chamados de "angiossarcomas radiogênicos da mama" e representam uma entidade mais rara ainda e de prognóstico ruim. Atualmente, na literatura, são encontrados apenas 307 casos desses tumores. Relatamos o caso de uma mulher de 73 anos, com história prévia de tratamento conservador de câncer de mama direita, apresentando alteração rósea discreta em pele da mama homolateral. A mamografia demostrou resultado compatível com alterações benignas (BI-RADS 2). No material de biópsia incisional, a hematoxilina-eosina demonstrou lesão vascular atípica e sugeriu imuno-histoquímica para elucidação diagnóstica. A paciente foi submetida à ressecção de lesões, e a histologia demonstrou angiossarcoma radiogênico. Em seguida, mastectomia simples foi realizada. A imuno-histoquímica demonstrou positividade para os antígenos relacionados a CD31 e CD34, e a amplificação do oncogene C-MYC confirmou o diagnóstico de angiossarcoma induzido por radiação mamária. O atraso no diagnóstico constitui questão importante. Considerando que as alterações iniciais da pele do angiossarcoma radiogênico são sutis e podem ser confundidas com outras condições benignas da pele, como telangiectasias, neste relato destacamos os aspectos clínicos, no intuito de alertar sobre a possibilidade de angiossarcoma de mama em pacientes com história prévia de radioterapia adjuvante para tratamento de câncer de mama. A paciente permaneceu assintomática 16 meses após a cirurgia.

Descripción clínico-radiológica de un angiosarcoma cardiaco con metástasis cerebral que simula un quiste hidatídico / A radiological and clinical description of metastatic angiosarcoma simulating a hydatid cyst

RESUMEN Los angiosarcomas son sarcomas malignos que se originan en las células endoteliales vasculares. Su diagnóstico diferencial es muy amplio debido a su parecido con otras enfermedades, como las parasitarias, y usualmente es un diagnóstico por exclusión. La neurocisticercosis y la hidatidosis cerebral son parasitosis intestinales que pueden comprometer el sistema nervioso central y tienen mayor incidencia en los países suramericanos. El diagnóstico se establece a partir del perfil epidemiológico, el estudio parasitológico, la apariencia radiológica de las lesiones y el estudio de histopatología del espécimen. Se presenta el caso de una adolescente con factores de riesgo para parasitosis y neuroimágenes sugestivas de hidatidosis cerebral, cuyo diagnóstico definitivo fue angiosarcoma cardiaco metastásico.

ABSTRACT Angiosarcoma is the most malignant sarcoma originating in endothelial vascular cells. It has a wide differential diagnosis due to its similarities with other entities, such as parasitic diseases. More often, angiosarcoma is diagnosed by exclusion. Neurocysticercosis and hydatid disease, or echinococcosis, are parasitic infections that may involve the central nervous system and their incidence is higher in South American countries. Diagnosis is established based on the epidemiological profile, the parasitological examination, the radiological appearance of the lesions, and the histopathology analysis of specimens. We present the case of a female adolescent with parasitosis risk factors whose neuroimages suggested cerebral hydatid cysts and who was finally diagnosed with cardiac metastatic angiosarcoma.

Angiosarcoma de cuero cabelludo. revisión de la literatura actual a propósito de un caso / Angiosarcoma of the scalp. a case report and review of the literature

RESUMEN El angiosarcoma es un tumor de células neoplásicas de origen vascular, cuya presentación clínica es variada y su diagnóstico es tardío. Se presenta el caso de una paciente de 83 años, con múltiples úlceras dolorosas en cuero cabelludo, con diagnóstico incierto, por lo que se realiza biopsiaincisional de la lesión, detectándose un angiosarcoma. La inmunohistoquímica de la lesión fue positiva para CD34 y vimentina, negativo para S-100 y Melan-A. Este tumor se presenta en adultos mayores, siendo altamente invasivo y de mal pronóstico. Su diagnóstico requiere un alto índice de sospecha y a la fecha, no existe un tratamiento definido para esta patología.

