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The occurrence of multifocal rhabdomyoma is exceedingly rare with less than half a dozen cases collected in the literature. We present a case of multifocal rhabdomyoma of the head and neck that involves extension of the tumor to the vallecula, the glossoepiglottic fold, and the aeriepiglottic fold.
We have experienced anesthetic management for cardiac rhabdomyoma in a 21-day-old male, whose pulmonary trunk was obstructed by the tumor. He was anesthetized with fentanyl 70 micrograms.kg-1 and oxygen. The operative and post-operative courses were uneventful. Cardiac tumor, especially cardiac rhabdomyoma, in infant is rare. Anesthesia for the resection of the cardiac tumor must be carried out carefully, because sudden death due to arrhythmias as well as cardiac or respiratory failure is the most dangerous complication.
Case of a rhabdomyoma of the vagina in a 49 yr-old lady has been reported. The lesion has recurred 4 years after. This appears to be the first case reported in the literature and is similar to a case of pharyngeal adult rhabdomyoma which has also recurred. It is proposed that the present tumor is neoplastic in origin.
A case is presented of a euthyroid male in whom a right thyroid mass was discovered. Physical displacement of the right thyroid lobe by a mass determined on CT scan. Scintigraphy and ultrasound showed the mass to be separate from the thyroid. Fine needle aspirations did not predict the tumor. Surgical removal of an adult rhabdomyoma was uneventful. This case and review of the literature demonstrate the rare presentation of a rhabdomyoma as a suspected thyroid nodule.
A case of an unusual tumor of skeletal muscle origin is described. The tumor was located in the tunica vaginalis of the testis in a 19-year-old man. Histologic examination showed a proliferation of elongated or round cells, with clearly discernible cross striations, surrounded by abundant mature connective tissue, consistent with genital rhabdomyoma. Immunohistochemical and electron microscopic features supported this diagnosis. Rhabdomyoma must be considered in the differential diagnosis of paratesticular tumors.
Rhabdomyomas may be the underlying cause of accessory pathways in patients with tuberous sclerosis, leading to symptomatic supraventricular tachycardia that does not respond to antiarrhythmic drugs. We report an infant with tuberous sclerosis and cardiac rhabdomyomas who underwent successful radiofrequency catheter ablation of a left free wall accessory pathway and we describe the clinical features, ablation findings and long-term follow-up.
Cardiac rhabdomyomas are frequent in association with tuberous sclerosis and they are the first symptoms of Bourneville disease in fetal and neonatal period. Clinical findings of cardiac rhabdomiomas are quite heterogeneous: asymptomatic, cardiac murmur, cardiomegaly, heart failure or arrhythmias. Echocardiography can determine site, dimensions, numbers and haemodynamic consequences of cardiac tumours and their clinical evolution at follow-up. In this study the Authors report clinical findings in 9 cases of cardiac rhabdomyomas in newborn infants: 3 has familiarity for tuberous sclerosis and two of these had prenatal echocardiographic diagnosis; in the other 6 cases diagnosis was casual (1 in fetal period). All patients with prenatal diagnosis were asymptomatic. In other cases 1 had extrasystolic arrhythmias and five had only cardiac murmurs. Echocardiographic follow-up (6 months to 5 years) showed regression of number and dimension of cardiac masses in all cases. In 7 cases cardiac rhabdomiomas were associated with tuberous sclerosis.
Benign voluntary muscle tumours are exceedingly rare. Only 9 fetal and 35 adult type rhabdomyomata have been reported in the literature, former usually in children, the latter in adults. Both types affect the musculature of the head and neck almost exclusively. In this ultrastructural study of a fetal rhabdomyoma in a girl aged eleven years, we have found that the tumour is composed of cells showing myoblastic or myotubular differentiation although the myofilaments are poorly arranged and the myofibrils irregularly distributed. Motor-end plates have not been observed. These observations, together with the light microscopic findings and a review of previously described cellular characteristics, suggest that fetal and adult rhabdomyoma are different stages in the life history of the same tumour. Whether the lesion should be considered to be a hamartoma or a neoplasm is uncertain.
