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1.
Khirurgiia (Mosk) ; (6): 114-117, 2020.
Artigo em Russo | MEDLINE | ID: mdl-32573542

RESUMO

Patients with anterior mediastinum tumors fall into a difficult category due to high risk of invasion of vital structures and complexity of surgical correction. We report resection of recurrent hemangioma of anterior mediastinum with aortic arch replacement and simultaneous resection of left atrial myxoma in a 35-year-old woman. Successful treatment of these patients correlates with aggressive surgical approach followed by total resection of tumor and all affected vessels and other tissues.


Assuntos
Neoplasias Cardíacas/cirurgia , Hemangioma/cirurgia , Neoplasias do Mediastino/cirurgia , Mixoma/cirurgia , Recidiva Local de Neoplasia/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Adulto , Aorta Torácica/cirurgia , Implante de Prótese Vascular , Procedimentos Cirúrgicos Cardiovasculares/métodos , Feminino , Átrios do Coração/cirurgia , Humanos , Mediastino/cirurgia
2.
Kyobu Geka ; 73(5): 380-383, 2020 May.
Artigo em Japonês | MEDLINE | ID: mdl-32398397

RESUMO

A 44-year-old male presented to our hospital with exertional dyspnea. Transthoracic echocardiography revealed a large cystic mass in the left atrium obstructing the mitral valve orifice. Transesophageal echocardiography during emergency operation showed a large cystic mass along with a solid part near the stalk. The mass was resected en bloc including the margin of the left atrium around the stalk. His hemodynamics improved immediately after the operation and the patient was discharged shortly with uneventful postoperative course. While intra cardiac cystic mass has some possible pathologies including malignancy, pathological examination of this tumor revealed a myxoma with large hematoma which had probably grown up rapidly. This tumor was successfully eradicated under the guide of both transthoracic and transesophageal echocardiography.


Assuntos
Neoplasias Cardíacas , Mixoma , Adulto , Ecocardiografia Transesofagiana , Átrios do Coração , Humanos , Masculino , Valva Mitral
3.
Br J Radiol ; 93(1111): 20200255, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32401545

RESUMO

OBJECTIVE: To evaluate the imaging characteristics of aggressive angiomyxoma (AA) and identify features which would help to differentiate it from similar appearing lesions. METHODS: A retrospective review of departmental records was done and cases where AA was suspected on CT or MRI were included. With histopathology as gold-standard, the cases were grouped into AA or mimics and differentiating features were identified on USG, CT and MRI. Fischer's exact test was applied for the statistical significance of the differentiating features. RESULTS: 18 patients were identified of which 2 were excluded for lack of histopathology records. 10 were AA and 6 mimics which included 2 fibromatoses, 2 neurogenic tumors, and 1 each of germ cell tumor and inflammatory myofibroblastic tumor. On MRI, all AA showed T2 hyperintensity and intense contrast enhancement with characteristic laminated pattern in 7/9 cases. Diffusion restriction was seen in 2/3 cases, cystic component in 4/10 and hemorrhage in 1/10 cases. Pelvic fibromatosis was the closest imaging differential showing laminated pattern in one of the cases. CONCLUSION: Large pelvic mass with abdominal/perineal extension in reproductive age female patient should lead to suspicion of AA. Laminated pattern on T2 weighted images and intense homogeneous contrast enhancement further add to the diagnostic confidence. ADVANCES IN KNOWLEDGE: This study for the first time describes radiological mimics of AA. Large cystic component, diffusion restriction, hemorrhage and organ infiltration have not been previously described in AA.


