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.

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.

Multiple hepatic metastases of cardiac angiosarcoma

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

A rare cause of abdominal pain in childhood: cardiac angiosarcoma

Abstract Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain. Echocardiography and thoracic computed tomography showed right atrial mass. The patient underwent surgery, chemotherapy, and radiotherapy. Eight months after treatment, abdominal recurrence was detected. The abdominal mass was resected, and radiotherapy and new chemotherapy protocol were given. The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in an adolescent girl.

Metástasis ovárica única de angiosarcoma mamario como localización infrecuente / Single ovarian metastases of breast angiosarcoma as unusual location

Los angiosarcomas son tumores vasculares extremadamente raros con alta tasa de proliferación e invasión. Están caracterizados por células anaplásicas que se originan en las paredes de los vasos sanguíneos. Poseen mal pronóstico debido a su comportamiento altamente agresivo, el cual condiciona una elevada probabilidad de recurrencia local y extensión multiorgánica. Se presentan principalmente en: pulmón, hígado, ganglios linfáticos y huesos. Su tratamiento es controvertido debido escasa casuística disponible acerca de esta estirpe tumoral, aunque parece claro que el tratamiento principal es la cirugía y que el tratamiento posterior puede ser la quimioterapia adyuvante(AU)

Angiosarcomas are extremely rare vascular tumors with high rate of proliferation and invasion. They are characterized by anaplastic cells originating in the walls of blood vessels. They have poor prognosis because of their highly aggressive behavior, which determines a high probability of local recurrence and spreading to multiple organs. They occur mainly in lung, liver, lymph nodes and bones. Treatment is controversial due to small number of cases available about this tumor type, although it seems clear that the main treatment is surgery and subsequent treatment can be adjuvant chemotherapy(AU)

Angiosarcoma primario de mama y carcinoma papilar de tiroides sincrónico: presentación de un caso / Primary angiosarcoma of breast and synchronous thyroid papillary carcinoma: a case report

Antecedentes: El angiosarcoma primario de mama constituye un tumor infrecuente que se desarrolla de forma predominante en mujeres en la tercera y cuarta décadas de la vida. Los nódulos tiroideos incidentales son aquellos que se objetivan en pruebas de imagen realizadas por otra causa y se observan en menos de un 10 por ciento con la tomografía por emisión de positrones (PET). Objetivo: Presentar el caso excepcional de una paciente con angiosarcoma primario de mama y un carcinoma papilar de tiroides sincrónicos. Caso clínico: Mujer de 34 años con angiosarcoma primario en la mama derecha sometida a mastectomía simple. En el estudio de extensión se halló un incidentaloma tiroideo derecho compatible con carcinoma papilar que requirió tiroidectomía total y linfadenectomía del compartimento central cervical en un segundo tiempo. Comentarlo: Los angiosarcomas primarios suponen menos del 0,05 por ciento de todos los tumores primarios malignos de la mama. El diagnóstico definitivo de estos tumores viene definido por el estudio anatomopatológico, que establece tres grados: alto, bajo e intermedio, los cuales se relacionan de forma directa con la supervivencia. La prevalencia de los incidentalomas malignos tiroideos objetivados por PET varía del 14-30,9 por ciento, según diferentes estudios. La planificación del tratamiento quirúrgico de las neoplasias incidentales tiroideas en pacientes con otro tumor primario debe analizarse de forma individualizada, según la supervivencia esperada del tumor primario.

Background: The primary angiosarcoma of breast is a rare tumor that develops predominantly in women in the third and fourth decades of life. Incidental thyroid nodules are observed in imaging tests performed for other reasons and it's observed in less than 10 percent with the positron emission tomography (PET). Aims: To report the exceptional case of a patient with primary angiosarcoma of the breast and synchronous thyroid papillary carcinoma. Clinical case: Female patient 34 year old with primary angiosarcoma in the right breast, submitted to a simple mastectomy. In the extension study we found an incidental thyroid papillary carcinoma that required a total thyroidectomy and central cervical lymphadenectomy in a second time. Comments: The primary angiosarcoma are less than 0.05 percent of all primary malignant tumors of the breast. The definitive diagnosis is determined by the pathology, which defines three grades: high, low and intermediate, which relate directly to survival. Prevalence of malignant thyroid incidentalomas observed by PET varies from 14 to 30.9 percent, according different studies. Planning the surgical treatment of incidental thyroid malignancies in patients with other primary tumor should be analyzed individually, according the expected survival of the primary tumor.