Rhabdomyomas are the most common primary cardiac tumors in children, and have been shown to undergo spontaneous regression. The aim of our study was to investigate morphologically and immunohistochemically some mechanisms that may explain this clinical phenomenon. Eleven tumors from three term newborn girls who had physical and radiographic features pathognomonic of tuberous sclerosis were evaluated. Control specimens were left and right heart sections from five autopsies of age- and sex-matched patients who died of causes unrelated to the cardiovascular system. The tumors had been surgically excised from various regions in the heart, and all had similar "typical" histology. Histomorphologic evaluation with von Kossa and alizarin-red stains and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end-labeling (TUNEL) method were performed to evaluate cell calcifications, necrosis, and apoptosis. Ubiquitin immunohistochemical study was also conducted to evaluate intracytoplasmic protein degradation. In cardiac rhabdomyomas (CR), all myocytes with acidophilic cytoplasmic myofibrils showed strong intracytoplasmic ubiquitin immunoreactivity, compared with the occasional weak cytoplasmic and focal nuclear positivity in control heart sections. Calcified myocyte nuclei were commonly seen in CR tumoral and nontumoral rhabdomyocytes, whereas control nontumoral cardiac myocytes did not show any calcification. The incidence of TUNEL reactivity seen in CR (4.8 nuclei per 100 cardiac rhabdomyocyte nuclei) was higher than that seen in control heart sections (1.7 nuclei per 106 cardiac myocytes, P < 0.005). The data led us to conclude that the cytoplasmic contents in CR were degraded via the ubiquitin pathway, and from our observation of increased TUNEL positivity, the rate of cell death in CR appeared increased. These findings may explain, to some extent, the mechanism of tumor regression.
A case of congenital rhabdomyoma of the heart in a 5-month-old Korean infant is described. The patient presented with a congenital supraventricular tachyarrhymia that was detected in utero by fetal sonography. The tumor was multiple, but no obvious association with tuberous sclerosis complex was demonstrated. Microscopic examination revealed classic "spider cells" with rich glycogen content. Ultrastructurally, the cells contained numerous leptofibrils, clumped Z band material, and desmosome-like cell junctions. The case is a second documented case of cardiac rhabdomyoma in this country, and its presentation as an etiological factor of supraventricular tachycardia is a very unusual manifestation.
The clinical and morphologic features of a recurrent, multifocal, adult, extracardiac rhabdomyoma are reported. This benign skeletal muscle neoplasm is composed of large round to polyhedral cells containing cross striations. The ultrastructural features consist of hypertrophic Z bands, intranuclear inclusions, and numerous mitochondria with linear, intracristal inclusions. The low thymidine labeling index and long potential doubling time correspond wi th the length of time for clinical recurrence.
An unusual benign tumor of skeletal muscle origin is described. The tumor was located in the left retroperitoneum of a newborn female. The tumor contained features of both an adult and fetal rhabdomyoma when studied by light and electron microscopy, immunocytochemistry, and histochemistry. This is the only report of this type of neoplasm in the literature.
We describe a young child with tuberous sclerosis and cardiac rhabdomyoma who developed reversible heart block after being placed on carbamazepine treatment for seizures. Patients of any age with known or suspected intrinsic cardiac disease should be monitored for conduction disturbance if they are treated with carbamazepine.
The angiographic and echocardiographic findings of a right ventricular pedunculated rhabdomyoma prolapsing through the pulmonic valve which mimicked the hypoxic spells of tetralogy of Fallot are presented. A dense mass of echoes was seen within the pulmonic valve during systole. The presence of right ventricular tumor was confirmed by angiography and surgery. The abnormal echoes were no longer seen following surgical excision of the tumor.
Rhabdomyomas are usually found in the myocardium. Of the extracardiac sites, the head and neck is the most frequent region where this rare tumor arises. Multifocal head and neck lesions have only been described in 11 cases. We herein present two additional multifocal lesions.
The case of a parapharyngeal adult extracardiac rhabdomyoma (ER) in a 69-year-old man is presented focusing on computed tomographic diagnosis, surgical approach, and pathologic diagnosis. Although a rare neoplasm, adult ER has a propensity for occurring in the head and neck. Systematic computed tomography evaluation with contrast serves to define the extent of the lesion and assist in the differential diagnosis of parapharyngeal masses. In this case, a transcervical extra-pharyngeal approach for excision was utilized with minimal associated morbidity and a rapid return of normal function. Careful pathologic diagnosis is required to distinguish ER from other neoplasms, especially granular cell myoblastoma.
A unique report of an abdominal wall rhabdomyoma is presented. The rarity of this tumor may lead to confusion with a malignant neoplasm. Computerized axial tomography localized the tumor to a specific muscle group and demonstrated absence of invasion into surrounding tissues. Computerized tomography is valuable in the assessment of abdominal wall tumors and provides useful information in planning an operative approach.
A case of rhabdomyoma of the vagina in a 47-year-old woman is reported. This newly recognised entity is distinct from sarcoma botryoides and the socalled "benign vaginal polyp".