987 results match your criteria Cardiac Rhabdomyoma


Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma.

Ann Pediatr Cardiol 2019 Jan-Apr;12(1):45-48

Department of Pediatric Cardiology, Care Hospital, Hyderabad, Telangana, India.

Background: Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein.

Objective: The aim of the study is to observe the efficacy of everolimus in infants with significant CR. Read More

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http://dx.doi.org/10.4103/apc.APC_79_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343372PMC
February 2019
1 Read

An unusual tumour: Hamartoma of mature cardiac myocytes.

Rev Esp Patol 2019 Jan - Mar;52(1):50-53. Epub 2018 Oct 9.

Department of Pathology, Universidad Industrial de Santander, Bucaramanga, Colombia.

Heart neoplasms are uncommon and usually benign. Hamartoma of mature cardiac myocytes is an unusual lesion with only a few reported cases. It is a heterogeneous mixture of well-differentiated myocytes, fibroblasts, adipocytes and blood vessels. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16998855183008
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http://dx.doi.org/10.1016/j.patol.2018.07.004DOI Listing
October 2018
2 Reads

Rapid Regression of Prenatally Identified Intrapericardial Giant Rhabdomyomas with Sirolimus.

CASE (Phila) 2018 Dec 23;2(6):258-261. Epub 2018 Aug 23.

Yale School of Medicine, Section of Pediatric Cardiology, New Haven, Connecticut.

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http://dx.doi.org/10.1016/j.case.2018.07.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302122PMC
December 2018
1 Read

In-utero treatment of a large symptomatic rhabdomyoma with sirolimus.

Ultrasound Obstet Gynecol 2018 Dec 14. Epub 2018 Dec 14.

Department of Obstetrics and Gynaecology, Fetal Medicine Unit, Mount Sinai Hospital and University of Toronto, Toronto, Canada.

A healthy 27-year-old woman was referred to our hospital at 21 weeks gestation. Fetal echocardiography revealed multiple cardiac rhabdomyomas, with the largest lesion in the left ventricle measuring 10x5mm. This article is protected by copyright. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/uog.20196
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http://dx.doi.org/10.1002/uog.20196DOI Listing
December 2018
3 Reads

Hamartomas and other tumor-like malformations of the lungs and heart.

Authors:
Mark R Wick

Semin Diagn Pathol 2019 Jan 16;36(1):2-10. Epub 2018 Nov 16.

Division of Surgical Pathology-Cytopathology & Autopsy Pathology, University of Virginia Medical Center, Room 3020, 1215 Lee Street, Charlottesville, VA 22908-0214, United States. Electronic address:

Tumor-like malformative lesions are seen throughout the body, and they may be confused with true neoplasms by clinicians and pathologists alike. In the lungs, they are principally represented by hamartomas-which may contain chondroid, adipocytic, fibroblastic, and myxoid tissue, with entrapped bronchiolar epithelium-and congenital pulmonary airway malformations (CPAMs). The latter have been subdivided into 5 groups, based on their histological features, but they basically comprise proliferations of malformed bronchopulmonary tissues of different types. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07402570183011
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http://dx.doi.org/10.1053/j.semdp.2018.11.002DOI Listing
January 2019
15 Reads

Autopsy case of right ventricular rhabdomyoma in tuberous sclerosis complex.

Leg Med (Tokyo) 2019 Feb 9;36:37-40. Epub 2018 Oct 9.

Medical Examiner's Office of Hyogo Prefecture, Kobe, Japan; Department of Forensic Medicine, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.

Tuberous sclerosis complex (TSC) is a genetic multisystem disorder characterized by widespread hamartomas in several organs, including the brain, heart, skin, eyes, kidney, lung, and liver. Rhabdomyoma is the most common cardiac tumor diagnosed in fetuses, neonates and infants, and is closely linked to TSC. Here we describe an autopsy case of right ventricular rhabdomyoma in TSC. Read More

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http://dx.doi.org/10.1016/j.legalmed.2018.10.001DOI Listing
February 2019
12 Reads

Perioperative management of critical right ventricular inflow obstruction from right atrial rhabdomyoma.

