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1.
BMJ Case Rep ; 14(1)2021 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-33472808

RESUMO

We report a rare case of cardiac angiosarcoma in a young boy who presented with cardiac tamponade. His initial symptoms were non-specific. He was initially being managed in the line of fungal infection, with a possibility of malignancy. Cardiac imaging was also not conclusive and he worsened on antibiotics and antifungals and succumbed to the illness. After his death tissue biopsy from heart and lung was done and histopathological examination revealed the diagnosis of metastatic angiosarcoma. The case highlights the importance of considering the diagnosis of cardiac angiosarcoma in the patients presenting with haemorrhagic pericardial effusion and non-specific symptoms.


Assuntos
Tamponamento Cardíaco/etiologia , Neoplasias Cardíacas/complicações , Hemangiossarcoma/complicações , Derrame Pericárdico/etiologia , Tamponamento Cardíaco/diagnóstico por imagem , Tamponamento Cardíaco/terapia , Drenagem , Ecocardiografia , Evolução Fatal , Átrios do Coração , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Hemangiossarcoma/diagnóstico por imagem , Hemangiossarcoma/patologia , Hemangiossarcoma/secundário , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/secundário , Imagem por Ressonância Magnética , Masculino , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/terapia , Choque/etiologia , Tomografia Computadorizada por Raios X , Adulto Jovem
2.
J Surg Res ; 257: 213-220, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32858322

RESUMO

Angiosarcomas (AS) are a diverse group of soft tissue sarcomas, arising from blood and lymphatic vessels. They frequently present in the elderly, and in patients with previous radiation or lymphedema. A wide range of genetic derangements contribute to their development, and AS histology is often high-grade in keeping with aggressive disease biology. The clinical presentation, while often innocuous, is marked by its infiltrative and aggressive nature, with a proclivity for metastatic spread, and outcomes are often poor. Surgery is performed for localized, resectable cases. A multidisciplinary approach, appropriately employing surgery, radiation, chemotherapy, or potentially recently approved immune-oncology agents, can result in positive outcomes.


Assuntos
Hemangiossarcoma/terapia , Recidiva Local de Neoplasia/prevenção & controle , Equipe de Assistência ao Paciente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Vasos Sanguíneos/patologia , Vasos Sanguíneos/efeitos da radiação , Quimioterapia Adjuvante , Ensaios Clínicos Fase II como Assunto , Hemangiossarcoma/genética , Hemangiossarcoma/mortalidade , Hemangiossarcoma/patologia , Humanos , Vasos Linfáticos/patologia , Vasos Linfáticos/efeitos da radiação , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/patologia , Intervalo Livre de Progressão , Radioterapia Adjuvante , Procedimentos Cirúrgicos Operatórios
5.
Anticancer Res ; 40(10): 5743-5750, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32988901

RESUMO

BACKGROUND/AIM: Angiosarcoma of primary gynecologic origin is an extremely rare and highly malignant tumor of endothelial origin with a 5-year survival rate of less than 35%. To date, only 61 cases have been described in the literature. The aim of this study was to present more cases and discuss potential therapy options. CASE REPORT: The following case series presents three cases of gynecologic angiosarcomas that were under therapy at the Charité - University medicine of Berlin from June 2014 to February 2018. RESULTS: Two of the cases deal with primary angiosarcomas of the uterus whereas the third case was diagnosed after the suspicion of a recurrence of a poorly differentiated squamous cell carcinoma of the cervix uteri. In case one a 75-year old patient with initial postmenopausal bleeding and a tumor mass of the uterus is described. After surgery a hemangiosarcoma of the uterus was confirmed. After two months the patient presented with a presacral peritoneal sarcomatosis. Chemotherapy of weekly paclitaxel was administered. Case two deals with a patient presenting with abdominal pain. A uterine sarcoma with infiltration of the parametry and angiosarcomatosis peritonei was diagnosed during an emergency laparotomy because of spontaneous peritoneal bleeding. Moreover, osseous metastasis was found. The patient underwent weekly paclitaxel. Due to tumor progression, chemotherapy was changed to doxorubicin and olaratumab and radiotherapy was induced. The patient died 33 months after initial diagnosis. Case three describes a 34-year old patient with suspected local recurrence of cervical cancer with infiltration of the bladder. During TURB an angiosarcoma was found. Following laparoscopy revealed peritoneal metastasis. The patient underwent weekly paclitaxel followed by a paclitaxel and pazopanib maintainance therapy which showed a regression. Due to progression afterwards, chemotherapy was changed to gemcitabine and docetaxel and gemcitabine monotherapy. The patient died 33 months after initial diagnosis. CONCLUSION: Even though there is no evidence on standard treatment of this extremely rare and aggressive tumor entity of the female genital tract the patients showed the longest stability of disease during chemotherapy with weekly paclitaxel.


