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1.
Rev Med Suisse ; 19(817): 464-470, 2023 Mar 08.
Artigo em Francês | MEDLINE | ID: mdl-36883707

RESUMO

Cardiac tumors are rare in clinical practice but remain an essential part of the fast-growing field of cardio-oncology. They can be detected incidentally and consist of primary tumors (benign or malignant) and of the more prevalent secondary tumors (metastasis). They form a heterogeneous group of pathologies presenting with a wide panel of clinical symptoms according to their location and size. Multimodality cardiac imaging (echocardiography, CT, MRI and PET), in association with clinical and epidemiological factors, plays a key role in the diagnosis of cardiac tumors and a biopsy is therefore not systematically required. Treatment strategies for cardiac tumors vary depending on the malignancy and class of the tumor, but also consider associated symptoms, hemodynamic impact and embolic risk.


Les tumeurs cardiaques sont des pathologies rares mais elles ­appartiennent à un domaine en plein essor de la cardio-oncologie. Souvent découvertes fortuitement, elles comprennent les ­tumeurs cardiaques primaires (bénignes ou malignes) et, plus fréquemment, secondaires (métastases). Elles constituent un ­ensemble très hétérogène de pathologies, dont les manifestations cliniques varient en fonction de la taille et de la localisation. Le diagnostic repose sur une approche clinique, épidémiologique et plusieurs modalités d'imagerie (échocardiographie, CT, IRM et PET-CT), sans nécessité systématique de biopsie. La stratégie de traitement d'une tumeur cardiaque dépend de la symptomatologie du patient, du risque embolique ou hémodynamique lié à la masse, ainsi que de sa malignité.


Assuntos
Neoplasias Cardíacas , Humanos , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/terapia , Coração , Biópsia , Técnicas de Imagem Cardíaca , Ecocardiografia
2.
J Card Surg ; 37(9): 2653-2660, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35662249

RESUMO

BACKGROUND: Treatment of benign primary cardiac tumors involves surgical resection, but reported outcomes from multi-institutional or national databases are scarce. This study examines contemporary national outcomes following surgical resection of benign primary atrial and ventricular tumors. METHODS: The 2016-2018 Nationwide Readmissions Database was queried for all patients ≥18 years with a primary diagnosis of benign neoplasm of the heart who underwent resection of the atria, ventricles, or atrial/ventricular septum. Primary outcomes were 30-day mortality, readmission, and composite morbidity (defined as stroke, permanent pacemaker implantation, bleeding complication, or acute kidney injury). Multivariable analysis was used to identify independent predictors of worse outcomes. RESULTS: A weighted total of 2557 patients met inclusion criteria. Mean age was 61 years, 67.9% were female, and patients had relatively low comorbidity burdens (mean Charlson Comorbidity Index 1.39). The majority of patients underwent excision of the left atrium (71.5%), followed by the intra-atrial septum (26.6%), right atrium (2.9%). There was no difference in 30-day mortality (2.1% vs. 1.3%, p = .550), 30-day readmission (7.0% vs. 9.1%, p = .222), or 30-day composite morbidity (56.8% vs. 53.8%, p = .369) between females and males, respectively. However, on multivariable analysis, female sex was independently associated with increased risk of 30-day mortality (adjusted odds ratio = 2.65, p = .028). Tumor location (atria, ventricles, septum) was not predictive of mortality. CONCLUSION: Benign atrial and ventricular tumors are uncommon, but disproportionately impact female patients, with female sex being an independent predictor of 30-day mortality. Root-cause analysis is necessary to determine the ultimate cause of this disparity.