Ann Card Anaesth 2018 Oct-Dec;21(4):430-432

Manchester Academic Health Science Centre, Cardiothoracic Anaesthesia and Intensive Care, Manchester University, Manchester University NHS Foundation Trust, Manchester, UK; Department of Anaesthesiology and Intensive Care, The Prince Charles Hospital, The University of Queensland, Critical Care Research Group, Brisbane, Australia; Department of Anaesthesiology and Intensive Care, Philipps University, Marburg, Germany.

Rhabdomyoma is the most common cardiac tumor in infancy and commonly located in the ventricles causing outflow obstruction or arrhythmias. We report a rare pediatric (7 month old) case of a right atrial rhabdomyoma presenting with severe cyanosis and low cardiac output from significant tricuspid inflow obstruction with right to left shunt across a stretched patent foramen ovale. We present an emergency cardiac surgery for right atrial tumor resection, and the management of separating the patient with failing right ventricle from cardiopulmonary bypass using a Glenn shunt, since extracorporeal membrane oxygenation (ECMO) or nitric oxide was not available. Read More

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http://www.annals.in/text.asp?2018/21/4/430/243539
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http://dx.doi.org/10.4103/aca.ACA_233_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206783PMC
October 2018
22 Reads

Neonatal supraventricular extrasystole as early clinical debut of cardiac rhabdomyoma.

Ann Pediatr Cardiol 2018 Sep-Dec;11(3):318-321

Department of Pediatric Cardiology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

We are reporting the case of a newborn with a diagnosis of frequent supraventricular extrasystoles, up to 25% of beats at Holter monitoring, and partial response to beta-blockers. Initial echocardiographic studies were normal until the identification of a right atrial mass at 4 months of life. Given the progressive growth of the mass and the suspicion of myxoma or thrombus in the magnetic resonance study, surgical resection of the tumor was performed. Read More

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http://dx.doi.org/10.4103/apc.APC_61_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6146865PMC
October 2018
4 Reads

Prenatal diagnosis of fetal cardiac rhabdomyoma associated with tuberous sclerosis: A case report.

Case Rep Womens Health 2018 Jul 20;19:e00070. Epub 2018 Jun 20.

Sanliurfa Education and Research Hospital, Obstetrics and Gynecology Department, Sanliurfa, Turkey.

Cardiac tumors are rarely diagnosed in utero. Rhabdomyomas are the most common fetal cardiac tumors. They are usually diagnosed during the first year of life after obstruction of a valve orifice or a cardiac chamber; but they can be detected by echocardiography as early as the second trimester. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22149112183006
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http://dx.doi.org/10.1016/j.crwh.2018.e00070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6071374PMC
July 2018
27 Reads

Multiple Accessory Pathways in an Infant With Cardiac Rhabdomyomas and Tuberous Sclerosis.

JACC Clin Electrophysiol 2018 Apr;4(4):553-554

Division of Cardiology, Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, New York.

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https://linkinghub.elsevier.com/retrieve/pii/S2405500X183001
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http://dx.doi.org/10.1016/j.jacep.2017.12.019DOI Listing
April 2018
7 Reads

Incidence of tuberous sclerosis and age at first diagnosis: new data and emerging trends from a national, prospective surveillance study.

Orphanet J Rare Dis 2018 Jul 17;13(1):117. Epub 2018 Jul 17.

Department of Pediatric Neurology, Saarland University Medical Center, Building 9, Kirrberger Strasse, 66421, Homburg, Saarland, Germany.

Background: Tuberous Sclerosis Complex (TSC) is a rare multisystem disorder. In 2012 diagnostic criteria for TSC were revised. However, data on the incidence of TSC are limited. Read More

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http://dx.doi.org/10.1186/s13023-018-0870-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6050673PMC
July 2018
3 Reads

Outcomes After Surgical Resection of Primary Non-Myxoma Cardiac Tumors.

Braz J Cardiovasc Surg 2018 Mar-Apr;33(2):162-168

Kartal Koşuyolu Research and Training Hospital, Cardiovascular Surgery Department, Istanbul, Turkey.

Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors.

Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. Read More

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http://dx.doi.org/10.21470/1678-9741-2017-0152DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985843PMC
January 2019
17 Reads

Fetal cardiac tumors: fetal echocardiography, clinical outcome and genetic analysis in 53 cases.

Ultrasound Obstet Gynecol 2018 Jun 6. Epub 2018 Jun 6.