Assuntos
Mixoma/patologia , Neoplasias Pélvicas/patologia , Neoplasias Abdominais/patologia , Adolescente , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia Computadorizada Multidetectores , Períneo , Estudos Retrospectivos , Adulto Jovem
4.
Adv Exp Med Biol ; 1226: 51-56, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32030675

RESUMO

Cardiac tumors are found in less than 1% of adult and pediatric autopsies. More than three-fourths of primary cardiac neoplasms are benign, with myxomas and rhabdomyomas being the most common cardiac tumors seen in adults and children, respectively. Primary malignant cardiac tumors are extremely rare, whereas metastatic lesions can be seen in approximately 8% of patients dying from cancer. Attempting to understand why the heart is so resistant to carcinogenesis and which fail-safe mechanisms malfunction when cardiac tumors do develop is particularly challenging considering the rarity of these tumors and the fact that when relevant clinical studies are published, they rarely focus on molecular pathogenesis. Apart from cancer cells, solid tumors are comprised of a concoction of noncancerous cells, and extracellular matrix constituents, which along with pH and oxygen levels jointly constitute the so-called tumor microenvironment (TME). In the present chapter, we explore mechanisms through which TME may influence cardiac carcinogenesis.


Assuntos
Carcinogênese , Neoplasias Cardíacas/patologia , Microambiente Tumoral , Humanos , Mixoma/patologia , Rabdomioma/patologia
7.
J Oral Pathol Med ; 49(3): 278-283, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32050038

RESUMO

BACKGROUND: The molecular pathogenesis of odontogenic myxoma has not been established yet. Considering that odontogenic myxoma may show myofibroblastic differentiation and myxoid areas can be observed in intra-osseous myofibromas, we tested the hypothesis whether both tumors share a common molecular profile. As recent studies have reported PDGFRB recurrent driver mutations in myofibroma, we evaluated PDGFRB mutations in odontogenic myxomas. METHODS: A convenience sample of 15 odontogenic myxomas cases was selected. We direct sequenced PDGFRB exons 12 and 14, where p.R561C (c.1681C>T) and p.N666K (c.1998C>G) hotspot mutations have been reported among others in single and/or multiple myofibromas. RESULTS: All 15 odontogenic myxoma samples were successfully sequenced, and all 15 had wild-type sequences for the PDGFRB mutations investigated. CONCLUSION: Our findings suggest that PDGFRB mutations do not play a role in odontogenic myxoma pathogenesis, which might be helpful in the differential diagnosis of challenging cases.


Assuntos
Miofibroma/genética , Mixoma/genética , Tumores Odontogênicos/genética , Receptor beta de Fator de Crescimento Derivado de Plaquetas/genética , Adulto , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Adulto Jovem
8.
J Neuropathol Exp Neurol ; 79(3): 347-351, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-32016322

RESUMO

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that arises primarily in the extremities of young adults. Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family have been reported in a diverse group of tumors, including AFH. AFH-like lesions have been reported to occur intracranially and the reported cases show low proliferation indices, frequently have a connection with the dura, and show recurrent EWSR1 rearrangements. These tumors have been termed intracranial myxoid mesenchymal tumor with EWSR1-CREB family gene fusions. A literature search identified 11 reported cases of intracranial AFH-like lesions with an EWSR1 rearrangement. Here, we report a case of intracranial myxoid mesenchymal tumor with an EWSR1-ATF1 fusion in an adult patient, and review the existing literature on this recently described entity.


Assuntos
Fator 1 Ativador da Transcrição/genética , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Células-Tronco Mesenquimais/patologia , Mixoma/genética , Mixoma/patologia , Proteína EWS de Ligação a RNA/genética , Idoso , Fusão Gênica , Histiocitoma Fibroso Maligno/genética , Histiocitoma Fibroso Maligno/patologia , Humanos , Masculino
10.
J Craniofac Surg ; 31(3): e236-e239, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31977681

RESUMO

Odontogenic myxoma is a benign tumor, mostly located in the mandible. It shows locally aggressive behavior and requires surgical removal. Common treatment options for reconstructing the bone defects are immediate or delayed autologous bone graft or free flap. In this article, the authors present the successful reconstruction with autogenous bone graft and autologous human bone marrow mesenchymal stem, followed by distraction osteogenesis, dental implant placement and prosthodontic restoration in the mandibular defect.


