Analysis of clinicopathological features of 18 cases of hepatic angiosarcoma / 中华肝脏病杂志

Objective: To investigate the clinicopathological features, treatment, and prognosis of hepatic angiosarcoma. Methods: Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively. The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method. A Cox regression analysis was used to explore the survival-related risk factors. Results: There were 12 male and 6 female patients, with an average age of 57 (37 ~ 70) years. The tumor's average diameter was 8.40 (2.00 ~ 18.00) cm. Seven cases had multiple tumors, while two cases had large vessel tumor thrombuses. Microscopically, the tumor tissues were irregularly anastomosed, with vascular lacunar or solid bundle-like weaving, and the tissue morphology mimicked capillary hemangioma, cavernous hemangioma, or angioepithelioma, while tumor cells were spindle-shaped or epithelioid, lined with hobnails in the lumen, or formed papillary structures in the lumen. The proportion of highly, moderately, and poorly differentiated tumors was 4:8:6, with six cases having clear tumor boundaries, eight having microvascular tumor thrombi, and sixteen having blood lake formation. Different levels of expression of CD31, CD34, erythroblast transformation-specific related genes, and Fli-1 markers were demonstrated in all of the cases. Four cases had a P53 mutation, and six cases had Ki-67 > 10%. During the follow-up period of 0.23-114.20 months, the five-year recurrence-free survival rate and overall survival rate were 16.7% and 37.2%, respectively. Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival, while preoperative symptoms and Ki-67 > 10% were significant risk factors for overall survival. Conclusion: Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis. Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis. However, the complexity of angiosarcomas' histological and cytological conformations and the overlap of pathological features with benign vascular tumors, sarcomas, and carcinomas pose difficulties in the differential diagnosis. Thus, the only effective ways to prolong survival are early detection and radical surgical resection.

Single-center study of different treatment for advanced or unresectable angiosarcoma patients / 中华肿瘤杂志

Objective: To evaluate the efficacy and safety of different medical treatment in advanced or unresectable angiosarcoma. Methods: This study was a single-center retrospective clinical study. Fifty-five advanced or unresectable angiosarcoma patients treated in Sun-Yat Sen University Cancer Center from January 2005 to August 2020 were enrolled. There were 34 patients who received first-line doxorubicin-based chemotherapy (doxorubicin group), 12 patients received first-line doxorubicin or liposome doxorubicin plus paclitaxel or albumin bound paclitaxel chemotherapy (combination therapy group), and 4 patients received first-line paclitaxel-based treatment (paclitaxel group). There were 6 patients who received anti-angiogenesis targeted therapy, another 2 patients received anti-PD-1 antibody plus anti-angiogenesis targeted therapy. Targeted therapy and immunotherapy plus targeted therapy included 5 cases of first-line therapy and 3 cases of second-line therapy. The therapeutic effect was evaluated by RECIST 1.1 standard. The adverse reactions were evaluated by CTCAE4.0 standard. Kaplan-Meier survival analysis was evaluated with Log rank test. Cox proportional hazard model was used to analyze the influencing factors. Results: There were 18 patients achieved partial response (PR) in 34 patients in the doxorubicin group, median progression-free survival (mPFS) was 4.5 months, and median overall survival (mOS) was 15 months. Four patients achieved PR in 12 patients in the combination therapy group, mPFS and mOS were 4 months and 19 months. Two patients achieved PR in 4 patients in the paclitaxel group, mPFS and mOS were 3 months and 9 months. However, only 1 in 6 patients achieved PR for anti-angiogenesis targeted therapy, mPFS and mOS were 3 months and 16 months. Two patients who received anti-PD-1 immunotherapy combined with anti-angiogenesis targeted therapy acquired PR for 17 months and more than 16 months. Median PFS (7.5 months) were longer in those with primary liver, lung and spleen angiosarcoma than in those with other primary site (3.0 months, P=0.028). The mOS (20 months) was longer in females than that in males (12 months, P=0.045). Primary tumor site, sex, age and treatment were not independent prognostic factors for angiosarcoma patients (P>0.05). Grade 3-4 cardiac toxicity was found in 2 patients in the combination therapy group. Conclusions: Doxorubicin-based and paclitaxel-based chemotherapy are the most important treatment for advanced angiosarcoma. Potential efficacy for targeted therapy combined with anti-PD-1 immunotherapy are showed in some patients with long duration of response and moderate adverse event.