Maternal-Fetal Consultation Center of Congenital Heart Disease, Department of echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Objective: To analyse the imaging and clinical features of fetal cardiac tumours, and to explore the relationship between tuberous sclerosis complex (TSC) and cardiac rhabdomyoma in fetuses.

Methods: In total, 53 pregnant women with fetal cardiac tumour(s) were examined by standardized fetal echocardiography(FE) and familial TSC genetic testing, and relevant pathological features were also collected.

Results: Of the 53 fetuses, 37 cases exhibited multiple cardiac tumours and 16 exhibited a single cardiac tumor by FE. Read More

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http://dx.doi.org/10.1002/uog.19108DOI Listing
June 2018
10 Reads

Atrial flutter and nonconducted bigeminy premature atrial contraction in a neonate with cardiac rhabdomyoma.

Turk Kardiyol Dern Ars 2018 06;46(4):313-317

Department of Pediatric Cardiology, Health Sciences University İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey.

During the neonatal period the majority of cardiac tumors seen are rhabdomyomas, and the clinical manifestations are notably variable. Symptoms of rhabdomyoma depend on the presence of intracardiac obstructions, myocardial involvement, or rhythm disturbances. Most cardiac rhabdomyomas regress in follow-up, but some need medical/surgical intervention during the neonatal period as a result of resistant atrial or ventricular arrhythmia. Read More

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http://archivestsc.com/jvi.aspx?un=TKDA-72177
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http://dx.doi.org/10.5543/tkda.2017.72177DOI Listing
June 2018
15 Reads

A novel TSC2 missense variant associated with a variable phenotype of tuberous sclerosis complex: case report of a Chinese family.

BMC Med Genet 2018 05 30;19(1):90. Epub 2018 May 30.

Institute of Pediatrics, Children's Hospital of Fudan University, Shanghai, People's Republic of China.

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by the development of hamartomas in multiple organs, including the brain, heart, skin, kidney, lung and retina. A diagnosis of TSC is established with a recently revised clinical/radiological set of criteria and/or a causative mutation in TSC1 or TSC2 gene.

Case Presentation: We report a Chinese TSC family with two siblings presenting with multiple hypomelanotic macules, cardiac rhabdomyomas and cortical tubers associated with a small subependymal nodule. Read More

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http://dx.doi.org/10.1186/s12881-018-0611-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5975528PMC
May 2018
10 Reads

Fetal Type Rhabdomyoma of the Soft Palate in an Adult Patient: Report of One Case and Review of the Literature.

Head Neck Pathol 2018 May 14. Epub 2018 May 14.

Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston, Houston, TX, 77030, USA.

Rhabdomyoma is a rare benign tumor with skeletal muscle differentiation. Rhabdomyoma is further classified into cardiac, adult, fetal, and genital subtypes. Out of these, fetal type rhabdomyoma (FTR) is the rarest. Read More

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http://dx.doi.org/10.1007/s12105-018-0931-5DOI Listing
May 2018
9 Reads

Giant left ventricular rhabdomyoma treated successfully with everolimus: case report and review of literature.

Cardiol Young 2018 Jul 15;28(7):903-909. Epub 2018 May 15.

2Neonatal Intensive Care Unit,Instituto Nacional de Perinatología Isidro Espinosa de los Reyes,Mexico City,Mexico.

IntroductionIntracardiac rhabdomyomas can cause severe ventricular dysfunction and outflow tract obstruction.Case reportA term newborn infant with antenatal diagnosis of giant left ventricle rhabdomyoma presented with cardiac failure and duct-dependent systemic circulation after birth. She was treated successfully with everolimus, showing decrease in tumour size and improvement in left ventricular ejection fraction. Read More

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https://www.cambridge.org/core/product/identifier/S104795111
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http://dx.doi.org/10.1017/S1047951118000598DOI Listing
July 2018
9 Reads

Antenatal screening and diagnosis of tuberous sclerosis complex by fetal echocardiography and targeted genomic sequencing.

Medicine (Baltimore) 2018 Apr;97(15):e0112

Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing Key Laboratory of Maternal-Fetal Medicine in Fetal Heart Disease.

