Cardiac Fibroma with Asymptomatic Ventricular Arrhythmia in an Adolescent with Gorlin's Syndrome.

IF 0.4 Q4 PEDIATRICS
Dipika Menon, John N Dentel, Yamuna Sanil, David Lawrence
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引用次数: 0

Abstract

Nevoid basal cell carcinoma syndrome (NBCCS), also referred to as Gorlin's syndrome, is an autosomal dominant inherited condition that predisposes affected individuals to various tumors such as cardiac fibromas. Though technically benign, cardiac fibromas may result in malignant arrhythmias and sudden death. The pertinent literature pertaining to pediatric cases of cardiac fibromas and their clinical features were reviewed. We present the case of an asymptomatic teenage with de novo NBCCS who was diagnosed with both NBCCS and cardiac fibroma later in life. The patient was noted to have clinically significant ventricular arrhythmias that were eliminated with tumor resection. There are no established best practice guidelines for the management of cardiac fibromas in patients with NBCCS. Given the risk of sudden arrhythmic death, the presence of ventricular arrhythmias should prompt strong consideration of tumor resection.

青少年Gorlin综合征并发无症状室性心律失常的心脏纤维瘤。
Nevoid基底细胞癌综合征(NBCCS),也被称为Gorlin综合征,是一种常染色体显性遗传疾病,使患者易患各种肿瘤,如心脏纤维瘤。虽然从技术上讲是良性的,但心脏纤维瘤可能导致恶性心律失常和猝死。本文回顾了有关小儿心脏纤维瘤病例及其临床特征的相关文献。我们提出的情况下,无症状的青少年新生NBCCS谁被诊断为NBCCS和心脏纤维瘤后来的生活。患者被注意到有临床上显著的室性心律失常,并通过肿瘤切除消除。对于NBCCS患者心脏纤维瘤的治疗,目前还没有确定的最佳实践指南。考虑到突发性心律失常死亡的风险,室性心律失常的存在应强烈考虑肿瘤切除。
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来源期刊
自引率
0.00%
发文量
32
期刊介绍: The Journal of Pediatric Genetics is an English multidisciplinary peer-reviewed international journal publishing articles on all aspects of genetics in childhood and of the genetics of experimental models. These topics include clinical genetics, molecular genetics, biochemical genetics, medical genetics, dysmorphology, teratology, genetic counselling, genetic engineering, formal genetics, neuropsychiatric genetics, behavioral genetics, community genetics, cytogenetics, hereditary or syndromic cancer genetics, genetic mapping, reproductive genetics, fetal pathology and prenatal diagnosis, multiple congenital anomaly syndromes, and molecular embryology of birth defects. Journal of Pediatric Genetics provides an in-depth update on new subjects and current comprehensive coverage of the latest techniques used in the diagnosis of childhood genetics. Journal of Pediatric Genetics encourages submissions from all authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, short report, rapid communications, case reports, letters to the editor, and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines in the field of pediatric genetics. This journal is a publication of the World Pediatric Society: http://www.worldpediatricsociety.org/ The Journal of Pediatric Genetics is available in print and online. Articles published ahead of print are available via the eFirst service on the Thieme E-Journals platform.
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