Three children with cardiac tumors are described: a 12-year-old female child who had left atrial myxoma, and two males having rhabdomyoma of the right ventricle associated with tuberous sclerosis. The child with left atrial myxoma was symptomatic and the tumour was subsequently excised. The other two children with rhabdomyoma were managed conservatively.
Histopathologically adult extracardiac rhabdomyoma is a benign tumour of mature striated muscle. It is a slow- growing tumour with predilection to occur in head and neck region. They appear mainly as a solitary lesion, and about 15% of them can be multicentric. A 56-year-old man presented to us with the floor of mouth swelling persisted for one year and the right neck swelling for three years mimicking a plunging ranula. Radiological imaging revealed enhancing mass at the floor of mouth and submandibular region. The mass represented a nonvascular benign tumour. Subsequently, the patient underwent excision of the mass via transcervical and transoral method. Histopathological examination revealed adult type of extracardiac rhabdomyoma. Adult type of extracardiac rhabdomyoma should be considered in the differential diagnosis for the head and neck masses. A complete excision will reduce the risk of recurrence.
Cardiac rhabdomyoma is the most prevalent cardiac tumors in the pediatric population, in close association with tuberous sclerosis complex. It is usually detected antenatally or postnatally by echocardiography. Clinical presentations depend greatly on the size and position of the tumor mass. Interestingly, rhabdomyoma has a propensity to regress spontaneously and is not usually operated upon, unless the patient becomes hemodynamically compromised. Herein, we report an unusual case of surgically treated cardiac rhabdomyoma in a baby boy presented at birth with a progressive enlarging intraventricular mass, complicated with left ventricular outflow tract obstruction 7 weeks later. Histopathological examination of the intracardiac mass revealed sheets of tumor cells with spider-like morphology (known as "spider cells"), confirmed the diagnosis of rhabdomyoma. Close disease monitoring of patient's hemodynamic status in a newly diagnosed cardiac rhabdomyoma is inevitable as the tumor, although rare, may progress.