Assuntos
Tratamento Farmacológico , Neoplasias dos Genitais Femininos/tratamento farmacológico , Hemangiossarcoma/tratamento farmacológico , Paclitaxel/administração & dosagem , Adulto , Idoso , Terapia Combinada , Feminino , Neoplasias dos Genitais Femininos/diagnóstico por imagem , Neoplasias dos Genitais Femininos/patologia , Neoplasias dos Genitais Femininos/cirurgia , Hemangiossarcoma/diagnóstico por imagem , Hemangiossarcoma/patologia , Hemangiossarcoma/cirurgia , Humanos , Laparotomia , Pessoa de Meia-Idade , Metástase Neoplásica , Paclitaxel/efeitos adversos
6.
Leg Med (Tokyo) ; 47: 101785, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32977197

RESUMO

Splenic angiosarcoma (SA) is a rare disease that can cause spontaneous splenic rupture leading to unexpected death. The rare incidence and non-specific clinical presentations made the early correct diagnosis of SA impossible in clinical practice. Even with medical intervention, 80% of patients died within 6 months after diagnosis. Here, we report a man who had complained of abdominal distension for 2 weeks and succumbed to the disease nine hours after admission. Diagnosis of hepatic and splenic angiosarcomas was based on post-mortem histopathological examination and immunohistochemistry, and the cause of death was hemorrhagic shock caused by spontaneous splenic rupture secondary to SA. This present case was the first forensic autopsy of spontaneous splenic rupture secondary to SA, which can highlight the diagnosis of rare diseases in forensic practice, and forensic pathologists should bear in mind these rare diseases even in routine practice.


Assuntos
Autopsia , Morte Súbita/etiologia , Morte Súbita/patologia , Patologia Legal , Hemangiossarcoma/complicações , Hemangiossarcoma/diagnóstico , Neoplasias Esplênicas/complicações , Neoplasias Esplênicas/diagnóstico , Ruptura Esplênica/etiologia , Adulto , Evolução Fatal , Hemangiossarcoma/patologia , Humanos , Masculino , Doenças Raras , Ruptura Espontânea , Choque Hemorrágico/etiologia , Neoplasias Esplênicas/patologia
7.
Cancer Imaging ; 20(1): 59, 2020 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-32795351

RESUMO

BACKGROUND: Owing to its low incidence, there is insufficient clinical awareness and diagnostic experience with primary hepatic malignant vascular tumors (PHMVTs). The aim of our study was to investigate the imaging and clinicopathological features of patients with PHMVTs and analyze the clinicopathological correlations. METHODS: We retrospectively analyzed 42 patients who had pathologically confirmed PHMVT during the period from June 2012 to December 2019 and enrolled them in our study. The computed tomography (CT) and magnetic resonance (MR) images and pathological findings of each patient were recorded. RESULTS: There were more female (29/42) than male patients. The imaging features of primary hepatic angiosarcoma (PHA) (n = 11) included ill-defined margins (11/11, 100%), necrosis (5/11, 45%), calcification (3/11, 27%) and "slow in-slow out" centripetal enhancement (7/11, 64%). Patients with epithelioid hemangioendothelioma (EHE) (n = 15) presented with ill-defined margins (15/15, 100%), necrosis (6/15, 40%), calcification (2/15, 13%), "fast in-slow out" centripetal enhancement (10/15, 67%), halo sign (15/15, 100%), pseudocapsule sign (4/15, 27%), lollipop sign (2/15, 13%) and capsule retraction sign (2/15, 13%). Patients with malignant hemangiopericytoma (MHP) (n = 3) showed ill-defined margins (3/3, 100%), necrosis (3/3, 100%) and "fast in-slow out" progressive enhancement (3/3, 100%). Infantile hemangioendotheliomas (IHEs) (n = 13) were defined by ill-defined margins (7/13, 54%), necrosis (8/13, 62%), calcification (5/13, 38%) and "fast in-slow out" centripetal enhancement (13/13, 100%). Immunohistochemistry showed strong positive expression of CD31, CD34, ERG, FaVIII and FLI-1. Patients with IHE (96 months) and EHE (88 months) had the longest survival times, followed by those with MHP (23 months), while patients with PHA (15 months) had the shortest survival time. CONCLUSION: On CT and MR images, most PHMVTs were ill-defined, heterogeneous, hypervascular masses with centripetal progressive enhancement and possibly calcification, especially in female patients. The prognosis of patients with PHMVT was associated with the pathological type of the tumor.