Assuntos
Neoplasias Cardíacas , Readmissão do Paciente , Comorbidade , Bases de Dados Factuais , Feminino , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Risco
3.
J Card Surg ; 37(5): 1275-1286, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35226369

RESUMO

INTRODUCTION: Despite the significant clinical importance of sex among factors affecting cancer progression and survival, it remains one of the least studied factors. Therefore, we sought to examine these differences in relation to primary malignant cardiac tumors (PMCTs) using a national data set. METHODS: The 2004-2017 National Cancer Database was queried for patients with PMCTs. Annual trend of females' percent was assessed. Overall survival predictors were evaluated with Kaplan-Meier and Cox-regression. Subgroup analysis was done based on histology, comorbidity index, race, insurance, and surgical treatment. RESULTS: PMCTs were identified in 736 patients (median age 52, female [47.8%]). Most of them were high-grade (49.2%). About 60% underwent surgery. Angiosarcoma (43%), fibrosarcoma (5.2%), and leiomyosarcoma (5.2%) were the most common pathologies. Based on multivariate Cox-regression, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were associated with higher late mortality, while year of diagnosis and use of surgery or chemotherapy were associated with lower mortality. Among the surgical group, age, higher income, higher comorbidity index, angiosarcoma, and Stage III/IV were independent predictors of higher late mortality, while private insurance and year of diagnosis were associated with lower late mortality. No difference was seen between males and females in 30-day and late mortality (p = .71). Subgroup analysis based on Cox-regression showed no differences in late mortality between males and females. CONCLUSION: PMCTs have poor overall survival. Surgery and chemotherapy were associated with longer survival benefits. On the contrary, the associated risk factors for mortality were advanced age, higher comorbidity index, angiosarcoma histology, and Stage III/IV.


Assuntos
Neoplasias Cardíacas , Hemangiossarcoma , Estudos de Coortes , Bases de Dados Factuais , Feminino , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/cirurgia , Hemangiossarcoma/epidemiologia , Hemangiossarcoma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Caracteres Sexuais
4.
Genet Med ; 23(1): 80-85, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32893266

RESUMO

PURPOSE: Carney complex (CNC), is an autosomal dominant multiple neoplasia and lentiginosis syndrome. We aimed to identify risk factors associated with the occurrence and recurrence of cardiac myxomas, the predominant cause of death in CNC patients. METHODS: Patients with CNC were monitored prospectively between 1995 and 2020 for the development of cardiac myxomas. RESULTS: Of the 319 patients studied, 136 (42.6%) developed myxomas. The mean age at diagnosis was 28.7 ± 16.6 years in females and 25.0 ± 16.4 years in males. By age 30, 35% of females and 45% of males had at least one myxoma. The CNC-related lesions, lentigines, cutaneous, mucosal, or breast myxomas, thyroid nodules, pituitary adenoma, and schwannoma were significantly more frequent (all p < 0.05) among patients with myxomas. Forty-four percent of patients had recurrences; nearly all within the first 8 and 16 years for males and females, respectively. Recurrences were more common in females. CONCLUSION: This is the largest study to date and provides the first-time risk estimates by age and gender for cardiac myxomas in CNC patients. Cardiac myxomas are common by age 30 and often recur, especially in women, but the risk drops in 10 to 20 years. These findings may guide patient counseling, screening intervals, and surgical approaches. CLINICAL TRIAL REGISTRATION: Clinical Trial Registration: Defining the Genetic Basis for the Development of Primary Pigmented Nodular Adrenocortical Disease and the Carney complex, Registration number: NCT00001452 URL: https://clinicaltrials.gov/ct2/show/NCT00001452.


Assuntos
Complexo de Carney , Neoplasias Cardíacas , Mixoma , Adulto , Complexo de Carney/diagnóstico , Complexo de Carney/epidemiologia , Complexo de Carney/genética , Feminino , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/genética , Humanos , Masculino , Mixoma/diagnóstico , Mixoma/epidemiologia , Mixoma/genética , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/genética , Fatores de Risco
5.
Cardiology ; 146(6): 748-753, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34469887

RESUMO

Cardiac neoplasms are uncommon tumors. For epidemiological purposes, they can be divided into benign and malignant subtypes, with the former occurring at a significantly higher rate than the latter. Due to their uncommon nature, there are few data-driven studies examining the characteristics and trends of benign cardiac neoplasms. Our retrospective HCUP-NIS data review purports to illuminate some of the trends surrounding benign cardiac neoplasms and their associated co-occurrences. The data consisted of 482,872,274 weighted discharges. There were 45,568 weighted discharges that included a benign cardiac neoplasm. Benign cardiac neoplasms were more often observed in women (64.33%), and the average age was 63.8 years. The most common cardiovascular co-occurrences in patients with benign cardiac neoplasm were atrial tachyarrhythmias (28.93%), heart failure (19.61%), and embolic events such as stroke, myocardial infarct, or pulmonary embolism (19.82%). Other co-occurrences included pulmonary hypertension (7.55%), ventricular arrhythmias (3.23%), and other EKG abnormalities (3.70%). Procedures were numerous in patients with benign cardiac neoplasms. 43% of patients with this diagnosis had some form of cardiac surgery during their hospitalization. Overall, this study found low incidence of benign cardiac neoplasms in the USA during this 13-year study period. However, in the presence of benign cardiac neoplasms, our study showed that cardiovascular co-occurrences are not uncommon and may help to illuminate this otherwise rare diagnosis.