Assuntos
Células da Medula Óssea , Neoplasias Mandibulares/terapia , Reconstrução Mandibular , Mixoma/terapia , Tumores Odontogênicos/terapia , Transplante de Células-Tronco , Implantes Dentários , Humanos , Masculino , Neoplasias Mandibulares/patologia , Pessoa de Meia-Idade , Tumores Odontogênicos/patologia , Osteogênese por Distração , Células-Tronco
13.
World Neurosurg ; 134: e1053-e1061, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31760186

RESUMO

OBJECTIVE: Lesions affecting the sciatic nerve (SN) can mimic lumbar radiculopathy. In patients presenting with sciatica, approximately 10% have a nondiscogenic etiology. Through neurological examination and imaging, it may be possible to confirm nondiscogenic sciatica (NDS). This study aims to present a series of 6 patients with infragluteal NDS, highlighting clinical and imaging aspects that may suggest this diagnosis. METHODS: This is a retrospective study of 6 patients treated for NDS from 2010 to 2018. The mean and median ages were 41.2 and 38.5 years, respectively, with all patients female. RESULTS: All patients presented with sciatic pain, tenderness to deep infragluteal palpation, and a positive Tinel's sign related to the SN. Four patients were referred for surgical treatment, whereas 1 underwent pharmacological therapy and 1 underwent incisional biopsy and radiotherapy. In our series, 6 different causes for NDS were diagnosed: 1 nontumorous cause: extrauterine endometriosis and 5 tumors: metastasis from rectal adenocarcinoma, low-grade sarcoma, schwannoma, high-grade sarcoma, and myxoma. CONCLUSIONS: Differentiating between discogenic and NDS can be challenging for clinicians. When patients present with sciatic pain, a Tinel's sign related to the SN elicited at the deep infragluteal region, tenderness to deep infragluteal palpation, occasionally with an SN motor deficit and imaging findings of the lumbar spine that do not justify a discogenic source, the cause should be considered nondiscogenic and they should be scheduled for magnetic resonance imaging of the gluteal and pelvic region.


Assuntos
Adenocarcinoma/secundário , Endometriose/diagnóstico por imagem , Mixoma/diagnóstico por imagem , Neurilemoma/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias Retais/patologia , Sarcoma/diagnóstico por imagem , Ciática/etiologia , Adenocarcinoma/complicações , Adenocarcinoma/diagnóstico por imagem , Adulto , Endometriose/complicações , Endometriose/patologia , Feminino , Humanos , Imagem por Ressonância Magnética , Mixoma/complicações , Mixoma/patologia , Neurilemoma/complicações , Neurilemoma/patologia , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/patologia , Neoplasias do Sistema Nervoso Periférico/complicações , Neoplasias do Sistema Nervoso Periférico/patologia , Exame Físico , Estudos Retrospectivos , Sarcoma/complicações , Sarcoma/patologia , Nervo Isquiático/diagnóstico por imagem , Nervo Isquiático/patologia , Adulto Jovem
14.
World Neurosurg ; 133: e376-e384, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31521750

RESUMO

OBJECTIVE: We present a case of multiple myxoma-related intracranial aneurysms and reviewed the recent relevant literature to investigate their natural course and develop a reasonable treatment algorithm. METHODS: We have reported a new case of multiple myxoma-related intracranial aneurysms and performed a search of current studies (2001 to the present) in PubMed. The keywords used were as follows (single words or combinations): neoplastic aneurysm, oncotic aneurysm, aneurysm, and myxoma. Only patients with multiple aneurysms resulting from cardiac myxoma and with adequate follow-up information pertinent to the analysis were included. RESULTS: A total of 41 patients, including our present patient, were studied. Of the 41 patients, 28 had received conservative treatment as the primary choice. Most lesions (n = 22) were stable during follow-up, except for 3 in which aneurysm evolution was observed. A total of 8 patients had undergone microsurgery, including aneurysm resection in 3, aneurysm clipping in 2, clipping followed by resection in 2, and aneurysm trapping in 1. Endovascular treatment was performed in 2 patients. Radiation therapy was used in 1 patient, and the parent artery proved to be occluded at the 1-year follow-up examination. A combination of chemotherapy and microsurgery was used in 1 patient. All the cases that had been managed aggressively were stable during further follow-up. CONCLUSION: The prognosis was good for most patients with multiple myxoma-related intracranial aneurysms, and most myxoma-related aneurysms were stable. Conservative treatment and routine follow-up are recommended for most patients. However, for patients with evolving or ruptured aneurysms, invasive treatment, including open surgery and endovascular treatment, should be considered.