Assuntos
Hemangioendotelioma Epitelioide/diagnóstico por imagem , Hemangiossarcoma/diagnóstico por imagem , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Vasculares/diagnóstico por imagem , Adulto , Feminino , Hemangioendotelioma Epitelioide/patologia , Hemangiossarcoma/patologia , Humanos , Neoplasias Hepáticas/patologia , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Prognóstico , Tomografia Computadorizada por Raios X/métodos , Neoplasias Vasculares/patologia
9.
Clin Nucl Med ; 45(8): 638-639, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32558714

RESUMO

Primary cardiac angiosarcoma is rare and often advances by dissemination. Lungs are the most common metastatic sites, especially when the tumor originates in the right side of the heart. Bone metastases from cardiac angiosarcoma very rarely occur without concurrent pulmonary metastases. We report a case of cardiac angiosarcoma having prominent bone metastases without concurrent pulmonary lesions, as demonstrated by FDG PET/CT scan.


Assuntos
Neoplasias da Medula Óssea/diagnóstico por imagem , Neoplasias Cardíacas/diagnóstico por imagem , Hemangiossarcoma/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Neoplasias da Medula Óssea/secundário , Fluordesoxiglucose F18 , Neoplasias Cardíacas/patologia , Hemangiossarcoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Compostos Radiofarmacêuticos
10.
World Neurosurg ; 139: 232-237, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32330618

RESUMO

BACKGROUND: Cerebral angiosarcoma is an extremely rare malignant tumor that originates from vascular endothelial cells of the brain or meninges. Especially, primary brainstem angiosarcoma has not been reported. CASE DESCRIPTION: A 64-year-old man presented with left subjective weakness, hypesthesia, and dizziness. Brain imaging showed a multistage hemorrhagic mass in the right dorsal pons, which was initially misdiagnosed as a cavernous malformation. The patient's neurologic status suddenly deteriorated over a few months, and the mass grew rapidly. Surgical resection was performed, and the final pathology showed brainstem angiosarcoma. CONCLUSIONS: To our knowledge, this is the first case of brainstem angiosarcoma confirmed by pathology after surgical resection. This report highlights that clinicians need to consider angiosarcoma as part of the differential diagnosis for rare hemorrhagic lesions in the brainstem when both imaging findings and neurologic deterioration indicate rapid progression.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico , Hemangiossarcoma/diagnóstico , Neoplasias do Tronco Encefálico/patologia , Erros de Diagnóstico , Hemangiossarcoma/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroimagem/métodos , Tomografia Computadorizada por Raios X
14.
J Pathol ; 251(2): 123-134, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32166747

RESUMO

Spontaneously regressing infantile haemangiomas and aggressive angiosarcomas are vascular tumours with excessive angiogenesis. When analysing haemangiomas and angiosarcomas immunohistochemically with respect to their chaperone profiles we found that angiosarcomas have significantly elevated protein levels of binding immunoglobulin protein (BIP) and PERK with concomitant attenuated IRE1α levels, whereas haemangioma tissue exhibits the same pattern as embryonal skin tissue. We show that BiP is essential for the maintenance of VEGFR2 protein, which is expressed in the endothelium of both tumour types. When studying the effects of BiP, the IRE1α/Xbp1 -, and PERK/ATF4-signalling pathways on the migration and tube-forming potential of endothelial cells, we show that downregulation of BiP, as well as inhibition of the kinase activity of IRE1α, inhibit in vitro angiogenesis. Downregulation of PERK (PKR-like kinase; PKR = protein kinase R) levels promotes Xbp1 splicing in endoplasmic reticulum (ER)-stressed cells, indicating that in angiosarcoma the elevated PERK levels might result in high levels of unspliced Xbp1, which have been reported to promote cell proliferation and increase tumour malignancy. The data presented in this study revealed that in addition to BiP or PERK, the kinase domains of IRE1α and Xbp1 could be potential targets for the development of novel therapeutic approaches for treating angiosarcomas and to control tumour angiogenesis. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.