Assuntos
Insuficiência Cardíaca , Neoplasias Cardíacas , Infarto do Miocárdio , Feminino , Átrios do Coração , Neoplasias Cardíacas/epidemiologia , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Estados Unidos/epidemiologia
6.
J Card Surg ; 36(10): 3586-3592, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34314042

RESUMO

INTRODUCTION: Primary cardiac tumors (PCT) are rare, and their contemporary outcomes are not well characterized in the literature. We assessed temporal trends in patient characteristics and management of admissions for PCT in US hospitals. METHODS: Admissions with the principal diagnoses of a PCT (benign neoplasm of heart: ICD-9 212.7, ICD-10 D15.1; malignant neoplasm of heart: ICD-9 164.1, ICD-10 C38.0) between 2006 and 2017 were extracted from the National Inpatient Sample. Trends in demographics and clinical profiles were evaluated. We conducted descriptive analyses on the cohort and compared outcomes between those managed medically and surgically. RESULTS: Between 2006 and 2017, 19,111 admissions had the primary diagnosis of a PCT. Of these, 91.1% were benign. Admissions were mostly female (65.0%), caucasian (72.0%), and aged more than 50 years (76.0%). The annual admission rate for PCT was similar from 2006 to 2017 (p trend > .05) and associated with congestive heart failure, diabetes, renal failure, and valvular lesions. PCTs were managed surgically in 12,811 (67.0%) of overall cases, 70.8% for benign and 28.3% for malignant tumors. Overall, the in-hospital mortality rate was 2.3%. Medically managed cases reported a 2.5% higher mortality (p < .001) than those surgically managed. Admissions with malignant tumors were more likely to expire during hospitalization than those with benign tumors (odds ratio, 9.75; 95% confidence interval 6.34-14.99; p < .001). CONCLUSION: Admissions for primary cardiac tumors were primarily women or in their fifth or sixth decade of life. Surgical intervention is more commonly practiced and is associated with better in-hospital survival.


Assuntos
Insuficiência Cardíaca , Neoplasias Cardíacas , Feminino , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/cirurgia , Mortalidade Hospitalar , Hospitalização , Humanos , Pacientes Internados , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
7.
Hematol Oncol ; 38(3): 334-343, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32311106

RESUMO

This study aimed to evaluate the incidence and prognosis of primary cardiac lymphoma (PCL) by using the Surveillance, Epidemiology, and End Results Program (SEER) database. Patients diagnosed with PCL and the disease incidence in the SEER database from 1975 to 2016 were included. Overall survival (OS) and cause-specific survival (CSS) curves were calculated using the Kaplan-Meier method and compared by the log-rank test. Univariate and multivariable Cox proportional hazard regression analyses were used to identify associations with outcome measures. The incidence of PCL was 0.011/100 000, and a predominance of elderly and male patients was observed. A total of 144 patients were enrolled. The median age of onset was 68 (9-96) years, including 80 (55.6%) males and 64 (44.4%) females. Multivariate analysis revealed that age and chemotherapy were independent prognostic factors for OS (both P < .05). Ann Arbor stage and chemotherapy were independent prognostic factors for CSS (both P < .05). In terms of treatment modality, chemotherapy combined with surgery was an independent protective factor for OS and CSS (both P < .05). For patients with primary cardiac diffuse large B-cell lymphoma (cardiac DLBCL), multivariate analysis also showed that age, Ann Arbor stage, and chemotherapy were all independent prognostic factors for OS and CSS (all P < .05). Chemotherapy combined with surgery was associated with a significant benefit in terms of OS and CSS (both P < .05). Our study confirmed that older age and advanced Ann Arbor stage were independent risk factors for PCL, and treatment with chemotherapy or cooperation with surgery resulted in better long-term survival.