SUMMARY Angiosarcoma is an aggressive malignant tumor of the vascular endothelial cells, and its clinical presentation is varied and of late diagnosis. The present case has a female patient, aged 83 who has multiple painful ulcers in scalp, whose lack of treatment response required a biopsy, where it was found an angiosarcoma. The immunohistochemistry of the lesion was positive for CD34 and vimentin, and negative for S-100 and Melan-A. This tumor develops in older people and is highly invasive withpoor prognosis. Its diagnosis requires high rates of suspicion and todate there is not a defined treatment for this pathology.

Angiosarcoma primario de la mama / Primary angiosarcoma of the breast

RESUMEN El angiosarcoma primario de mama es una neoplasia infrecuente y agresiva con una etiología desconocida. Presentamos el caso de una mujer de 33 años que acude a consulta por aumento de volumen en la mama izquierda no doloroso, ulcerado, se le realiza mastectomía total amplia, donde se diagnostica un angiosarcoma primario de la mama.

ABSTRACT Primary angiosarcoma of the breast is an infrequent and aggressive neoplasm with an unknown etiology. A case-report of a 33-year-old woman who came to the clinic because of an increase in volume in the left breast that was not painful and ulcerated is presented. She underwent a wide-total mastectomy, where a primary angiosarcoma of the breast was diagnosed.

Angiosarcoma cervical / Neck angiosarcoma

Introducción: Los angiosarcomas son tumoraciones malignas de rápido crecimiento que se originan en las células endoteliales. Representan un 2% de los sarcomas, y abar-can menos del 0.1% de las neoplasias de cabeza y cuello. Su etiología es multifactorial y el tratamiento va desde la resección quirúrgica hasta quimioterapia y radioterapia.Objetivo: Presentar un caso de angiosarcoma cervical en un paciente de la tercera edad. Diseño: Reporte de caso. Materiales y métodos: Se presenta el caso de una mujer de 84 años tratada en el Hospital Universitario Marqués de Valdecilla, Santander-Es-paña, quien consultó por masa asintomática en ángulo mandibular izquierdo de 3 días de evolución de aproximadamente 7-8 cm de diámetro. En la TC se evidencia masa en contacto con parótida izquierda, adenopatías bilaterales. Se realizó PAAF de la lesión con resultado compatible con angiosarcoma por lo cual se llevó a cabo la extirpación quirúrgica de la lesión. Resultados: El resultado de anatomía patológica fue angiosar-coma cervical. La paciente inicia radioterapia y durante esta se diagnóstica recidiva tumoral, falleciendo a los 13 meses del diagnóstico. Conclusiones: El angiosarcoma es un tumor maligno poco frecuente. Su diagnóstico incluye examen anatomo-patoló-gico, inmunohistoquímica, biología molecular y, técnicas de imagenología que ayudan a identificar la extensión tumoral y el planteamiento quirúrgico. El tratamiento se basa en la exéresis quirúrgica asociada a radioterapia y/o quimioterapia, dependiendo de la estadificación de la enfermedad y la resección con márgenes libres.

Introduction: Angiosarcomas are malignant neoplasias of rapid growth that are ori-ginated from endothelial cells. They represent 2% of all sarcomas, and of these, only 1­4% are localized in the upper aerodigestive mucosa, they account for less than 0.1% of all head and neck malignancies. The aetiology is multifactorial and its treatment includes surgery, radiotherapy and chemotherapy. Objective: To present a case of an elderly patient with neck angiosarcoma. Design: Case report. Mate-rials and Methods: We report a case of a 84 years-old woman who consulted to the Hospital Universitario Marqués de Valdecilla, Santander-Spain; because of an asymptomatic 7-8cm sized neck mass of 3 days of evolution in the left mandibular angle. On CT a mass in contact with the parotid gland and bilateral nodes were found. A fine needle aspiration biopsy was performed and its result was a mass compatible with angiosarcoma. Results: The final histology was neck angiosarcoma. During her treatment with radiotherapy, tumor recurrence was diagnosed. She died 13 months after the main diagnosis. Conclusions: Angiosarcoma is a rare malignant tumour. Diagnosis includes histological analysis, inmunochemistry, molecular biology, and images that will help to identify the tumor size and extension, as well as the surgical plan. Treatment includes surgery resection, radiotherapy and/or chemotherapy, de-pending on the disease stage and margins.