Although fetal cardiac rhabdomyoma can be the initial finding in patients with tuberous sclerosis complex (TSC), the challenges of precise genetic diagnosis of TSC can now be potentially overcome by new whole or targeted genomic sequencing. The goals of this study were to investigate the correlation between suspected cardiac rhabdomyoma and TSC to provide the information for a prenatal diagnosis of TSC and to validate the TSC genotype in this cohort of fetuses with suspected prenatal cardiac rhabdomyoma.We retrospectively analyzed 10,728 fetal echocardiograms from January 2013 to March 2016 in our institution. Read More

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http://Insights.ovid.com/crossref?an=00005792-201804130-0000
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http://dx.doi.org/10.1097/MD.0000000000010112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5908597PMC
April 2018
14 Reads

Spontaneous Regression of Cardiac Rhabdomyoma Presenting as Severe Left Ventricular Inlet Obstruction in a Neonate with Tuberous Sclerosis.

Case Rep Cardiol 2018 28;2018:8395260. Epub 2018 Jan 28.

Department of Pediatrics, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju, Republic of Korea.

Cardiac rhabdomyoma can be subclinical or have a fatal presentation according to the onset age and involved site, size, and degree of invasion. Although most cardiac rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction has occurred. In this report, we describe the spontaneous regression of a large cardiac rhabdomyoma (20. Read More

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http://dx.doi.org/10.1155/2018/8395260DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5829321PMC
January 2018
16 Reads

Adult Rhabdomyoma of the Tongue in a Child: Report of a Case and a Literature Appraisal.

Contemp Clin Dent 2018 Jan-Mar;9(1):2-4

Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, Maharashtra, India.

Rhabdomyoma, by definition is a benign muscle tumour.. Rhabdomyomas constitute 2% of all myogenous neoplasms. Read More

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http://dx.doi.org/10.4103/ccd.ccd_835_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5863402PMC
March 2018
3 Reads

Diagnosis and Treatment of Tuberous Sclerosis Manifestations in Children: A Multicenter Study.

Neuropediatrics 2018 Jun 20;49(3):193-199. Epub 2018 Mar 20.

Department of Pediatric Neurology, Saarland University Medical Center, Homburg, Germany.

Tuberous sclerosis complex (TSC) is a genetic disease with a significant morbidity and mortality. We conducted a retrospective analysis of two cohorts (Vall d'Hebron University Hospital [HVH], Barcelona, Spain, 1982-2015, and at Saarland University Medical Center [UKS], Homburg, Germany, 1998-2015) to assess prevalence and treatment of TSC associated manifestations and to evaluate if the follow-up was in line with published recommendations. This was considered if more than 15% of patients did not receive adequate examination with regard to potential organ involvement. Read More

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http://dx.doi.org/10.1055/s-0038-1637738DOI Listing
June 2018
3 Reads

Evolution of a rare ECG pattern in an aggressive case of neonatal tuberous sclerosis complex.

Int J Surg Case Rep 2018 23;44:197-201. Epub 2018 Feb 23.

Department of Paediatric and Congenital Cardiac Surgery and Cardiology, Azienda Ospedaliero-Universitaria Ospedali Riuniti Ancona "Umberto I, G. M. Lancisi, G. Salesi" Ancona, via Conca n. 71, 60128 Ancona, Italy.

Introduction: Rhabdomyomas are the most frequent cardiac tumors in children. Furthermore, they are often associated to tuberous sclerosis complex, an autosomal dominant neurocutaneous disorder characterized by tumor-like malformations that involve many organ systems.

Presentation Of The Case: We describe a rare ECG pattern in a severe case of neonatal tuberous sclerosis complex. Read More

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http://dx.doi.org/10.1016/j.ijscr.2018.02.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5927810PMC
February 2018
5 Reads

Prenatal diagnosis of tuberous sclerosis complex using fetal ultrasonography and magnetic resonance imaging and genetic testing.

Taiwan J Obstet Gynecol 2018 Feb;57(1):163-165

Department of Obstetrics and Gynecology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan. Electronic address:

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http://dx.doi.org/10.1016/j.tjog.2017.12.029DOI Listing
February 2018
6 Reads

Prediction of postnatal arrhythmia in fetuses with cardiac rhabdomyoma.

J Matern Fetal Neonatal Med 2018 Feb 20:1-6. Epub 2018 Feb 20.

b Department of Obstetrics and Gynecology , Mie University , Tsu , Japan.