Assuntos
Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/mortalidade , Linfoma Difuso de Grandes Células B/epidemiologia , Linfoma Difuso de Grandes Células B/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Terapia Combinada , Feminino , Seguimentos , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/terapia , Humanos , Incidência , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Programa de SEER , Taxa de Sobrevida , Estados Unidos/epidemiologia , Adulto Jovem
8.
Curr Cardiol Rep ; 22(12): 169, 2020 10 10.
Artigo em Inglês | MEDLINE | ID: mdl-33040219

RESUMO

PURPOSE OF REVIEW: Cardiac masses frequently present significant diagnostic and therapeutic clinical challenges and encompass a broad set of lesions that can be either neoplastic or non-neoplastic. We sought to provide an overview of cardiac tumors using a cardiac chamber prevalence approach and providing epidemiology, imaging, histopathology, diagnostic workup, treatment, and prognoses of cardiac tumors. RECENT FINDINGS: Cardiac tumors are rare but remain an important component of cardio-oncology practice. Over the past decade, the advances in imaging techniques have enabled a noninvasive diagnosis in many cases. Indeed, imaging modalities such as cardiac magnetic resonance, computed tomography, and positron emission tomography are important tools for diagnosing and characterizing the lesions. Although an epidemiological and multimodality imaging approach is useful, the definite diagnosis requires histologic examination in challenging scenarios, and histopathological characterization remains the diagnostic gold standard. A comprehensive clinical and multimodality imaging evaluation of cardiac tumors is fundamental to obtain a proper differential diagnosis, but histopathology is necessary to reach the final diagnosis and subsequent clinical management.


Assuntos
Neoplasias Cardíacas , Imageamento por Ressonância Magnética , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/epidemiologia , Humanos , Imagem Multimodal , Tomografia por Emissão de Pósitrons , Prognóstico
9.
Curr Treat Options Oncol ; 20(8): 66, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31250250

RESUMO

OPINION STATEMENT: Cardiac masses and tumors are a heterogenous group of disorders and include primary tumors (both benign and malignant), metastatic disease, and numerous masquerades such as thrombus. Clinical presentation ranges from incidental discovery on imaging tests ordered for other reasons to life-threatening presentations such as cardiac tamponade, arrhythmia, obstruction, and systemic embolization. Of the available imaging modalities, cardiac MRI is generally the most useful for assessment and helps to delineate the relevant anatomy. Due to the technical difficulties and risk of biopsy of cardiac masses, a presumptive diagnosis is typically made using imaging techniques with surgery serving both a diagnostic and curative role. Because these conditions can vary widely in their management, we recommend early involvement of a multidisciplinary group which should include a cardiologist, cardiac surgeon, and oncologist.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/terapia , Biópsia , Terapia Combinada , Diagnóstico Diferencial , Diagnóstico por Imagem , Gerenciamento Clínico , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/etiologia , Humanos , Incidência , Gradação de Tumores , Estadiamento de Neoplasias , Avaliação de Sintomas , Resultado do Tratamento
10.
Zhonghua Bing Li Xue Za Zhi ; 48(4): 293-297, 2019 Apr 08.
Artigo em Zh | MEDLINE | ID: mdl-30955265