Diagnóstico Errôneo de Angiossarcoma Cardíaco na Era COVID-19 / Misdiagnosis of a Cardiac Angiosarcoma During the COVID-19 Pandemic

Resumo Os últimos meses de 2019 foram marcados pelo surgimento de uma nova pandemia, denominada "COVID-19". Desde então, essa infecção e suas complicações têm sido a prioridade de profissionais de saúde, com muitos sintomas atribuídos às suas apresentações precoces e tardias. Até o momento, outras doenças, mesmo em situações fatais, têm sido negligenciadas ou diagnosticadas incorretamente devido à atribuição dos sintomas do paciente à presença da infecção por COVID-19. Apresentamos aqui um caso de angiossarcoma cardíaco, em um menino que, cerca de 2 meses antes, havia sido infectado com COVID-19. Dado o histórico de infecção, a abordagem inicial foi o manejo da miopericardite pós-COVID-19. No entanto, o quadro do paciente piorou, exigindo reavaliação por multimodalidades com maior precisão. Por fim, o paciente foi diagnosticado com um tumor cardíaco. Este artigo procura enfatizar a importância da atenção a outras doenças e condições fatais na era COVID-19, com ênfase em evitar diagnósticos incorretos de outras doenças.

Abstract The final months of 2019 saw the emergence of a new pandemic termed "COVID-19". Since then, this infection and its complications have been the priority of healthcare providers, with many symptoms attributed to its early and late presentations. Thus far, other diseases, even fatal situations, have been overlooked or misdiagnosed due to the attribution of patient symptoms to the presence of COVID-19 infection. We herein present a case of cardiac angiosarcoma in a young boy who had previously become infected with COVID-19 about two months earlier. Given the history of infection, the initial approach was post-COVID-19 myopericarditis management. However, the patient's condition worsened, necessitating reevaluation via multimodalities with higher precision. Ultimately, the patient was diagnosed with a cardiac tumor. This article seeks to underscore the significance of taking heed of other diseases and fatal conditions during the COVID-19 pandemic with an emphasis on avoiding misdiagnosing other diseases.

Imaging Characteristics of Primary Cardiac Angiosarcoma / 罕见病研究

Primary cardiac angiosarcoma is a type of soft tissue sarcoma originating in vascular endothelial cells, without obvious gender differences in the incidence rate and specific early clinical manifestations, whilstpericardial effusion often found at the first presentation of most patients. Tumors are mostly located in the right atrium and pericardium. Echocardiography is the preferred examination method for diagnosing cardiac angiosarcoma and multimodal imaging is important in the diagnosis and differential diagnosis of benign and malignant cardiac mass. This article retrospectively analyzes the 25 cases of clinical manifestations and imaging features of primary cardiac angiosarcoma.

Angiosarcoma primario de mama. Reporte de caso y revisión de la literatura / Primary breast angiosarcoma. A case report and literature review

Introducción. El angiosarcoma primario de la mama es una neoplasia maligna derivada de las células endoteliales de los vasos sanguíneos, potencialmente agresiva independientemente de su grado histológico, por lo que su pronóstico es malo. Su diagnóstico prequirúrgico es difícil, ya que las características clínicas e imagenológicas son inespecíficas, y el diagnóstico definitivo únicamente se realiza por estudios de patología. Para su tratamiento generalmente se requiere de resección quirúrgica, radioterapia y, ocasionalmente, quimioterapia.Caso clínico. Paciente de 49 años sin antecedentes, que consultó por cuadro clínico de 5 meses de evolución de aparición y rápido crecimiento de masa en mama izquierda. Se realizaron estudios imagenológicos que reportaron lesión BIRADS 4a y diagnóstico histológico de lesión vascular con atipía, por lo cual fue llevada a mastectomía simple, con informe final de patología de angiosarcoma primario de mama; tuvo que ser reintervenida por márgenes positivos. Completó 33 ciclos de radioterapia y dos años después de la cirugía presentó cambios inflamatorios en la cicatriz quirúrgica, de la cual se tomó biopsia con reporte de lesión vascular atípica, por lo que fue operada nuevamente, con reporte histológico negativo para angiosarcoma residual. Actualmente la paciente está en seguimiento imagenológico, sin evidencia de recaída tumoral. Conclusión. Los angiosarcomas primarios de la mama son neoplasias raras y muy agresivas, independientemente de su grado histológico, por lo cual es importante hacer un diagnóstico histológico y tratamiento oncológico oportunos.

