We report the case of a female neonate admitted to the neonatal ICU with a rapid, narrow-complex tachyarrhythmia determined to be supraventricular tachycardia. Multimodality imaging and genetic testing confirmed a diagnosis of tuberous sclerosis complex with multiple cardiac rhabdomyomas. At 13 days of age, the patient was readmitted, exhibiting recurrent supraventricular tachycardia non-responsive to first-line treatment. Management required triple-drug therapy, whereafter the patient remained stable without recurrences. This is a rare report of supraventricular tachycardia in a functionally normal heart with the occurrence of supraventricular tachycardia due to structural abnormalities, with the possibility of multiple concealed accessory pathways.

Cardiac rhabdomyomas represent the most common primary paediatric cardiac tumour and typically regresses over time in the majority of patients. Among those who are symptomatic, surgical resection or catheterisation procedures have traditionally proven effective. More recently, those invasive or challenging tumours have been successfully treated with mammalian target of rapamycin inhibitors, typically everolimus and sirolimus. This review outlines the current medical literature of the state-of-the-art medical treatment of these tumours. We specifically focus on dosing regimens, duration of therapy, and side-effect profiles of mammalian target of rapamycin inhibitors among this population. Although the majority of cases responded to mammalian target of rapamycin inhibition, standardised guidelines for dosing and duration of treatment remain to be defined.

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. Here we report rapid regression of several massive cardiac rhadomyomas in two neonates with the use of the mammalian target of rapamycin inhibitor sirolimus.

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.

Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.

Rhabdomyoma is the most common cardiac tumour in children often associated with tuberous sclerosis. Arrhythmia caused by cardiac rhabdomyomas may be the initial sign of tuberous sclerosis. Rhabdomyomas unresponsive to other treatments could be successfully managed with everolimus, which has demonstrated benefit in tuberous sclerosis. We report a case of rhabdomyoma causing severe arrhythmia in a newborn managed successfully with everolimus.

We report a case of cardiac rhabdomyomas in an infant who presented with right ventricular tachycardia, and a clinical picture of infective endocarditis. Typical features of tuberous sclerosis developed subsequently. To the best of our knowledge, cardiac rhabdomyoma has not been reported previously in association with infective endocarditis.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

Rhabdomyoma is the most frequent primary cardiac tumor seen during infancy. We report multiple rhabdomyomas diagnosed in twins in the neo natal period. To the best of our knowledge, this is the first instance of this happening.

Cardiac rhabdomyoma represents the commonest primary cardiac tumour. Its natural history is generally favourable, with resolution in the majority of cases. Surgical resection has been described for intractable arrhythmia, severe obstruction of the inflow or outflow tracts sufficient to compromise cardiac output, and systemic embolization. We describe an alternative palliative strategy, which was associated with regression of the tumour.

The histopathological and imaging findings of a rhabdomyoma of the base of the tongue were studied. An immunohistochemical examination of the tumour cells showed positiveimmunostaining for myoglobin, desmin, and striated muscle actin, but negative immunostaining for smooth muscle actin. Electron microscopy showed many glycogen granules and mitochondria in the tumour cells. The T2-weighted and contrast-enhanced magnetic resonance images (MRI) clearly delineated morphological features of this tumour, but T1-weighted MRI and computed tomography (CT) images showed no important features. These findings are typical for an adult extracardiac rhabdomyoma located in the head and neck region, and they will be useful for diagnosis of this tumour.

A review of extracardial rhabdomyomas of the larynx reported in the literature is presented. A new case is added (the largest described yet). The diagnosis was based on routine histological and immunohistological staining, and electron microscopy. The extracardial rhabdomyomas were divided into three types according to histopathological findings: (i) adult; (ii) foetal cellular type; and (iii) foetal myxoid. There are 23 well-documented cases (including this case) of extracardial rhabdomyomas of the larynx; 15 of the adult type, four of the foetal myxoid type and four of the foetal cellular type. The differential diagnosis and the requisite diagnostic procedure is discussed. The tumour is benign. The treatment is surgical excision. Although rare, its existence should be kept in mind in the differential diagnosis of laryngeal tumours.