Assuntos
Endorribonucleases/metabolismo , Células Endoteliais/enzimologia , Proteínas de Choque Térmico/metabolismo , Hemangioma/enzimologia , Hemangiossarcoma/enzimologia , Neovascularização Patológica , Proteínas Serina-Treonina Quinases/metabolismo , eIF-2 Quinase/metabolismo , Movimento Celular , Proliferação de Células , Células Cultivadas , Endorribonucleases/genética , Células Endoteliais/patologia , Regulação Enzimológica da Expressão Gênica , Regulação Neoplásica da Expressão Gênica , Proteínas de Choque Térmico/genética , Hemangioma/genética , Hemangioma/patologia , Hemangiossarcoma/genética , Hemangiossarcoma/patologia , Células Endoteliais da Veia Umbilical Humana/enzimologia , Humanos , Proteínas Serina-Treonina Quinases/genética , Transdução de Sinais , Receptor 2 de Fatores de Crescimento do Endotélio Vascular/genética , Receptor 2 de Fatores de Crescimento do Endotélio Vascular/metabolismo , Proteína 1 de Ligação a X-Box/genética , Proteína 1 de Ligação a X-Box/metabolismo , eIF-2 Quinase/genética
15.
Anticancer Res ; 40(3): 1645-1649, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32132069

RESUMO

In inoperable patients, the management of angiosarcoma of the scalp is challenging. Due to intrinsic, dosimetric and radiobiological properties, proton beam radiotherapy may be an effective and safe option to offer to these difficult-to-cure patients. Here, we report a case of angiosarcoma of the scalp treated successfully with proton beam radiotherapy. Angiosarcoma is a rare malignancy concerning around 2% of soft-tissue sarcomas and 5% of cutaneous soft-tissue sarcomas. Cutaneous angiosarcomas can occur in any part of the body, but the head and neck region is a common primary site and the scalp is a frequent site in elderly patients.


Assuntos
Neoplasias de Cabeça e Pescoço/radioterapia , Hemangiossarcoma/radioterapia , Terapia com Prótons/métodos , Couro Cabeludo/patologia , Idoso de 80 Anos ou mais , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/patologia , Hemangiossarcoma/mortalidade , Hemangiossarcoma/patologia , Humanos , Masculino , Taxa de Sobrevida
16.
Anticancer Res ; 40(2): 625-633, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32014903

RESUMO

Primary angiosarcoma of the kidney is a rare tumor, hence little is known concerning its diagnostic features and therapeutic management. We conducted this survey to present a complete literature review with emphasis on clinicopathological features, diagnosis and therapy. A thorough search was conducted in MEDLINE/PubMed. All relevant studies concerning primary renal angiosarcomas in adults were thoroughly reviewed. Primary renal angiosarcoma is characterized by an overall poor prognosis, is of unknown etiology and occurs most commonly in males between 60 and 70 years old. Presence of distant metastasis at the time of diagnosis is prevalent. Histopathological examination and immunohistochemical studies are the most important diagnostic tools. Treatment options include surgery, chemotherapy, radiotherapy and immunotherapy. Conclusion: Primary renal angiosarcoma is a rare but aggressive malignancy with low response to available therapeutic regimens and dismal survival rates.