RESUMO

Objective: To study the clinicopathological characteristics of cardiac neoplasms. Methods: A total of 689 cases of cardiac neoplasms from January 1st 1992 to December 31th 2017 at Guangdong Provincial People's Hospital were collected. The clinical data and histologic features were analyzed along with a review of literature. The pathological diagnosis and classification were based on the criteria of WHO 4th edition(2015). Results: Among 689 cases of cardiac neoplasms, 259 were male and 430 were female patients, with age from 0 to 84 years (mean of 48 years). The peak incidence was between the fourth and sixth decade. Among patients younger than 20 years, there were 24 males and 12 females. 674 cases(674/689,97.8%)were primary cardiac tumors and 15 cases were secondary tumors (15/689,2.2%). Amongst the primary cardiac neoplasms, 625 cases were benign(625/674,92.7%), 7 cases were borderline (7/674, 1.0%), and 42 cases were malignant (42/674, 6.2%). The incidences of benign, borderline and malignancy heart tumors among patients below 20 years old were lower than those of patients over 20 years of age (4.8% vs. 95.2%; 3/9 vs. 6/9; 5.5% vs. 94.5%, respectively). Of the benign tumors, 406 cases were female and 219 cases were male. More male than female patients were seen in borderline and malignancy cardiac tumor categories (6∶3; 34∶21). Of 625 benign tumors, 577 cases were myxoma(85.6%), which mainly occurred in patients over 20 years of age(85.9% vs. 14.1%) with a female predominance. Non-myxomas mainly occurred in children and adolescent patients compared to adult (55.6% vs. 44.4%, P<0.01) with a male predominance. Overall, 524 tumors originated from the left atrium, 84 cases from the right atrium, 26 cases from the pericardium, 23 cases from the right ventricular, and 11 cases from the left ventricle. However, 21 cases were multicentric or involving cardiac valves. Benign tumors mainly involved left heart(76.3%) vs. right heart(81/625, 12.6%). The mostly common location of borderline tumors was right heart(5/9). Malignant tumors tended to involve the right heart(22/55,40.0%) and pericardium(18/55, 32.7%). Conclusions: Although the incidence of cardiac neoplasms is low,various tumor types can occur, most of which are myxoma with a female predominance. Non-myxomas mainly occur in children and adolescents with a male predominance.


Assuntos
Neoplasias Cardíacas/patologia , Mixoma/patologia , Adolescente , Adulto , Distribuição por Idade , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , China/epidemiologia , Feminino , Átrios do Coração/patologia , Neoplasias Cardíacas/epidemiologia , Ventrículos do Coração , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Mixoma/epidemiologia , Distribuição por Sexo , Adulto Jovem
11.
BMC Cancer ; 18(1): 202, 2018 02 20.
Artigo em Inglês | MEDLINE | ID: mdl-29463229

RESUMO

BACKGROUND: To evaluate the prevalence of metastatic tumors involving the myocardium and study their presentation in order to increase awareness to their existence. METHODS: Pathological reports from Sheba Medical Center (Israel, January 1, 2010 through December 31, 2015) and medical records from The Institute for Cardiovascular Diseases of Vojvodina, Sremska Kamenica (Serbia, 23 years period) were screened for cases of metastatic cardiac tumors. Medical, radiological and pathological data of identified cases was retrieved and reviewed. RESULTS: Out of thousands of registered cardiac surgeries we found less than a dozen cases of metastatic cardiac tumors classified as melanoma, carcinomas of lung, colon and kidney and sarcomas of uterine origin. We found that metastatic cardiac tumors comprised 15.8% of all the cardiac tumors. CONCLUSIONS: Metastatic cardiac tumors are extremely rare. As new diagnostic technologies and improved survival of oncological patients may increase the incidence of metastatic cardiac tumors in the future, awareness to their existence and knowledge of their presentation are key factors in their timely recognition.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/secundário , Neoplasias Cardíacas/terapia , Adulto , Idoso , Biópsia , Terapia Combinada , Evolução Fatal , Feminino , Neoplasias Cardíacas/epidemiologia , Humanos , Masculino , Melanoma/patologia , Pessoa de Meia-Idade , Imagem Multimodal , Miocárdio/patologia , Neoplasias Embrionárias de Células Germinativas , Vigilância da População , Prevalência , Resultado do Tratamento
12.
J Card Surg ; 33(8): 432-437, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29992619