Angiosarcoma intracardíaco: presentación de un caso / Intracardiac sarcoma: a case report

Objetivo: Revisión y presentación de un caso de angiosarcoma cardíaco. Introducción: Los tumores malignos cardíacos primarios aparecen con una frecuencia de 0,0017-0,33 por ciento (sarcoma 16 por ciento y angiosarcoma 26 por ciento); predominan los metastáticos. Alta malignidad; relación hombres-mujeres, 2:1; edad, entre los 20 y los 60 años. La clínica depende de la localización, el tamaño y la histología. Predominan en la aurícula derecha. Trombogénicos; destruyen la estructura; producen derrame, isquemia; arritmias; muerte súbita. Invaden contiguamente; metastatizan a distancia. Escasa respuesta a radioterapia y quimioterapia. Pronóstico ominoso. Presentación del caso: Mujer de 72 años; antecedente de disnea CF II, 2 síncopes. Masa en la aurícula derecha, heterogénea, sin pedículo y otra en el ventrículo derecho, infiltración pericárdica. Pericardiocentesis diagnóstica y terapéutica. Estudios negativos de lesión extracardíaca. ASA IV. Estimación del riesgo perioperatorio: bloqueo A-V, SIRS, embolizaciones. Anestesia general balanceada, infusión de ípsilon y solución GIK. Derivación cardiopulmonar (CPB) 71' y clampeo aórtico 49'. Cirugía: resección de masa en la aurícula, el pericardio y la pleura adyacente. Destete de CPB con marca paso transitorio, apoyo inotrópico. Postoperatorio: hipoperfusión tisular, acidemia, hiperglucemia, hipopotasemia, sin respuesta terapéutica. Óbito a las 4 horas, con diagnóstico presuntivo de respuesta inflamatoria sistémica.

Objective: Review and presentation of a case of cardiac angiosarcoma. Introduction: Primary cardiac malignant tumors have frequency between 0,0017-0,33 per cent (sarcoma 16 per cent and angiosarcoma 26 per cent), predominantly metastatic. High malignancy; male-female ratio: 2:1; appears between ages 20 to 60. Symptoms depend on location, size and histology. Predominate in right atrium. Thrombogenic, they destroy the structure; cause spills, ischemia, arrhythmia, sudden death. Invade contiguously; metastasize at a distance. Poor response to radiotherapy and chemotherapy. Ominous prognosis. Case report: 72 year old female. Dyspnoea CF II, two syncopes. Heterogeneous mass in right atrium (RA) and another one in right ventricle with pericardial infiltration. Diagnosis and treatment by puncture of the pericardium. Studies negative for primary extracardiac injury. ASA IV. Perioperative risk estimation: AV Block, SIRS, embolizations. Balanced general anesthesia, infusion of GIK solution and Ipsilon. Cardiopulmonary by-pass 71' and cross aortic clamp 49'. Surgery: resection mass in RA and adjacent pericardium and pleura. Weaning from CPB: transitory pacemaker and inotropic support. Surgery: resection mass in RA and adjacent pericardium and pleura. Postoperative period: tissue hypoperfusion, lactacidemia, hyperglycemia resistant to insulin, hypopotassemia. No response to treatment. Cardiac arrest at the 4th hour with a presumptive diagnosis of systemic inflammatory response. Death.

Objetivo: Revisao e apresentaçao de um caso de angiosarcoma cardíaco. Introduçao: A ocorrência de tumores cardíacos malignos primários está na faixa de 0,0017-0,33 por cento (sarcoma 16 por cento e angiosarcoma 26 por cento); predominam os metastáticos. Alta malignidade; relaçao homens-mulheres, 2:1; faixa etária: 20 a 60 anos. A clínica depende da localizaçao, do tamanho e da histologia. Predominam na aurícula direita. Trombogénicos; destroem a estrutura; causam derrame, isquemia; arritmias; morte súbita. Invadem estruturas vizinhas; produzem metástase a distancia. Fraca resposta a radioterapia e quimioterapia. Prognóstico nefasto.Apresentaçao do caso: Mulher de 72 anos; antecedente de dispnéia CF 11,2 síncopes. Massa na aurícula direita, heterogênea, sem pedículo e outra no ventrículo direito, infiltraçao pericárdica. Pericardiocentese diagnóstica e terapêutica. Estudos negativos de lesao extracardíaca. ASA IV. Estimaçao do risco perioperatório: bloqueio A-V, SIRS, embolizaçoês. Anestesia geral balanceada, infusao de Ipsílon e soluçao GIK. By-pass cardiopulmonar (CPB) 71' e clampeamento da aorta 49'. Cirurgia: ressecçao de massa na aurícula, no pericárdio e na pleura adjacente. Retiro gradual do CPB com marca-passo transitório, apoio inotrópico. Pósoperatório: hipoperfusao tissular, acidemia, hiperglicemia, hipopotassemia, sem resposta terapêutica. Óbito 4 horas depois, com diagnóstico presuntivo de resposta inflamatória sistêmica.