Assuntos
Neoplasias Cardíacas/complicações , Aneurisma Intracraniano/etiologia , Mixoma/complicações , Adolescente , Adulto , Idoso , Angiografia Digital , Criança , Tratamento Conservador , Feminino , Humanos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Aneurisma Intracraniano/terapia , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Adulto Jovem
16.
Cardiovasc Pathol ; 45: 107183, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31865270

RESUMO

Cardiac myxoma is the most frequently encountered primary neoplasm of the heart; however, other tumefactive lesions can share similar radiologic features. We present briefly the case of a 69-year-old man incidentally found to have a mobile right atrial mass that based on initial radiologic findings was considered to represent a myxoma. After pathologic examination, the lesion was determined instead to be a cardiac varix: an endocardial, blood filled cystic space lined by endothelium and considered to represent a dilated vein.


Assuntos
Vasos Coronários/patologia , Cardiopatias/patologia , Neoplasias Cardíacas/patologia , Mixoma/patologia , Varizes/patologia , Idoso , Biópsia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Erros de Diagnóstico , Dilatação Patológica , Ecocardiografia Transesofagiana , Cardiopatias/diagnóstico por imagem , Cardiopatias/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Humanos , Masculino , Mixoma/diagnóstico por imagem , Valor Preditivo dos Testes , Tomografia Computadorizada por Raios X , Varizes/diagnóstico por imagem , Varizes/cirurgia
17.
J Card Surg ; 35(2): 511-513, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31856315

RESUMO

Myxomas are the most common cardiac tumors, benign, and usually located in the left atrium. Typically echocardiography reveals a solid tumor, whereas cystic myxomas are rare with only a few cases documented in the literature. We describe the case of a 63-year-old, female patient with an unusual presentation of a left atrial myxoma as a cystic tumor.


Assuntos
Cistos/etiologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/patologia , Mixoma/complicações , Mixoma/patologia , Cistos/diagnóstico por imagem , Cistos/patologia , Ecocardiografia Transesofagiana , Feminino , Átrios do Coração , Neoplasias Cardíacas/diagnóstico por imagem , Humanos , Pessoa de Meia-Idade , Mixoma/diagnóstico por imagem , Tomografia Computadorizada por Raios X
18.
J Card Surg ; 35(2): 507-510, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31872924

RESUMO

BACKGROUND: Cardiac myxoma is the most common type of primary cardiac tumors. It is known that inflammatory markers are increased in the setting of myxoma, like C-reactive protein, erythrocyte sedimentation rate, and interleukin-6. The association between cardiac myxoma and abnormal blood cell counts has been scarcely reported. RESULTS: We present a patient with a right atrial myxoma found incidentally, who had hypereosinophilia, which dramatically resolved after resection of the tumor. CONCLUSION: Hypereosinophilia has mostly been found in patients with heart malignancies. It is extremely uncommon in patients with cardiac myxoma; therefore, its presence may signify a distinct biological tumoral behavior with the potential to become a marker of disease activity or recurrence. The true significance of this finding is still unknown and requires further investigation.