RESUMO

BACKGROUND: Primary cardiac adipose tumors are rare. There are two distinct pathologically defined entities that represent this tumor type: lipoma and lipomatous hypertrophy of the interatrial septum (LHIS). We present a single-center experience with these tumors and demonstrate that the location may not correspond to the pathologic diagnosis. METHODS: A retrospective review of a prospectively collected cardiac surgery database from January 1990 to July 2016 identified 254 cases of surgically treated primary cardiac tumors at our Institution. Of these, 06/254 (2%) were primary adipose tumors. RESULTS: In 3/6 (50%) cases, patients were asymptomatic or had symptoms referable to other known intracardiac lesions. Five patients (83%) had preserved ventricular function. In 4/6 cases (67%), the tumor was identified preoperatively. All patients presented in New York Heart Association functional class ≤2. Pathologic diagnosis of LHIS was made in 5/6 cases (83%), with 2/5 LHIS (40%) located in the interatrial septum. A bovine pericardial patch was utilized for reconstruction following tumor resection in 3/6 cases (50%). Mean cardiopulmonary bypass time was 88 ± 43 min. All the patients tolerated the procedure well without any postoperative complications. CONCLUSIONS: Primary cardiac adipose tumors are responsible for a small portion of all primary heart tumors. Surgical resection provided excellent outcomes, and did not affect cardiac performance, in spite of the need for extensive resections. LHIS was identified in locations other than the interatrial septum and was usually symptomatic.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/patologia , Lipoma/diagnóstico , Lipoma/patologia , Idoso , Procedimentos Cirúrgicos Cardíacos/métodos , Feminino , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/cirurgia , Septos Cardíacos , Humanos , Lipoma/epidemiologia , Lipoma/cirurgia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
13.
Ann Dermatol Venereol ; 145(12): 741-748, 2018 Dec.
Artigo em Francês | MEDLINE | ID: mdl-30217683

RESUMO

AIM: To describe in a large paediatric cohort the characteristics of hypopigmented and depigmented (hypochromatic and achromic) macules with no clear diagnosis but potentially evocative of tuberous sclerosis (TS). PATIENTS AND METHODS: This was a retrospective multicentre study performed between 2010 and 2017 at a reference centre for rare skin diseases; it included all children consulting for hypochromic and achromic macules. A descriptive analysis was made of the characteristics of macules with no clear diagnosis, enabling them to be classified in three secondary groups: TS certain, TS ruled out, TS uncertain. RESULTS: Of the 3300 children seen during this 7-year period 7,265 were consulting for hypochromic or achromic macules, with no clear diagnosis in 18 cases: 7 girls and 11 boys of median age at 7.21 years (range: 4 months to 16 years and 7 months). The lesions were congenital in 7 cases. The number of macules varied, with over 20 in some cases. The majority were in the form of ash-leaf spots, followed by the oval form. Two children were diagnosed at clinical examination, and 16 underwent it is not examinations, resulting in a diagnosis of certain ST in 6 of these cases. No particular characteristics of the macules appeared to guide the clinical examination towards ST or isolated lesions. Café-au-lait spots were more frequent in the group in which ST was ruled out than in the other two groups: 67% vs. 33% and 33%. Neurologic involvement was more common in children with certain or uncertain ST than in children in whom ST was ruled out (83% and 67% vs. 11%). CONCLUSION: No identified characteristics of stains enabled the clinical examination to confirm or rule out tuberous sclerosis. Screening for acute any signs of ST is essential. Diagnostic efficacy is enhanced by additional exams.


Assuntos
Hipopigmentação/etiologia , Esclerose Tuberosa/epidemiologia , Adolescente , Angiomiolipoma/epidemiologia , Criança , Transtornos do Comportamento Infantil/etiologia , Pré-Escolar , Deficiências do Desenvolvimento/etiologia , Diagnóstico Diferencial , Epilepsia/etiologia , Feminino , Neoplasias Cardíacas/epidemiologia , Humanos , Hipopigmentação/congênito , Lactente , Neoplasias Renais/epidemiologia , Masculino , Estudos Retrospectivos , Rabdomioma/epidemiologia , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico
14.
Eur J Pediatr ; 176(2): 253-260, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28074279

RESUMO

The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died. CONCLUSION: The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.