Cutaneous angiosarcoma in a patient with xeroderma pigmentosum.

Xeroderma pigmentosum (XP) is a rare, autosomal recessive disorder characterized by photosensitivity, cutaneous pigmentary changes, premature skin ageing and development of various cutaneous and internal malignancies at an early age as a result of a defect in nucleotide excision repair following ultraviolet light exposure. Cutaneous angiosarcomas are aggressive neoplasms that are rarely associated with XP. In this communication, we report the case of a 40-year-old male patient with XP who developed an angiosarcoma of the face and discuss the implications of this association in view of recent developments in this field.

Diagnóstico ecocardiográfico de tumor primário do coração - A propósito de um caso de angiossarcoma / Echocardiography diagnosis of primary cardiac tumor - a case of cardiac angiossarcoma

Neste artigo, descreve-se o caso de uma paciente de 39 anos, com diagnóstico de angiossarcoma no apêndice atrial esquerdo, com infiltração da parede do átrio e das cúspides da valva mitral, que ocasionou estenose mitral, documentados por ecocardiograma transtorácico e transesofágico. O caso ilustra a importância da ecocardiografia no diagnóstico diferencial das massas cardíacas e no diagnóstico de tumores primários do coração. Para orientar o diagnóstico ecocardiográfico diferencial e etiológico da massa aplicou-se o método de análise chamado diagnóstico em alvo que considera três características: alterações clínicas e hemodinâmicas associadas, a localização e a forma de implantação e as características ecocardiográficas e do comportamento da massa. A paciente tinha síntomas de insuficiência cardíaca, foi submetida a tratamento cirúrgico e o diagnóstico de angiossarcoma foi confirmado.

Angiossarcoma cardíaco primário / Primary cardiac angiosarcoma

O angiossarcoma primário do coração é um tumor maligno raro, derivado do mesênquima, com predileção pelo átrio direito. Acomete mais homens, com idade média de 40 anos. Na maioria dos casos já tornando pior o prognóstico. As modalidades terapêuticas atualmente têm pouco sucesso, apenas prolongando a sobrevida em alguns meses, mesmo nos casos em que se consegue ressecção cirúrgica ampla ou transplante cardíaco. Relata-se o caso de uma paciente negra, de 53 anos, que apresentou súbito quadro de dor torácica direita e síncope e, posteriormente, evoluiu com dispnéia e síndrome da veia cava superior. A investigação pelos métodos de imagem demonstrou um tumor cardíaco no átrio direito sugestivo de sarcoma, sendo considerado de difícil abordagem cirúrgica. Em poucas semanas a paciente evoluiu para insuficiência pré-renal, congestão pulmonar e óbito após 4 meses do início dos sintomas

A Case of Primary Angiosarcoma of Small Intestine Presenting as Recurrent Gastrointestinal Bleeding / 대한소화기학회지

Angiosarcoma is a rare malignant tumor which occurs frequently in the skin and soft subcutis. Moreover, primary gastrointestinal angiosarcomas are very rare. This tumor manifests as non-specific symptoms such as gastrointestinal bleeding, abdominal pain and nausea. The diagnosis is often made at an advanced stage. Surgery, chemotherapy and radiotherapy are the mainstay of treatment. However, the prognosis is very poor. We report a case of primary angiosarcoma of the small intestine presenting as recurrent gastrointestinal bleeding. A 54-year-old man was admitted with recurrent gastrointestinal bleeding. An abdominal CT scan revealed an ileo-ileal intussusception. Segmental resection was performed with ileo-ileal anastomosis. The ileal mass was diagnosed as angiosarcoma on immunohistochemical stain. He received 3 cycles of chemotherapy, but died 5 months after the diagnosis.

Bilateral Spontaneous Pneumothorax During Cytotoxic Chemotherapy for Angiosarcoma of the Scalp: A Case Report

Spontaneous pneumothorax is a rare manifestation of metastatic lung cancers and described in advanced diseases or during cytotoxic chemotherapy which is manifested by sudden onset of dyspnea. The cause or mechanism of spontaneous pneumothorax has been unknown, as well as the association with site of metastases or type of cancers or side effect of chemotherapeutic drugs has been reported rarely. A 68-yr-old man underwent excision of angiosarcoma of the scalp. Chest radiography did not show any evidence of possible metastatic lung lesion at that time. Therefore, systemic doxorubicin and dacarbazine were given. After nineteen days of chemotherapy, he developed a bilateral spontaneous pneumothorax and palpable cervical lymph nodes. Both parietal and visceral pleura were intact and showed no evidence of metastatic and pathologic lesions on thoracoscopic evaluation. The patient managed with bilateral tube thoracostomy and both lungs were expanded. Lymph nodes became unpalpable during three cycles of the paclitaxel and doxorubicin, however, bilateral lung metastases were developed and progressed despite chemotherapy. The patient died due to respiratory failure after five months. This report underlines that spontaneous pneumothorax can occur as the first manifestation of metastatic angiosarcoma even if imaging studies do not show of a metastatic lesion.