Atypical epithelioid hemangioendothelioma: a clinicopathological analysis of eight cases / 中华病理学杂志

Objective@#To study the clinicopathological features, diagnosis, and differential diagnosis of atypical epithelioid hemangioendothelioma (EHE).@*Methods@#Eight cases of atypical EHEs were collected from Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University) between 2010 and 2018. EnVision method and fluorescence in situ hybridization (FISH) were used to detect immunophenotype, WWTR1-CAMTA1 and TFE3 gene rearrangement, respectively.@*Results@#There were 4 males and 4 females, ranging from 42 to 59 years (median 47.5 years). The tumors located in soft tissue (3 cases), lung (3 cases), liver (1 case) and chest wall (1 case). One soft tissue EHE involved also adjacent fibula and pleural involvement was present in all three lung cases at the diagnosis. Regional lymph node metastases were present in two cases (one involving soft tissue tumor and one involving liver). Morphologically, the tumor cells were epithelioid with abundant eosinophilic cytoplasm, moderate to marked nuclear pleomorphism, irregular nuclear membrane, unevenly chromatin, and prominent nucleoli. The cells arranged in cords, small nests or solid pattern. The mitotic rate was 4.3 mitoses/2 mm2 on average (ranging 2 to 9). Tumor necrosis was seen in every case. Among all 8 cases, blister cells were found upon careful observation. Myxohyaline stroma was present in 6 cases. Immunohistochemically, tumor cells expressed CD31 (8/8), CD34 (7/8), ERG (8/8), CKpan (2/7), and CAMTA1 (4/6). None of the tested cases stained for TFE3 (0/6). WWTR1-CAMTA1 fusion gene by FISH was found in all tested 6 cases and TFE3 gene rearrangement was not detected in any. Available clinical follow-up was obtained in 7 cases and the intervals range from 6 to 55 months (average 19.6 months). Six patients had metastasis and 3 patients died of disease. One patient was alive with no evidence of disease.@*Conclusions@#Atypical EHE is a more aggressive tumor than classic EHE, with histological features including high nuclear grade, increased mitotic activity, the presence of solid growth pattern and tumor necrosis. The differential diagnoses include epithelioid angiosarcoma, carcinoma and epithelioid sarcoma.

Clinical features and treatment of ten patients with primary hepatic angiosarcoma / 中华肝胆外科杂志

Objective@#To investigate the clinical characteristics, treatment and curative effect of primary hepatic angiosarcoma.@*Methods@#A retrospective study was conducted on 10 patients with primary hepatic angiosarcoma who were treated in the Department of Hepatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University from April 2012 to July 2018. There were 6 males and 4 females, with an average age of 55.1 (range 39.0 to 71.0) years. The clinical features, imaging findings, treatment methods and survival time of the patients were analyzed.@*Results@#The clinical manifestations of 10 patients with primary hepatic angiosarcoma were non-specific and all were confirmed by pathology. Three cases had diffuse liver echo changes on ultrasound, and 7 cases had hypoechoic lesions with blurred borders. Space occupying lesions were found in 10 cases by CT. A total of five patients underwent partial hepatectomy, 1 case received chemotherapy, 1 case received transcatheter arterial chemoembolization (TACE), 2 case received molecular targeted therapy, and 1 case was untreated. From diagnosis to the last follow-up, the survival time of the 5 patients receiving surgical resection were 9 months, 15 months, 19 months, 21 months and 26 months. The survival time of the patient receiving chemotherapy was 7 months. The survival time of the patient receiving TACE was 3 months. Two patients were targeted for treatment, one survived for 32 months, another had been targeted for 15 months, and treatment was continuing. One patient survived for 1 month without treatment.@*Conclusions@#Primary hepatic angiosarcoma has a high degree of malignancy and a poor prognosis. No specific clinical manifestations and imaging findings, confirmed by pathological examination. Patients with partial hepatectomy have a better survival. Targeted therapy may be the most effective treatment for primary hepatic angiosarcoma in the future.