Objectives: Fetal cardiac rhabdomyomas are rare but well-known to be associated with arrhythmia or conduction abnormalities. However, since in utero electrophysiological information is quite limited, it remains unclear which type of rhabdomyoma will develop arrhythmia after birth. The aim of this study is to identify factors that predict postnatal arrhythmia requiring therapy in fetuses with cardiac rhabdomyoma. Read More

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http://dx.doi.org/10.1080/14767058.2018.1438402DOI Listing
February 2018
23 Reads

Efficacy and safety of everolimus in patients younger than 12 months with congenital subependymal giant cell astrocytoma.

Brain Dev 2018 May 2;40(5):415-420. Epub 2018 Feb 2.

Department of Pediatric Neurosurgery, Osaka City General Hospital, Osaka, Japan.

Tuberous sclerosis complex (TSC) is a multisystem genetic disorder that activates mammalian target of rapamycin and produces tumor growth in several organs. We present five patients younger than 12 months who were diagnosed with TSC and treated with everolimus (EVL), after which congenital subependymal giant astrocytoma (cSEGA) promptly regressed in all patients. All patients achieved at least 50% reduction in the volume of cSEGA within 6 months. Read More

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http://dx.doi.org/10.1016/j.braindev.2018.01.001DOI Listing
May 2018
8 Reads

Early diagnosis of tuberous sclerosis complex: a race against time. How to make the diagnosis before seizures?

Orphanet J Rare Dis 2018 Jan 29;13(1):25. Epub 2018 Jan 29.

Department of Neurology and Epileptology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warszawa, Poland.

Background: Tuberous sclerosis complex (TSC) is a genetic disorder with an incidence of 1:6000 live births and associated with the development of benign tumors in several organs. It is also characterized by high rates of neurological and neuropsychiatric abnormalities, including epilepsy affecting 70-90% of patients and being one of the major risk factors of intellectual disability. The first seizures in TSC patients appear usually between the 4th and the 6th months of life. Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0764-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5789613PMC
January 2018
20 Reads

Adult-Type Rhabdomyoma of the Larynx: Clinicopathologic Study of an Uncommon Tumor in a Rare Location.

Case Rep Otolaryngol 2017 26;2017:7186768. Epub 2017 Nov 26.

Department of Sense Organs, Sapienza University of Rome, Rome, Italy.

Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extracardiac sites. Even though the head and neck region is the most common area of extracardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia, and dysphonia. Read More

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http://dx.doi.org/10.1155/2017/7186768DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5727691PMC
November 2017
11 Reads

Prenatal and Postnatal Diagnosis of Rhabdomyomas and Tuberous Sclerosis Complex by Ultrafast and Standard MRI.

Indian J Pediatr 2018 09 9;85(9):729-737. Epub 2018 Jan 9.

Department of Radiology, Shanghai Children's Medical Center, 1678 Dongfang Rd., Shanghai, 200127, China.

Objective: To examine the features of cardiac rhabdomyomas and tuberous sclerosis in fetuses and infants using magnetic resonance imaging (MRI) and to determine whether MRI is an effective tool to facilitate early detection of tuberous sclerosis complex (TSC).

Methods: Fifteen patients with TSC were evaluated by ultrafast or standard MRI between June 2005 and September 2016. Fifteen patients were divided into two groups. Read More

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http://dx.doi.org/10.1007/s12098-017-2592-xDOI Listing
September 2018
14 Reads

Giant cardiac tumours in the newborn: an unusual image.

Folia Morphol (Warsz) 2018 3;77(3):601-603. Epub 2018 Jan 3.

Department of Pediatric Cardiology and Congenital Heart Defects, Medical University of Gdansk, Poland, Gdańsk, Poland.

Primary heart tumours in the paediatric population are very rare and they range from 0.01% to 0.04%. Read More

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http://dx.doi.org/10.5603/FM.a2017.0119DOI Listing
January 2018
11 Reads

Tuberous sclerosis presenting as neonatal cyanosis because of rhabdomyoma causing tricuspid valve obstruction needing a Blalock-Taussig shunt.

J Saudi Heart Assoc 2018 Jan 21;30(1):66-68. Epub 2017 Apr 21.

Prince Rashid Hospital, Pediatrics, Royal Medical Services, Irbid, Jordan.