Assuntos
Eosinofilia/complicações , Eosinofilia/cirurgia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/cirurgia , Mixoma/complicações , Mixoma/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Átrios do Coração , Humanos , Achados Incidentais , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
19.
Medicine (Baltimore) ; 98(51): e18386, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31861001

RESUMO

RATIONALE: Pulmonary embolisms (PEs) are caused by emboli, which mostly originate from deep venous thrombi that travel to and suddenly block the pulmonary arteries. The emboli are usually thrombi, and right atrial myxoma emboli are rare. PATIENT CONCERNS: A 55-year-old man presented with shortness of breath and syncope. We proceeded with computed tomography pulmonary angiography (CTPA) and transthoracic echocardiogram (TTE), the results of which suggested that the diagnosis was a right atrial mass. DIAGNOSIS: A definitive diagnosis compatible with a right atrial myxoma (RAM) with tumoral pulmonary emboli after surgical excision was made. INTERVENTION: Right atrial and pulmonary artery embolectomy. OUTCOMES: The patient followed an uneventful course during the 6 years of follow-up after surgery. According to a review of the literature, RAMs are often not diagnosed in a timely manner or even go completely undiagnosed. TTE, transesophageal echocardiography (TEE), CT, magnetic resonance imaging (MRI), and positron emission tomography/computed tomography may be helpful in the preoperative diagnosis. Surgical removal of the masses from the atrium and pulmonary arteries was relatively uneventful. LESSONS: RAMs should be considered unlikely reasons for fatal pulmonary embolisms.


Assuntos
Átrios do Coração/diagnóstico por imagem , Neoplasias Cardíacas/diagnóstico por imagem , Mixoma/diagnóstico por imagem , Embolia Pulmonar/etiologia , Angiografia por Tomografia Computadorizada , Ecocardiografia , Átrios do Coração/cirurgia , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Mixoma/patologia , Mixoma/cirurgia , Embolia Pulmonar/cirurgia
20.
J Med Case Rep ; 13(1): 389, 2019 Dec 25.
Artigo em Inglês | MEDLINE | ID: mdl-31874650

RESUMO

BACKGROUND: Atrial myxomas are generally considered benign neoplasms. The majority of tumors are sporadic and less than 10% are associated with an autosomal dominant condition known as the Carney complex, which is most often caused by germline mutation in the gene PRKAR1A. Whether this gene plays a role in the development of sporadic myxomas has been an area of debate, although recent studies have suggested that some fraction of sporadic tumors also carry mutations in PRKARIA. Extra-cardiac complications of atrial myxoma include dissemination of tumor to the brain; however, the dissemination of viable invasive tumor cells is exceedingly rare. CASE PRESENTATION: We present here a 48-year-old white woman who developed multiple intracranial hemorrhagic lesions secondary to tumor embolism that progressed to 'false' aneurysm formation and invasion through the vascular wall into brain parenchyma 7 months after resection of an atrial myxoma. Whole exome sequencing of her tumor revealed multiple mutations in PRKAR1A not found in her germline deoxyribonucleic acid (DNA), suggesting that the myxoma in this patient was sporadic. CONCLUSIONS: Our patient illustrates that mutations in PRKAR1A may be found in sporadic lesions. Whether the presence of this mutation affects the clinical behavior of sporadic tumors and increases risk for metastasis is not clear. Regardless, the protein kinase A pathway which is regulated by PRKAR1A represents a possible target for treatment in patients with metastatic cardiac myxomas harboring mutations in the PRKARIA gene.


Assuntos
Neoplasias Encefálicas/secundário , Complexo de Carney/diagnóstico , Subunidade RIalfa da Proteína Quinase Dependente de AMP Cíclico/genética , Dopaminérgicos/uso terapêutico , Neoplasias Cardíacas/diagnóstico , Memantina/uso terapêutico , Mixoma/diagnóstico , Neoplasias Encefálicas/fisiopatologia , Neoplasias Encefálicas/terapia , Complexo de Carney/genética , Quimiorradioterapia , Feminino , Regulação Neoplásica da Expressão Gênica , Genes Supressores de Tumor , Mutação em Linhagem Germinativa , Neoplasias Cardíacas/fisiopatologia , Neoplasias Cardíacas/terapia , Humanos , Hemorragias Intracranianas , Pessoa de Meia-Idade , Mixoma/fisiopatologia , Mixoma/terapia , Resultado do Tratamento , Sequenciamento Completo do Exoma
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