Angiosarcoma of the heart presenting as recurrent cardiac tamponade.

We present an interesting case of angiosarcoma of the heart presenting as recurrent percardial effusion and cardiac tamponade.

Angiosarcomas radioinducidos / Radioinducted angiosarcomas

Se contribuye a la casuística con la presentación de 2 casos de angiosarcoma radioinducido, diagnosticados ambos, en pacientes de sexo femenino que previamente habían recibido dosis total de radioterapia, uno por presentar un linfoma tipo Hodgkin, y otro por carcinoma ductal de mama. En una de las pacientes el tiempo de latencia fue de 10 años, mientras que en la otra fue de 25 años. La evolución fue dispar, ya que mientras una tiene un año de evolución sin evidencias de enfermedad, la otra falleció debido a metástasis pulmonares y complicaciones cardíacas al año de ser operada

Vertebral hemangiosarcoma as a cause of spinal cord compression in a horse

A case compression of the spinal cord in a horse by a tumor located in the vertebra is described. A 10-year-old- gelding was euthanized after being found recumbent with hind limb paresis of unknown duration. At necropsy an hemangiosarcoma was detected in the vertebral body of T3. The tumoral mass invaded upwards through the bone tissue of the vertebral body into the vertebral canal, compressing the spinal cord and causing Wallerian degeneration at T1-3 levels of the cord.

Angiosarcoma retroperitoneal: descripción de un caso / Retroperitoneal angiosarcoma: description of a case

Los angiosarcomas son tumores vasculares malignos extremadamente raros, que se desarrollan principalmente en tejidos cutáneos, mamas e hígado. La ubicación retroperitoneal es excepcional. Se describe un caso anatomoclínico de angiosarcoma retroperitoneal en un hombre de 40 años, que cosnulta por lumbago de 2 años de evolución y caquexia extrema. Al momento del ingreso hospitalario destacan la hepatomegalia importante en la que el estudio ecográfico revela múltiples metástasis y un tumor retroperitoneal. Una biopsia incisional hepática diagnostica angiosarcoma pobremente diferenciado. El paciente fallece a los 2 meses del diagnóstico por compromiso respiratorio y cuadro de hemorragia digestiva alta. El estudio anatomopatológico demostró la existencia de un angiosarcoma retroperitoneal con metastásis hepáticas y pleurales

Angiosarcoma hepatico asociado a coagulacion intravascular diseminada / Hepatic angiosarcoma with disseminated intravascular coagulation

En este trabajo presentamos el caso de una mujer de 70 años con anemia y hepatomegalia. Los estudios de diagnóstico por imagen (ecografía y TC) demostraron un tumor hepático. La paciente desarrolló una coagulopatía por consumo y falleció. El estudio de necropsia reveló un angiosarcoma hepático. Se discuten los aspectos fisiopatológicos de la coagulación intravascular diseminada asociada a tumores vasculares y las alteraciones de la coagulación en pacientes con cáncer.

Angiosarcoma del corazón y su ruptura espontánea. Rara causa de pericarditis efusivo-constrictiva. Presentación de un caso y revisión de la literatura / Angiosarcoma of the heart and its spontaneous rupture. A rare cause of effusive-constrictive pericarditis. A case report and review of the literature

The authors present the clinical history of a male 44 year old patient who was hospitalized with the diagnosis of pericardial constriction and effusion and operated on as an emergency because of spontaneous cardiac rupture and was found to have a cardiac adenosarcoma. They review the literature in order to discuss a very rare cause of hemopericardium and constrictive pericarditis with epidemiologic, diagnostic, therapeutic and prognostic commentaries and secondly, to try to establish if this type case does not represent a diagnostic problem for the ecocardiographer since an angiosarcoma that occupies the pericardial space can be confused with a hemopericardium. They also mention other imaging studies that are used to better characterize and diagnose these tumors

Angiosarcoma of cecum: unusual presentation with intussusception.

We report a 55-year-old man with angiosarcoma of the cecum who presented with generalized colicky abdominal pain with a mass in the periumbilical region. The lesion at surgery was a colo-colic intussusception extending to the midtransverse colon. A radical right hemicolectomy was done. Metastases to regional lymph nodes were noted in the specimen.