Introduction. Primary breast angiosarcoma is a malignant pathology derived from the endothelial cells of the blood vessels of the breast. They are potentially aggressive regardless of histological grade, reason why its prognosis is poor and treatment requires surgical resection plus radiation therapy and occasionally chemotherapy depending on the degree. Its pre-surgical diagnosis is difficult since the clinical and imaging characteristics are nonspecific, and the definitive diagnosis is only made by means of pathology studies. Clinical case. A 49-year-old patient was admitted to the breast surgery outpatient clinic due to clinical symptoms of 5 months of evolution consisting of the appearance of a painful mass in the left breast. Imaging of the lesion with ultrasound report BIRADS 4a and a tricot biopsy was taken with histological diagnosis of vascular lesion with atypia. It was decided to take the patient to a simple mastectomy, with a final report of breast angiosarcoma but with a margin compromised by a tumor for which she was reoperated. She received 33 cycles of radiotherapy and continued in follow-up for two years. During this period, the patient presented inflammatory changes in the surgical scar for which a punch biopsy was done with histological report of vascular atypical lesion. Surgical resection was performed with final report of negative pathology for residual angiosarcoma. Nowadays the patient continues imaging follow-up without evidence of a tumour relapse. Conclusion. Primary breast angiosarcomas are a rare malignant pathology, very aggressive regardless of its histological grade, for which it is important to perform a timely histological diagnosis and oncological treatment

Angiosarcoma de cavidad nasal: reporte de caso y revisión de literatura

Los tumores malignos de cavidad nasal y cavidades paranasales son poco frecuentes y sus síntomas de presentación suelen ser unilaterales e inespecíficos; éstos incluyen: epistaxis, rinorrea y obstrucción nasal. Encontramos una amplia variedad histológica de tumores, entre ellos tenemos el angiosarcoma nasal, que es considerado una rareza y el diagnóstico definitivo lo da la confirmación anatomopatológica complementado con estudios inmunohistoquímicos. Su modalidad de tratamiento incluye múltiples opciones, pero la cirugía radical temprana con márgenes negativos asociado a radioterapia adyuvante, ofrece el mejor pronóstico. Se presenta a un paciente adulto masculino con un angiosarcoma nasal izquierdo que fue detectado de forma precoz y se manejó con cirugía radical y radioterapia.

Malignant tumors of the nasal cavity and paranasal cavities are rare, while presenting symptoms are usually unilateral and nonspecific like, epistaxis, rhinorrhea, and nasal obstruction. There are many histological varieties of tumors, among them we have nasal angiosarcoma, which is considered a rarity pathology. The definitive diagnosis is given by biopsy confirmation complemented with immunohistochemical studies. Its treatment modality includes multiple options, but early radical surgery with negative margins associated with adjuvant radiotherapy offers the best prognosis. We present a male adult patient with a left nasal angiosarcoma that was detected early and managed with radical surgery and radiotherapy.

Lupus-like Syndrome and Cardiac Tamponade as Initial Presentation of a Primary Cardiac Malignant Tumor: Report of a Case

This is the case of a 38-year-old man with a history of two female relatives with Systemic Lupus Erythematosus and personal factors of high cardiovascular risk (gender, stress, smoking, chronic consumption of electronic cigarette). The patient presented tamponade due to pericardial effusion secondary to Lupus-like syndrome that preceded and accompanied at all times an angiosarcoma of the roof and anterior wall of the right atrium. His clinical evolution is described, as well as the surgical and pharmacological treatment in the Cardiovascular Care Unit of a third-level hospital and the possible causes of the poor response to management that led to his death.