We report a newborn female baby who presented at 6 hours of age with cyanosis without any signs of respiratory distress. Cardiovascular and systemic examination was unremarkable apart from cyanosis (saturation 75%). An echocardiogram showed multiple echogenic and homogeneous masses in the interventricular septum, one of which was big and protruding through the tricuspid valve causing right ventricular inflow obstruction. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10167315173003
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http://dx.doi.org/10.1016/j.jsha.2017.04.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5744029PMC
January 2018
16 Reads

Rapid regression of large cardiac rhabdomyomas in neonates after sirolimus therapy.

Cardiol Young 2018 Mar 13;28(3):485-489. Epub 2017 Dec 13.

1Department of Pediatrics,Division of Pediatric Cardiology,Duke University Hospital and Health Center,Durham,NC,United States of America.

Cardiac rhabdomyomas are the most common tumours in children and are typically seen in association with the tuberous sclerosis complex. Although benign and often associated with spontaneous regression, in rare circumstances surgical resection is indicated to relieve obstruction or other mass-related effects. Recent clinical trials have demonstrated the benefits of mammalian target of rapamycin inhibitors for the treatment of other tumour sub-types associated with tuberous sclerosis. Read More

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http://dx.doi.org/10.1017/S104795111700244XDOI Listing
March 2018
14 Reads

Primary cardiac tumors associated with genetic syndromes: a comprehensive review.

Pediatr Radiol 2018 02 6;48(2):156-164. Epub 2017 Dec 6.

Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan Health System, University Hospital Floor B1 Reception C, 1500 E. Medical Center Drive SPC 5030, Ann Arbor, MI, 48109, USA.

Various cardiac tumors occur in the setting of a genetic syndrome such as myxomas in Carney complex and rhabdomyomas in tuberous sclerosis. Tumor biology can be different in syndromic forms, and on imaging children sometimes demonstrate additional manifestations of the underlying syndrome. We discuss the imaging appearance of cardiac tumors occurring in the framework of a genetic syndrome, the findings that suggest an underlying syndrome, and the impact on management. Read More

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http://dx.doi.org/10.1007/s00247-017-4027-2DOI Listing
February 2018
9 Reads

[Multiple cardiac rhabdomyomas revealing tuberous sclerosis in a neonate].

Arch Pediatr 2017 Dec 16;24(12):1321-1323. Epub 2017 Nov 16.

Service de radiologie B, centre de maternité et de néonatologie, Monastir, Tunisie.

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http://dx.doi.org/10.1016/j.arcped.2017.09.028DOI Listing
December 2017
2 Reads

Cardiac Rhabdomyomas in Tuberous Sclerosis Complex.

J Pediatr 2018 01 8;192:264-264.e1. Epub 2017 Nov 8.

Fundación Cardiovascular de Colombia Hospital Internacional de Colombia Floridablanca, Colombia; Division of Human Genetics Cincinnati Children's Hospital Medical Center University of Cincinnati College of Medicine Cincinnati, Ohio.

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http://dx.doi.org/10.1016/j.jpeds.2017.09.050DOI Listing
January 2018
38 Reads

Cardiac Tumors in Pediatric Patients: A Systematic Review.

World J Pediatr Congenit Heart Surg 2017 09;8(5):624-632

1 Surgery Working Group, Society of Junior Doctors, Athens, Greece.

This systematic review sought to investigate the current evidence regarding surgical management of primary cardiac tumors in children and adolescents. Twenty-eight studies were deemed eligible, reporting on 745 pediatric patients. Rhabdomyoma was the most prevalent histologic type and echocardiography was the most common diagnostic tool. Read More

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http://dx.doi.org/10.1177/2150135117723904DOI Listing
September 2017
21 Reads

Everolimus for the Treatment of Tuberous Sclerosis Complex-Related Cardiac Rhabdomyomas in Pediatric Patients.

Authors:
Nagib Dahdah

J Pediatr 2017 11 6;190:21-26.e7. Epub 2017 Sep 6.

Division of Pediatric Cardiology, CHU Sainte-Justine, University of Montreal, Quebec, Canada. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00223476173092
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http://dx.doi.org/10.1016/j.jpeds.2017.06.076DOI Listing
November 2017
5 Reads

Maternal and Fetal Tuberous Sclerosis: Do We Know Enough as an Obstetrician?

J Reprod Infertil 2017 Apr-Jun;18(2):257-260

Department of Radiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences Shillong, Meghalaya, India.

Background: Tuberous sclerosis, also known as tuberous sclerosis complex (TSC), is a rare genetic condition that mainly causes hamartomas to develop in different parts of the body. TSC, an autosomal dominant trait with variable penetrance, can adversely affect maternal and fetal outcome.