Assuntos
Fibroma/diagnóstico , Neoplasias Cardíacas/diagnóstico , Mixoma/diagnóstico , Rabdomioma/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Fibroma/epidemiologia , Fibroma/cirurgia , Seguimentos , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/cirurgia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Mixoma/epidemiologia , Mixoma/cirurgia , Estudos Retrospectivos , Rabdomioma/epidemiologia , Rabdomioma/cirurgia
15.
Herz ; 42(7): 690-695, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27909765

RESUMO

OBJECTIVE: Cardiac masses comprise a category that includes benign, malignant, and nontumoral mass lesions. The present study aims to share the findings relating to cardiac masses observed at our tertiary cardiology hospital in Turkey. METHODS: The records of patients who presented with cardiac masses and underwent surgery at our institute between 2006 and 2015, and whose tissue samples were sent to a pathology laboratory, were retrospectively reviewed in a consecutive manner. RESULTS: The study included 228 patients with an average age of 52.5 ± 17.3 years. Of the study's subjects, 95 (41.7%) were male and 133 (58.3%) female. The most commonly observed mass was myxoma in 68 patients (29.8%), of whom 20 (29.4%) were male and 48 (70.6%) female. The second most frequently detected mass was pannus, with 38 cases (16.7%) - 10 (26.3%) in males and 28 (73.7%) in females. The third most common cardiac mass was thrombus (16.2%), with 18 cases of thrombi in men (48.6%) and 19 (51.4%) in women. CONCLUSION: The most commonly observed cardiac mass was myxoma and most were localized in the left atrium. The second most frequently detected mass was pannus, which was mostly found on mitral mechanical prosthetic valves. Thrombi were the third most prevalent mass and were commonly localized in the right atrium.


Assuntos
Neoplasias Cardíacas/diagnóstico , Tecido Adiposo/patologia , Tecido Adiposo/cirurgia , Adulto , Idoso , Biópsia , Diagnóstico Diferencial , Feminino , Átrios do Coração/patologia , Átrios do Coração/cirurgia , Cardiopatias/diagnóstico , Cardiopatias/epidemiologia , Cardiopatias/patologia , Cardiopatias/cirurgia , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Mixoma/diagnóstico , Mixoma/epidemiologia , Mixoma/patologia , Mixoma/cirurgia , Prevalência , Estudos Retrospectivos , Trombose/diagnóstico , Trombose/epidemiologia , Trombose/patologia , Trombose/cirurgia
16.
Wien Med Wochenschr ; 167(11-12): 271-275, 2017 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-27812765

RESUMO

BACKGROUND: Tuberous sclerosis complex (TSC) disease is a rare genetic, multi-organ disorder characterized by the occurrence of multiple hamartoma. METHODS: In cooperation with ESPED, Germany, a prospective, epidemiological study was performed to assess the incidence of newly diagnosed TSC disease in patients ≤18 years in Germany. Moreover, the following parameters were assessed: 1. Age distribution at initial diagnosis; 2. Percentage of patients with in utero diagnosis of TSC; 3. Detailed description of pathological clinical findings; 4. Results from genetic testing. RESULTS: In this one-year interim analysis, 84 electronic questionnaires were received, 17 of which did not contain complete sets of data and were not included in data analysis. Twenty-three of 67 questionnaires did not report TSC patients and 3 reports contained redundant data sets and were excluded. In total, 41 reports were included into data analysis (female: 23; male: 18); median age at first diagnosis was 6 months (range: 0-151 months). The three most common symptoms were: central nervous affection: 31/41 patients ((75.6 %); 29/31 with seizures); rhabdomyoma: in 20/41 (48.8 %); cutaneous affection: hypomelanotic maculae ("white spots"): 20/41 (48.8 %). The three following organ manifestations were seen most often in a comprehensive diagnostic work-up: rhabdomyoma: 23/41 ((56.1 %); cortical dysplasia: 22/41 (53.7 %); "white spots"): 20/41 (48.8 %). In 11/41 patients, cardiac rhabdomyoma were detected by ultrasonography prenatally. In 6 patients, a TSC-2 mutation was found while in 4 patients a TSC-1 mutation was noted; in 1 patient, genetic testing was negative. CONCLUSIONS: Based on our preliminary findings, the annual incidence rate for TSC disease is estimated at approximately 1:12,300 live births, but this is a very rough approximation.