Pulmonary carcinosarcoma with an aggressive heterologous angiosarcoma component: A case report

Pulmonary carcinosarcomas are rare biphasic lung tumors comprised of malignant epithelial and malignant mesenchymal components. The most common heterologous sarcomatous elements are osteosarcoma, rhabdomyosarcoma, and chondrosarcoma; a heterologous angiosarcoma component in a pulmonary carcinosarcoma is exceedingly rare. We report a case of a pulmonary carcinosarcoma containing adenocarcinoma, squamous cell carcinoma, undifferentiated malignant spindle cell, and heterologous angiosarcoma components. The patient, a 64-year-old woman, had initially presented to medical attention with hemoptysis. Although the tumor was thought to be confined to the lung at resection (pT3N0), she developed multiple metastatic foci within 3 weeks of lobectomy and required the evacuation of an intraparenchymal left occipital hematoma secondary to a hemorrhagic intra-axial focus of metastatic carcinosarcoma. She died 6 weeks after her primary lung resection from rapidly progressive metastatic disease. We hope the description and discussion provided herein will further the medical community's understanding of this rare malignancy.

Angiosarcoma radioinducido de mama: reporte de caso / Radioinduced angiosarcoma of the breast: Case report / Angiossarcoma radioinduzido de mama: reporte de caso

El angiosarcoma es un tumor vascular maligno poco frecuente. Constituye menos del 2% de todos los sarcomas. Existen varias formas clínicas, una es la producida después de radioterapia, en pacientes que fueron tratadas por un cáncer de mama, con cirugía conservadora y radioterapia. Se presenta como un sarcoma de alto grado, localizado en la piel o en el tejido subcutáneo y, ocasionalmente, el parénquima mamario. El único tratamiento curativo es la cirugía, con tendencia a la recurrencia y a hacer metástasis hematógena, el pronóstico es malo, con alta tasa de mortalidad.

Angiosarcoma is a rare malignant vascular tumor. It constitutes less than 2% of all sarcomas. There are several clinical forms; the one produced after radiation therapy is that associated with patients who were treated for breast cancer with conservative surgery and radiation therapy. It presents as a high-grade sarcoma located on the skin or the subcutaneous tissue and, occasionally, the breast parenchyma. The only curative treatment is surgery, with a tendency to recurrence and visceral hematogenous metastasis, with a poor prognosis and a high mortality rate.

El angiossarcoma é um tumor vascular maligno pouco frequente. Constitui menos de 2% de todos os sarcomas. Existem várias formas clínicas, uma é a produzida depois da radioterapia, em pacientes que foram tratadas por um câncer de mama, com cirurgia conservadora e radioterapia. Se apresenta como um sarcoma de alto grau, localizado na pele ou no tecido subcutâneo e, ocasionalmente, o parênquima mamário. O único tratamento curativo é a cirurgia, com tendência à recorrência e a fazer metástasehematogênica, o prognóstico é mau, com alta taxa de mortalidade.

Angiosarcoma cutáneo en una adolescente con Xeroderma Pigmentoso: Reporte de caso / Cutaneous angiosarcoma in an adolescent girl with Xeroderma Pigmentosum: A case report

Resumen El Xeroderma Pigmentoso (XP) es un trastorno poco frecuente, autosómico recesivo y caracterizado por una extrema sensibilidad a la radiación ultravioleta (RUV) de la luz solar, que produce una reparación defectuosa del daño del ADN y como consecuencia, marcada predisposición al desarrollo de cáncer de piel. Clínicamente, se manifiesta por fotosensibilidad, cambios cutáneos pigmentarios y envejecimiento prematuro de la piel. Este trastorno afecta aproximadamente 1 de cada 250.000 habitantes por año en Estados Unidos. Presentamos el caso de una paciente de 12 años con un Xeroderma Pigmentoso detectado a los 6 meses, quien desarrolló una lesión ulcerada en la vertiente nasal izquierda con reporte de patología de Angiosarcoma (AS) con CD31, EGR y CD34 positivos. La paciente fue tratada con resección amplia de la lesión y quimioterapia adyuvante con Plaquitaxel y Doxorrubicina sin radioterapia.

