11/310. Pre-excitation syndrome secondary to cardiac rhabdomyomas in tuberous sclerosis. Rhabdomyomas are not uncommon in infants with tuberous sclerosis. We describe a neonate who presented with hydrops fetalis arising from a tachyarrhythmia during fetal life related to rhabdomyomas. After reversion of the arrhythmia, pre-excitation was noted on an interval electrocardiogram. Following regression of the tumours, the delta wave disappeared with no further arrhythmias noted. ( info) |
12/310. Transposition of the great arteries, pulmonary atresia, and multiple ventricular septal defects associated with multiple cardiac rhabdomyomas in a case of tuberous sclerosis. Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report an unusual case of tuberous sclerosis with cerebral and renal lesions associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects, and parachute mitral valve deformity. After reviewing other cases of congenital heart diseases associated with tuberous sclerosis, emphasis is made on the potential influence of multiple rhabdomyomas developing very early during fetal life on final cardiac structures. ( info) |
13/310. Multiple cardiac rhabdomyomas detected in utero. Prenatal fetal echocardiography studies detected large multiple cardiac rhabdomyomas and led to the diagnosis of tuberous sclerosis. Despite episodes of bradyarrhythmias and severe lung compression in utero, the neonate did well and was discharged home on day 20 of life. On day 30 of life, the infant expired at home from cardiac arrest secondary to arrhythmia. ( info) |
14/310. Fetal rhabdomyomatous nephroblastoma: diagnosis by fine-needle aspiration cytology--a case report. The present report describes the case of a 21-mo-old boy with a fetal rhabdomyomatous nephroblastoma (FRN) diagnosed by fine-needle aspiration cytology (FNAC). The smears contained numerous isolated or clustered, deeply eosinophilic, thin, elongated rhabdomyoblasts with oval nuclei. Some of these cells presented cross-striations. Peculiar clusters of small, deeply eosinophilic, round cells with and without rounded nuclei in a mosaic pattern were interpreted as representing transversally oriented rhabdomyoblasts. Fetal rhabdomyoblasts were by far the predominant type of cell. histology of the surgical specimen proved 90% of the mass to be constituted of fetal rhadomyomatous tissue. Adequate recognition of FRN is useful for the planning of treatment. ( info) |
15/310. Transient neonatal cyanosis: unusual presentation of right-sided cardiac masses. We report a case of a neonate with multiple cardiac masses, cyanosis, and a heart murmur. Arterial desaturation was the result of right-to-left shunting at the foramen ovale level caused by tricuspid regurgitation. Three right-sided cardiac masses were detected by echocardiography. By 2 weeks of age the patient had complete resolution of his cyanosis and improved tricuspid regurgitation following the normal decrease in pulmonary vascular resistance. At 2 years of age, he has no cardiovascular symptoms and the masses are calcified and have no hemodynamic consequences. ( info) |
16/310. rhabdomyoma of the base of the tongue. A case report. A case of rhabdomyoma in the base of the tongue that caused dysphagia and sleep apnea is presented. diagnosis was accomplished by means of fine needle aspiration biopsy and MRI. The tumor was completely removed by a tongue midline split. ( info) |
17/310. adult rhabdomyoma: report of two cases of rhabdomyoma of the lip and of the eyelid. We describe two cases of adult rhabdomyoma. One was located in the lip of a 66-year-old woman and was removed because it was clinically suspicious for infiltrating carcinoma. The other arose in the eyelid of a 60-year-old woman with a glass eye and was initially interpreted as a reactive process due to the prosthesis. Both lesions were composed of cells with oval nuclei and deeply eosinophilic cytoplasms with occasional cross striations. Immunoreactivity for desmin and myoglobin excluded the diagnosis of other tumors with similar morphology. The unusual association of the eyelid tumor with the prosthesis suggests a role for chronic irritation in the pathogenesis of rhabdomyoma. ( info) |
| A 67-year-old man presented with a scrotal swelling. Intraoperatively, this swelling was found to arise from the spermatic cord structures and was completely excised. Histopathology revealed a rhabdomyoma arising from the cord. The patient has had no evidence of recurrence in the past 24 months. We present the various types of rhabdomyomas and discuss the differential diagnosis for a spermatic cord swelling. ( info) |
| A case of antenatal tuberous sclerosis was diagnosed by ultrasonography. Intracardiac tumour (highly suspected rhabdomyoma) with transitory heart failure and multiple brain lesions were observed. After delivery, echocardiography, spiral CT and MR imaging diagnosis of tuberous sclerosis was confirmed by typical skin lesions (depigmented macules) and development of seizures. ( info) |
| Extracardiac rhabdomyomas are rare benign tumours showing striated muscle differentiation. Seventy percent of these lesions occur in the head and neck region. The most common sites for these lesions are the larynx, pharynx, and the floor of the mouth. There has been only one previous report of a rhabdomyoma of the oesophagus; two further cases are described. ( info) |