Assuntos
Esclerose Tuberosa/epidemiologia , Adolescente , Criança , Pré-Escolar , Comorbidade , Análise Mutacional de DNA , Feminino , Alemanha/epidemiologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/genética , Humanos , Lactente , Masculino , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/diagnóstico , Malformações do Desenvolvimento Cortical/epidemiologia , Malformações do Desenvolvimento Cortical/genética , Prevalência , Rabdomioma/complicações , Rabdomioma/diagnóstico , Rabdomioma/epidemiologia , Rabdomioma/genética , Inquéritos e Questionários , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/genética , Proteína 2 do Complexo Esclerose Tuberosa/genética , Ultrassonografia Pré-Natal
17.
Cancer Causes Control ; 27(12): 1487-1489, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27812913

RESUMO

PURPOSE: Exposure to asbestos is thought to cause the large majority of pleural mesotheliomas in the USA. It is unknown whether asbestos exposure plays a role in the etiology of rarer forms of mesothelioma, e.g., those located in the pericardium or in the tunica vaginalis of the testis. In order to address this question, we sought to determine whether geographic patterns of incidence of these mesotheliomas have paralleled those of pleural mesotheliomas. METHODS: We used age-adjusted incidence data from the nine populations served by the National Cancer Institute's Surveillance, Epidemiology, and End Results program during 1973-2011. Among men ages ≥50 years, we compared the incidence of pericardial and paratesticular mesotheliomas, respectively, with the incidence of pleural mesothelioma across the nine populations. RESULTS: The rate of pleural mesothelioma was approximately twice as high in the San Francisco-Oakland (SFO) and Seattle-Puget Sound (SPS) areas compared to the other regions. In contrast, rates of paratesticular and pericardial mesotheliomas were not elevated in SFO (n = 3 paratesticular, 1 pericardial) or SPS (n = 4 paratesticular, 1 pericardial) relative to other regions. CONCLUSIONS: The results of this ecologic study do not support a role for asbestos exposure in the etiologies of either pericardial or paratesticular mesotheliomas; however, this study was limited by small numbers and was unable to directly ascertain asbestos exposure.


Assuntos
Mesotelioma/epidemiologia , Neoplasias Testiculares/epidemiologia , Neoplasias Cardíacas/epidemiologia , Neoplasias Cardíacas/patologia , Humanos , Incidência , Masculino , Mesotelioma/patologia , Pessoa de Meia-Idade , Pericárdio/patologia , Neoplasias Pleurais/epidemiologia , Neoplasias Pleurais/patologia , Sistema de Registros , Programa de SEER , Neoplasias Testiculares/patologia , Estados Unidos/epidemiologia
19.
J Card Surg ; 31(1): 15-7, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26585438

RESUMO

BACKGROUND: We retrospectively analyzed 367 patients receiving surgical resection of cardiac myxomas in our center over six years, and analyzed the incidence and surgical results of 28 cases of right atrial myxomas. We also compared the age, gender, and attached sites between left atrial myxoma and right atrial myxoma. METHODS: Between January 2007 and December 2012, 28 patients with right atrial myxomas underwent surgical resection. There were 16 males and 12 females. The mean age was 47.77 ± 13.20 years (range: 8.00-79.00 years). Associated cardiac lesions included moderate and severe tricuspid regurgitation in four, coronary atherosclerotic heart disease in five, and pulmonary embolism in one. Twenty-seven patients (96.43%) were followed from 26 to 94 months (mean 55.78 ± 21.10 months). RESULTS: There was no early death after operation. The incidence of right atrial myxomas among sporadic cardiac myxomas was 7.89%. One patient died of lung cancer 34 months after myxoma resection. Two patients underwent coronary artery stent implantation due to coronary atherosclerotic heart disease during the follow-up period. One patient underwent myxoma resection due to recurrence in the left atrium four years after the first operation. There was no significant difference in the age between left atrial myxoma and right atrial myxoma (p > 0.05). There was a significant difference in the gender between left atrial myxomas and right atrial myxomas (p < 0.05). The most common attached sites of left atrial myxomas and right atrial myxomas are the atrial septum. CONCLUSIONS: Surgical resection of the right atrial myxoma results in good clinical outcomes and a decreased incidence of recurrence.


Assuntos
Neoplasias Cardíacas/cirurgia , Mixoma/cirurgia , Adolescente , Adulto , Idoso , Criança , Feminino , Seguimentos , Átrios do Coração , Neoplasias Cardíacas/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Mixoma/epidemiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores Sexuais , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
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