Abstract Xeroderma Pigmentosum is a rare autosomal recessive disorder characterized by extreme sensitivity to ultraviolet radiation (UVR) from sunlight that results in a defective repair of DNA damage and, as a consequence, a marked predisposition to the development of cancer of the skin. Its clinical manifestations are photosensitivity, pigmentary skin changes, and premature aging of the skin. This disorder affects approximately 1 in 250,000 individuals per year in the United States. We present the case of a 12-year-old patient with Xeroderma Pigmentosum detected at 6 months of age, who developed an ulcerated lesion on the left nasal slope with a pathology report of angiosarcoma (AS), which we positive for ERG, CD31, and CD34. The patient was treated with wide resection of the lesion and adjuvant chemotherapy with paclitaxel and doxorubicin without radiation therapy.

Advances in Molecular Targeting and Immunotherapy on Angiosarcomas / 肿瘤防治研究

Angiosarcoma(AS) is a rare malignant soft tissue sarcoma with poor differentiation and outcome originating from vascular or lymphatic endothelial cells. Currently, there is still no consensus on the treatment of AS. The chemoradiotherapy and surgical resection are the main treatment, but the curative effect is not good. With the rapid development of molecular biology, new molecular targets have been found gradually, which can benefit some patients. In addition, with the development of immunotherapy, the treatment of AS has been greatly enriched. This article expands on the progress of molecular targeting and immunotherapy of angiosarcoma and provides a reference for clinical colleagues.

Síndrome de Stewart-Treves no membro inferior com apresentação exuberante: relato de caso / Profuse Stewart-Treves syndrome in the right leg: a case report

A Síndrome de Stewart-Treves é uma rara condição onde um angiossarcoma se desenvolve sobre área de linfedema crônico. Afeta mais frequentemente os membros superiores e representa 5% dos casos de angiossarcoma. Apesar de ser mais comum em mulheres submetidas a mastectomia radical e esvaziamento axilar por neoplasia de mama, pode ocorrer a partir de linfedema de outras etiologias. Clinicamente as lesões são caracterizadas por nódulos únicos ou múltiplos de cor avermelhada ou azulada no estágio inicial e, em estágio avançado, podem evoluir para massas hemorrágicas e até gangrena. O prognóstico é ruim e a conduta cirúrgica com excisão ampla ou amputação oferecem uma maior chance de sobrevida. Relata-se um caso de Síndrome de Stewart-Treves na perna direita, apresentando-se de forma exuberante e com acometimento de linfonodos pélvicos e abdominais.

Stewart-Treves syndrome is a rare condition characterized by the development of an angiosarcoma at sites of chronic lymphedema. It most often affects the upper limbs and accounts for 5% of cases of angiosarcoma. Although the syndrome is more common in women undergoing radical mastectomy and axillary dissection for breast cancer, it may result from lymphedema of other etiologies. Clinically, the lesions are characterized by single or multiple bluish-red nodules in the early stage and may progress to hemorrhagic masses and even gangrene in advanced stages. The prognosis is poor, and surgical management with wide excision or amputation offers a greater chance of survival. We report a case of profuse Stewart-Treves syndrome in the right leg, with involvement of pelvic and abdominal lymph nodes.

Misdiagnosis of angiosarcoma of foot as diabetic foot ulcer: One case report and review of literature / 中华内分泌代谢杂志

Angiosarcoma is a rare malignant tumor of soft tissue in adults. However, tumor ulcers caused by foot angiosarcoma are rarely reported in the literature. This paper outlines a case of foot angiosarcoma misdiagnosed as diabetic foot ulcer. It was treated as diabetic foot ulcer in other hospitals without notable improvement. In consideration of its special clinical characteristics and treatment process, pathological biopsy was performed after admission, and angiosarcoma was confirmed. The purpose of this article is to emphasize that foot angiosarcoma or other tumor ulcers in diabetes patients are easily misdiagnosed as diabetic foot ulcers. If there is no obvious improvement in treatment, pathological tissue biopsy should be performed as early as possible.

Proliferación vascular atípica mamaria inducida por radioterapia / Atypical vascular proliferation of the breast induced by radiation

La proliferación vasculara típica mamaria inducida por radioterapia es una proliferación angiomatoide que aparece sobre la piel previamente irradiada por el tratamiento conservador de un carcinomademama. Se presenta el caso de una paciente de 58años que consultó por la aparición de múltiples pápulas purpúricas milimétricas en la mama derecha. Había recibido radioterapia y cuadrantectomía por un carcinoma intraductal 5años antes y estaba medicada con tamoxifeno. El análisis histópatológico e inmunohistoquímico informó: "Proliferación vascular atípica inducida por radiación, variedad atípica inducida por radiación, variedad linfática". Se adoptó una conducta expectante, con seguimiento estrecho.