Case Presentation: In this paper, a case of maternal and fetal tuberous sclerosis having fetal cardiac rhabdomyoma detected in utero at 26 weeks was reported who subsequently had fetal demise at 31 weeks. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5565905PMC
September 2017
14 Reads

Punch-out lesion following regression of a large left ventricular outflow rhabdomyoma.

Cardiol Young 2018 Jan 29;28(1):153-154. Epub 2017 Aug 29.

Department of Pediatric Cardiology, Hadassah,Hebrew University Medical Center,Jerusalem,Israel.

Abstracts We report the case of a patient with rhabdomyoma of the left ventricular outflow tract, causing severe obstruction at birth. The tumour regressed completely by 6 years of age, leaving a punch-out lesion. The potential for spontaneous regression of these tumours and the formation of a myocardial lesion following rhabdomyoma regression are discussed. Read More

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https://www.cambridge.org/core/product/identifier/S104795111
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http://dx.doi.org/10.1017/S1047951117001512DOI Listing
January 2018
6 Reads

Early Detection of Tuberous Sclerosis Complex: An Opportunity for Improved Neurodevelopmental Outcome.

Pediatr Neurol 2017 Nov 26;76:20-26. Epub 2017 May 26.

Department of Medical Genetics, Sydney Children's Hospital, Randwick, NSW, Australia; School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Kensington, NSW, Australia. Electronic address:

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant condition associated with epilepsy, benign tumors, and variable neurodevelopmental outcomes. The diagnosis is most commonly made after epilepsy onset, although a proportion are diagnosed prenatally. Presymptomatic or early treatment with agents such as vigabatrin offers the hope of improved neurodevelopmental outcome. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2017.05.014DOI Listing
November 2017
13 Reads

Cardiac tumors in children: A 20-year review of clinical presentation, diagnostics and treatment.

Adv Clin Exp Med 2017 Mar-Apr;26(2):319-326

Department of Clinical Anatomy, Medical University of Gdańsk, Poland.

Background: The use of new imaging techniques has contributed significantly to earlier diagnosis and treatment of cardiac tumors.

Objectives: The aim of the study was to analyze data from children with cardiac tumors in terms of clinical presentation, the role of noninvasive diagnostic procedures and the long-term outcome.

Material And Methods: The data analyzed retrospectively concerned 30 children in whom cardiac tumors were diagnosed from January 1995 to July 2015. Read More

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http://dx.doi.org/10.17219/acem/62121DOI Listing
September 2017
8 Reads

Regression of Neonatal Cardiac Rhabdomyoma in Two Months Through Low-Dose Everolimus Therapy: A Report of Three Cases.

Pediatr Cardiol 2017 Oct 5;38(7):1478-1484. Epub 2017 Aug 5.

Pediatric Nephrology, China Medical University Children's Hospital, Taichung, 40447, Taiwan.

Cardiac rhabdomyoma (CR) is the most common cardiac tumor in newborns. Approximately 75% of cases are associated with tuberous sclerosis complex. Although these tumors usually spontaneously regress after 2 years of age, they can be life-threatening when they obstruct major cardiac inflow or outflow pathways. Read More

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http://dx.doi.org/10.1007/s00246-017-1688-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5628183PMC
October 2017
10 Reads

Epicardial ablation of tachyarrhythmia in children: Experience at two academic centers.

Pacing Clin Electrophysiol 2017 Sep 26;40(9):1017-1026. Epub 2017 Aug 26.

Department of Pediatrics, University of West Virginia School of Medicine, Morgantown, WV, USA.

Background: Experience with percutaneous epicardial ablation of tachyarrhythmia in pediatrics is limited. This case series addresses the feasibility, safety, and complications of the procedure in children.

Methods: A total of nine patients underwent 10 epicardial ablation procedures from 2002 to 2013 at two academic centers. Read More

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http://dx.doi.org/10.1111/pace.13152DOI Listing
September 2017
9 Reads
1.250 Impact Factor

Single giant mediastinal rhabdomyoma as a sole manifestation of TSC in foetus.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2017 Sep 12;161(3):326-329. Epub 2017 Jun 12.

Outpatient Cardiology Department, Pediatric Clinic, The University Hospital Brno, Czech Republic.