Assuntos
Hemangiossarcoma/patologia , Rim/patologia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico
17.
Jpn J Clin Oncol ; 50(5): 556-567, 2020 May 05.
Artigo em Inglês | MEDLINE | ID: mdl-32083280

RESUMO

BACKGROUND: Angiosarcoma is an aggressive and malignant neoplasm. Primary hepatic angiosarcoma is extremely rare and accounts for only approximately 5% of all angiosarcomas. Therefore, many doctors do not know enough about this disease; this lack of knowledge motivated us to perform this study. METHODS: We carried out a systematic review of the literature published worldwide from 1990 to 2019 to study the main characteristics, demographics, treatment and prognosis of primary hepatic angiosarcoma. RESULT: A total of 219 patients were included in this study. Patients were mainly middle-aged and elderly at diagnosis, with an average age at onset of 56.7 years. The vast majority of patients (61.5%) presented with abdominal pain or distension. Of 143 patients with clear records of metastasis, 31.5% (45 patients) had distant metastasis. The median overall survival time was only 6 months, and the 1- and 2-year survival rates were 30.4 and 17.3%, respectively. Sex, age, tumor size and metastasis at diagnosis showed no correlation with survival rate. Hepatic rupture was a significant predictor of survival. Surgery is a major treatment choice, and adjuvant chemotherapy can improve the prognosis of patients. Hepatic artery embolization is mainly used in cases of tumor rupture. However, liver transplantation is not advised. CONCLUSION: We presented an overview of the demographics, tumor characteristics and treatment outcomes of the largest number of primary hepatic angiosarcoma patients investigated to date. We highlight the use of routine physical examinations and surgery combined with adjuvant chemotherapy to improve the outcomes in these cases.


Assuntos
Hemangiossarcoma/patologia , Neoplasias Hepáticas/patologia , Idoso , Feminino , Hemangiossarcoma/diagnóstico por imagem , Humanos , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Recidiva Local de Neoplasia/patologia , Prognóstico , Estudos Retrospectivos
19.
Head Neck Pathol ; 14(1): 109-120, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31950467

RESUMO

This manuscript provides an overview of pleomorphic spindle cell tumors presenting on sun-damaged skin of the elderly and includes discussions of atypical fibroxanthoma, pleomorphic dermal sarcoma, spindle cell and metaplastic squamous cell carcinoma, spindle cell and dedifferentiated melanoma and poorly differentiated cutaneous angiosarcoma. These tumors share many of the clinical presenting and histological features, making confident diagnosis challenging. A reliable and robust diagnosis is necessary to predict behavior as the biologic potential of these tumors ranges from benign (e.g. atypical fibroxanthoma) to outright malignant with poor survival rates (e.g. cutaneous angiosarcoma). The salient clinical, histologic and immunohistochemical characteristics are discussed in detail with emphasis on distinguishing features and differential diagnosis to provide the reader with a better understanding of these entities and helpful clues for a more robust diagnosis.


Assuntos
Carcinoma de Células Escamosas/patologia , Fibroma/patologia , Hemangiossarcoma/patologia , Melanoma/patologia , Sarcoma/patologia , Neoplasias Cutâneas/patologia , Carcinoma de Células Escamosas/diagnóstico , Fibroma/diagnóstico , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/patologia , Hemangiossarcoma/diagnóstico , Humanos , Melanoma/diagnóstico , Sarcoma/diagnóstico , Neoplasias Cutâneas/diagnóstico
20.
J Am Anim Hosp Assoc ; 56(2): 120-126, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31961215

RESUMO

Canine hemangiosarcoma (HSA), a malignant neoplasm of vascular endothelial or bone marrow progenitor cell origin, most often affects the spleen, heart, and liver and typically has an aggressive biologic behavior. Canine HSA arising from the falciform fat/ligament represents a rare anatomic variant, with only two reports in the veterinary literature. In this study, we describe the clinical presentation, treatment, and outcome of seven dogs with primary HSA of the falciform ligament. Histologic grade and mitotic score were not significantly associated with outcome. All dogs had the primary tumor surgically excised except for one diagnosed at necropsy. Median overall survival for all dogs diagnosed prior to necropsy was 339 days, and the 1 yr survival rate was 50%. Four dogs were treated with adjuvant chemotherapy and had a significantly longer median overall survival (394 versus 83 days) than those that did not (P = .018). Dogs with HSA of the falciform ligament may have improved 1 yr survival rates and longer median survival time compared with dogs with HSA in more common visceral locations.


Assuntos
Doenças do Cão/patologia , Hemangiossarcoma/veterinária , Neoplasias Hepáticas/veterinária , Ligamento Redondo do Fígado/patologia , Animais , Antineoplásicos/uso terapêutico , Doenças do Cão/terapia , Cães , Feminino , Hemangiossarcoma/patologia , Hemangiossarcoma/terapia , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Masculino , Estudos Retrospectivos
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