Atypical vascular proliferation of the breast induced by radiation is an angiomatoid proliferation that appears on previously irradiated skin by the conservative treatament of a breast carcinoma. We present a 58-year-old female patient who consulted for multiple millimeter purpuric papules in the right breast. She received radiotherapy and quadrantectomy for an intraductal carcinoma 5 years before. She is currently on tomoxifen. The histopathology and immunohistochemistry reported atypical vascular proliferation induced by radiation, lymphatic variety. Watchuful waiting is adopted with close monitoring.

Neumotórax espontáneo bilateral metastásico. Inusual manifestación de metástasis pulmonar / Spontaneous metastatic bilateral pneumothorax. Unusual manifestation of lung metastasis

Resumen Objetivo: Comunicar el caso de una paciente de 77 años tratada de un angiosarcoma de cuero cabelludo, que evoluciona con neumotórax bilateral, cuyo estudio histopatológico informa enfermedad metastásica. Materiales y Método: Revisión de ficha clínica electrónica, registro informático de imagenología e informes de biopsias. Seguimiento ambulatorio en policlínico de cirugía. Resultados: Se realiza manejo por videotoracoscopía bilateral, con bulectomía más pleurodesis. Discusión: El neumotórax secundario por enfermedad metastásica es poco frecuente, aproximadamente 1% a 2% de los casos. De los tumores que más frecuentemente metastizan al pulmón se encuentran los sarcomas. Conclusión: El neumotórax metastásico es poco frecuente y requiere un alto índice de sospecha. Su manejo va a depender del pronóstico y de las condiciones generales del paciente.

Aim: To report the case of a 77-year-old patient treated for angiosarcoma of the scalp, who evolves with bilateral pneumothorax, whose histopathological study reports metastatic disease. Materials and Method: Review of electronic clinical record, computerized imaging record and biopsy reports. Outpatient follow-up at the polyclinic of surgery. Results: Management was performed by bilateral videothoracoscopy, with bulectomy plus pleurodesis. Discussion: Secondary pneumothorax due to metastatic disease is rare, approximately 1 to 2% of cases. Sarcomas are among the tumors that most frequently metastasize to the lung. Conclusion: Metastatic pneumothorax is infrequent and requires a high index of suspicion. Its management will depend on the prognosis and the general conditions of the patient.

Aortic Root and Pulmonary Artery Reconstruction in a Patient with Pulmonary Artery Intimal Sarcoma / 日本心臓血管外科学会雑誌

A 52-year old woman was referred to our hospital because of abdominal pain and restlessness. A chest contrast-enhanced CT showed huge pericardial effusion and intraluminal defects in the main pulmonary artery. We could not make a diagnosis based on the cytology of the pericardial effusion and histopathology of the mass with a sample taken by a catheter. Therefore, we undertook biopsies of the mass by median sternotomy, which led to the diagnosis of pulmonary intimal sarcoma. The tumor resection was performed to release the right ventricular outflow stenosis. We tried to resect the tumor as much as possible, and reconstructed the pulmonary artery and aortic root. She was discharged to home and survived 5 months after surgery.

Intra-Axial Metastatic Angiosarcoma of the Central Nervous System Associated with Anemia, Pulmonary Tuberculosis and Short Survival

Introduction Angiosarcoma (AG) is a malignant mesenchymal neoplasm that predominantly affects the soft tissues and, to variable degrees, expresses themorphological and functional characteristics of the endothelium. The incidence of sarcomas of the central nervous system(CNS) is low (0.5% to 2.7%), and AGs involving the brain are even rarer. Case Description A 45-year-old male patient presented with complaints of headache, nausea, and vomiting. An examination showed bilateral papilledema and a right lung pleurotomy. The patient's previous history included drug addiction, pulmonary tuberculosis, lung abscess, pleural empyema, and pulmonary artery embolization for severe hemoptysis. Computed tomography/magnetic resonance imaging scans revealed a large intra-axial lesion extending into the right parietal and temporal lobes, with hemorrhagic zones. The patient underwent surgical resection of the lesion. Microscopy showed a poorly-differentiated, high-grade malignant tumor composed of plump/epithelioid cells forming small vascular spaces and solid nests, compatible with AG.In the postoperative period, the patient developed recurrent hemoptysis. A biopsy of the tissues adjacent to the pleurotomy determined the diagnosis of pulmonary AG. At 30 days after the resection, the patient died from hemoptysis, hemothorax, lung atelectasis, and intracranial hypertension related to the recurrence of the brain tumor. Conclusion Angiosarcoma is a rare neoplasia related to short survival due to the high proliferative index, which must be considered in patients presenting hemorrhagic tumors. No specific genetic abnormalities have been described for this neoplasia.