Clinical and radiologic characteristics as well as outcomes of patients with primary cardiac angiosarcoma / 中华心血管病杂志

Objective@#To analyze the clinical, radiologic characteristics, and outcome of consecutive patients with primary cardiac angiosarcoma.@*Methods@#The medical records of patients with primary cardiac angiosarcoma diagnosed through pathology at Peking Union Medical College Hospital between January 2001 and May 2018 were obtained. The results of echocardiography, coronary artery CT angiography (CTA), cardiac magnetic resonance (CMR), positron emission computed tomography (PET-CT), operation, postoperative treatment and prognosis of the patients were analyzed.@*Results@#A total of 16 patients were included, 9 were male, 7 were female, the median age was 42.5 years (31.7, 52.5). The interval from symptoms onset to diagnosis was 4.5 months (0.5-18.0 months). Eight patients were diagnosed at non-metastatic phase, while 8 patients were in metastatic phase. The main complaints were dyspnea, short breath, chest pain, hemoptysis, syncope, edema, fever, fatigue, and cough. Three patients (18.8%) had pericardial tamponade in the course of the disease. Echocardiography was performed on all 16 patients, cardiac mass was found in 12 patients, and pericardial effusion or pericardial thickening was observed in 4 patients. In 8 cases with results of coronary CTA, 5 cases presented signs of right atrium occupation, 1 case presented sign of right ventricle occupation, and 1 case presented isolated massive pericardial effusion. None abnormality was found in 1 case. Of the 7 patients who underwent CMR, 6 presented with right atrium mass occupation and 1 mediastinal mass. Four patients received PET-CT examination and results showed that all presented with hypermetabolic lesions: 2 in right atrium, 1 in pericardium, and 1 in mediastinum, and lung metastasis was found in 2 cases. Among the 16 patients, 13 received surgical treatment, 2 received adjuvant chemotherapy, and 1 received chemotherapy and radiotherapy after biopsy. The median overall survival was 3.0 months.@*Conclusion@#Cardiac angiosarcoma is a rare malignancy with poor prognosis, and echocardiography has only limited diagnostic value for angiosarcoma. CMR, CTA or PET-CT examinations could provide valuable clues for the diagnosis of this rare disease.

Clinical features and treatment of ten patients with primary hepatic angiosarcoma / 中华肝胆外科杂志

Objective To investigate the clinical characteristics,treatment and curative effect of primary hepatic angiosarcoma.Methods A retrospective study was conducted on 10 patients with primary hepatic angiosarcoma who were treated in the Department of Hepatobiliary Surgery,the First Affiliated Hospital of Zhengzhou University from April 2012 to July 2018.There were 6 males and 4 females,with an average age of 55.1(range 39.0 to 71.0)years.The clinical features,imaging findings,treatment methods and survival time of the patients were analyzed.Results The clinical manifestations of 10 patients with primary hepatic angiosarcoma were non-specific and all were confirmed by pathology.Three cases had diffuse liver echo changes on ultrasound,and 7 cases had hypoechoic lesions with blurred borders.Space occupying lesions were found in 10 cases by CT.A total of five patients underwent partial hepatectomy,1 case received chemotherapy,1 case received transcatheter arterial chemoembolization(TACE),2 case received molecular targeted therapy,and 1 case was untreated.From diagnosis to the last follow-up,the survival time of the 5 patients receiving surgical resection were 9 months,15 months,19 months,21 months and 26 months.The survival time of the patient receiving chemotherapy was 7 months.The survival time of the patient receiving TACE was 3 months.Two patients were targeted for treatment,one survived for 32 months,another had been targeted for 15 months,and treatment was continuing.One patient survived for 1 month without treat-ment.Conclusions Primary hepatic angiosarcoma has a high degree of malignancy and a poor prognosis.No specific clinical manifestations and imaging findings,confirmed by pathological examination.Patients with partial hepatectomy have a better survival.Targeted therapy may be the most effective treatment for primary hepatic angiosarcoma in the future.

Intravascular Epithelioid Angiosarcoma in the Abdominal Aorta Mimicking an Infected Aneurysm

Primary aortic angiosarcoma is very rare, and preoperative diagnosis is challenging with resultant poor prognosis. Angiosarcoma may mimic an infected aneurysm or a mural thrombus. Clinical suspicion of angiosarcoma is vital for an early diagnosis and proper surgical treatment, especially in cases with atypical rapid growth of an aortic abdominal aneurysm with a thrombotic mass. Herein, we report a case of angiosarcoma in the abdominal aorta mimicking an infected aneurysm and present computed tomography and positron emission tomography findings.