Background: Presence of multiple cardiac rhabdomyomas is one of the major features of Tuberous sclerosis (TSC), but isolated progressing single giant rhabdomyoma is very rare and not typical of TSC.

Case Report: This report presents family without obvious history of TSC with occurrence of giant mediastinal rhabdomyoma affecting the haemodynamics in male foetus, without other TSC symptoms. Girl from the next gravidity had prenatally detected multiple rhabdomyomas and small subcortical tuber of brain detected after birth. Read More

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http://dx.doi.org/10.5507/bp.2017.023DOI Listing
September 2017
14 Reads

Massive biventricular rhabdomyoma in a neonate.

Ann Pediatr Cardiol 2017 May-Aug;10(2):218-219

Department of Cardiology, AIIMS, New Delhi, India.

Rhabdomyoma is a well characterised entity in a neonate. Herein, we report a massive biventricular rhabdomyoma in a neonate presenting with cyanosis and congestive heart failure which was confirmed on autopsy. The report is for documentation of an unusually large tumour. Read More

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http://dx.doi.org/10.4103/apc.APC_164_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5431041PMC
June 2017
4 Reads

Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma - Single Centre Experience.

Open Access Maced J Med Sci 2017 Apr 20;5(2):193-196. Epub 2017 Mar 20.

Main Center of Family Medicine, Prishtina, Kosovo.

Background: Cardiac rhabdomyoma (CRs) are the most common primary tumour of the heart in infants and children. Usually are multiple and, basing on the location can cause a haemodynamic disturbance, dysrhythmias or heart failure during the fetal and early postnatal period. CRs have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). Read More

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http://www.id-press.eu/mjms/article/view/oamjms.2017.040
Publisher Site
http://dx.doi.org/10.3889/oamjms.2017.040DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5420773PMC
April 2017
37 Reads

Chordoma Occurs in Young Children With Tuberous Sclerosis.

J Neuropathol Exp Neurol 2017 Jun;76(6):418-423

From the Department of Pediatrics, University of Colorado Denver, Aurora, Colorado (NAD, TL, ML, JMM-L); Morgan Adams Foundation Pediatric Brain Tumor Research Program, Children's Hospital Colorado, Aurora, Colorado (NAD, JMM-L); Department of Neurology (TL, BKK-D); Department of Radiology (IN); Department of Neurosurgery (MHH, BKK-D); and Department of Pathology, University of Colorado Denver, Aurora, Colorado (BKK-D).

Chordomas are rare bony neoplasms usually unassociated with a familial tumor predisposition syndrome. The peak incidence of this midline axial skeletal tumor is in adulthood but when very young children are affected, consideration should be given to occurrence within the tuberous sclerosis (TS) complex, especially when presenting in neonates <3 months of age. To call attention to this association, we present a brachyury-immunopositive chordoma occurring in the skull base of a 2-month-old male infant who was later realized to have metastases to the subcutaneous tissues and lungs, as well as rhabdomyoma of the heart and renal cysts/angiomyolipomas, that is, characteristic features of the TS complex. Read More

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http://dx.doi.org/10.1093/jnen/nlx032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6075544PMC
June 2017
31 Reads

Spontaneous Regression of a Gigantic Cardiac Rhabdomyoma.

Heart Lung Circ 2017 Nov 27;26(11):e105-e106. Epub 2017 Mar 27.

Department of Cardiology, Government Medical College, Kozhikode, Kerala, India.

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https://linkinghub.elsevier.com/retrieve/pii/S14439506173027
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http://dx.doi.org/10.1016/j.hlc.2017.02.019DOI Listing
November 2017
6 Reads

Primary cardiac lymphoma with unusual presentation: A report of two cases.

Mol Clin Oncol 2017 Mar 16;6(3):311-314. Epub 2017 Jan 16.

Department of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung 813, Taiwan, R.O.C.

Primary cardiac tumors are rare, with an incidence of 0.056% according to autopsy reports. The most common type is myxoma, while other types, including sarcoma, lipoma, papillary fibroelastoma, rhabdomyoma, fibroma, hemangioma, teratoma, lymphoma and mesothelioma also occur. Read More

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https://www.spandidos-publications.com/10.3892/mco.2017.1131
Publisher Site
http://dx.doi.org/10.3892/mco.2017.1131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5403538PMC
March 2017
11 Reads