Primary splenic angiosarcoma: a diagnostic enigma

Primary splenic angiosarcomas (PSA) arise from splenic endothelium are rare and impose a diagnostic challenge preoperative. They can present as asymptomatic splenomegaly however; the commonest presentation is abdominal pain. The spleen can rapidly increase in size and can manifest as spontaneous rupture which would cause peritoneal dissemination of disease. Early metastasis of PSA is seen in liver, lungs, lymph nodes and gastrointestinal system. Preoperative diagnosis requires a high index of suspicion and ultrasound, contrast enhanced computerized tomography may essential to differentiate from splenic hemangioma. Splenic angiosarcoma are best treated with splenectomy with a limited disease, with care taken not to rupture and cause spillage. PSA are resistant to adjuvant radiation and chemotherapy. Mortality is high with median survival rate of only 5 months, irrespective of treatment and hence the need to diagnose before complications. Bisphosphonates, adjuvant radiation with chemotherapy have been attempted to increase disease free survival. We report a case of PSA emphasizes on early preoperative diagnosis to avoid progression of the disease.

Angiosarcoma radioinducido: reporte de caso y revisión de la literatura / Radio-induced angiosarcoma: case report and literature review

El angiosarcoma de la mama es un tumor maligno de origen endotelial vascular que puede ser primario, si surge sin un precursor conocido, o secundario, si ocurre en el sitio de la piel previamente irradiada. En este último caso, se conoce como angiosarcoma inducido por radiación (rias) del seno. Se trata de una complicación rara y tardía de la radioterapia del cáncer de mama, con una alta tasa de recurrencia y progresión. En este artículo, presentamos el caso de una paciente de sexo femenino de 73 años, con antecedentes de carcinoma infiltrante de mama derecha, tratada con cirugía conservadora y posterior radioterapia, quien, al quinto año de seguimiento, presentó múltiples nódulos de color violáceo en los 4 cuadrantes de la mama previamente irradiada. El diagnóstico fue de angiosarcoma radioinducido, realizándose mastectomía y reconstrucción inmediata con colgajo de dorsal ancho, en la que, de forma inicial, se obtiene una resección R1 (microscópicamente márgenes < 1 cm). En espera de ampliación de márgenes, la paciente comienza con síntomas respiratorios y se documenta progresión pulmonar. Conclusión: La sospecha e identificación de los angiosarcomas radioinducidos permite realizar un manejo oportuno y preciso de una patología poco frecuente y con un comportamiento muy agresivo

Angiosarcoma of the breast is a malignant tumor of vascular endothelial origin that can be primary, if it arises without a known precursor, or secondary, if it occurs at the site of previously irradiated skin. In the latter case, it is known as radiation-induced angiosarcoma (rias) of the breast. It is a rare and late complication of radiation therapy for breast cancer, with a high rate of recurrence and progression. In this article, we present the case of a 73-year-old female patient with a history of infiltrating carcinoma of the right breast, treated with conservative surgery and subsequent radiotherapy, who, at the fifth year of follow-up, presented multiple purplish-colored nodules in the 4 quadrants of the previously irradiated breast. The diagnosis was radio-induced angiosarcoma, performing a mastectomy and immediate reconstruction with a latissimus dorsi flap, in which, initially, an R1 resection was obtained (microscopically margins < 1 cm). While waiting for margin expansion, the patient begins with respiratory symptoms and pulmonary progression is documented. Conclusions: The suspicion and identification of radio-induced angiosarcomas allows timely and precise management of a rare pathology and with very aggressive behavior