SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case of pharyngeal rhabdomyoma is reported with the unusual presenting symptom of haemoptysis. After complete excision of the lesion, the(71-year-old) patient remained tumour-free until his death from heart disease 19 months later. Seven other published cases of pharyngeal rhabdomyoma are reviewed together with 27 other reported extracardiac adult rhabdomyomas which, with a single exception, occurred in the head and neck region.
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.
We present 3 adults with cardiac rhabdomyomas, 2 in the atria and 1 in the right ventricle. One atrial tumor was discovered incidentally, and 1 resulted in supraventricular tachycardia. The ventricular lesion caused ventricular tachycardia. Compared with congenital rhabdomyomas, the tumors are relatively cellular, the cells are smaller, there are few spider cells, and there is evidence of cell proliferation. Two of the 3 tumors demonstrated spindling in contrast to adult rhabdomyomas of the head and neck. Although surgical excision was possible in all patients, long-term follow-up will be required to determine the true biologic behavior of these neoplasms.
Cardiac rhabdomyomas are benign tumours closely associated with tuberous sclerosis. We report on a neonate with a cardiac rhabdomyoma, which simulated tricuspid atresia with duct-depending pulmonary perfusion due to almost complete obliteration of right ventricular cavum. Under infusion of prostaglandin E1 the newborn stabilized and was successfully operated on the 3rd day of life. We conclude that successful tumour resection is possible in neonates with cardiac rhabdomyomas causing relevant hemodynamic obstruction.
Rhabdomyomas, which are rare striated muscle neoplasms, may occur on oral or genital mucosal surfaces as well as on the skin. Little discussion of rhabdomyomas occurs in any dermatology or dermatopathology literature. We report an 84-year-old patient who had a rhabdomyoma of her lateral tongue and discuss the clinical and histologic characteristics of this unusual type of benign neoplasm.
The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tuberous sclerosis. In a retrospective study, 33 children with cardiac rhabdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of cardiac rhabdomyoma was found in infancy, with 21/23 detected before the age of 1 year, and 11/33 before 1 month of age. Cardiac manifestations were present in 19 patients: cardiac rhythm disturbances were detected in 13; in 6/33 a Wolff-Parkinson-White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitative phenomena were present in 5; and in 2 patients surgical removal proved necessary. With the exception of one tumoural mass in the right atrium, all 77 tumours were located somewhere in the ventricles, including at atrio-ventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomatic, while surgical removal is required only in life-threatening conditions, as documented in 2 of our 33 patients.
BACKGROUND: The cytologic diagnosis of extracardiac rhabdomyoma is frequently hampered by its rarity and resemblance to various tumors. In this regard, the infrequent occurrence has hindered its prompt and early recognition. It is also confused with other tumors because of similarities in clinical and cytologic presentations. CASE: A submandibular rhabdomyoma occurred in an otherwise-healthy, 62-year-old man. The neoplasm was firstly diagnosed by fine needle aspiration cytology (FNAC. Complete local excision without radical surgery was performed. Histologic findings confirmed the cytologic diagnosis of adult rhabdomyoma. Treatment-related complications were minimal, and there was no evidence of recurrent disease 6 years later. CONCLUSION: Helpful FNAC features and immunocytochemical results permitted an early diagnosis and spared the patient unnecessary radical surgery.
BACKGROUND: Fetal rhabdomyoma is a relatively rare tumor that occurs mainly in the head and neck of toddlers. A similar lesion can occasionally be found in the adult female genital tract. Increased cellularity may be seen in the fetal type and can lead to confusion with the well-differentiated types of rhabdomyosarcoma. CASE: An 8-day-old infant presented with a unilateral, right-sided, postauricular mass. Intraoperative cytology of the mass showed numerous cells with spindled nuclei in a background of myxoid material. A provisional diagnosis of fetal rhabdomyoma was made and subsequently confirmed by histopathologic examination. CONCLUSION: In neonates presenting with cervical swelling, the possibility of fetal rhabdomyoma should always be considered and confirmed by both intraoperative cytology and histopathologic examination of the resected tumor.
We report a child in whom multiple cardiac rhabdomyomas were identified on routine fetal ultrasonography. Molecular genetic studies identified the TSC2 gene missense mutation (E36; 4672 G>A, 1558 E>K TSC2). Both general and neurodevelopment of the patient have been normal. When last examined at age 6 years, he had no skin manifestations of tuberous sclerosis complex. Computed tomography of the brain revealed two periventricular calcifications consistent with the molecular diagnosis. This is the first report of molecularly confirmed tuberous sclerosis complex in a child with multiple cardiac rhabdomyomas and no other clinical manifestations of the disease. We propose that all cases of multiple cardiac rhabdomyomas in an infant be given a tentative diagnosis of tuberous sclerosis complex.
Cardiac rhabdomyomas are extremely uncommon in the adult patient. We describe a previously healthy man who presented with ventricular arrhythmias resulting from a right ventricular, cardiac rhabdomyoma. Echocardiography, CT scanning, and MRI are recognized as useful diagnostic modalities for intracardiac lesions. Cardiac catheterization in our patient demonstrated the presence of a tumor blush. This has not previously been reported with cardiac rhabdomyomas. Although lesions may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrhythmia.
Clinical, catheterization, and pathologic findings were recorded in a newborn infant with tuberous sclerosis and multiple cardiac rhabdomyomas that produced a clinical picture simulating mitral atresia and the hypoplastic left-heart syndrome. The clinical picture was due to a left atrial tumor that completely obstructed the mitral valvular orifice. Even if the diagnosis of left atrial tumor had been made, successful surgical correction was unlikely because of left ventricular rhabdomyomas, which produced severe subvalvular aortic stenosis and did not appear to be resectable. This case demonstrates the possibility that a hamartoma, such as a rhabdomyoma, occasionally can mimic the hypoplastic left-heart syndrome.
Fetal rhabdomyoma is a rare benign neoplasm of skeletal muscle that must be distinguished histologically from various malignant lesions, including rhabdomyosarcoma. They have recently been subdivided into two histological variants myxoid and cellular fetal rhabdomyomas. This article describes a case of an 8 month infant with a fetal rhabdomyoma in the upper lip. Illustrate the histological features and discusses the means of distinguishing these lesions from various other conditions with which they may be confused.
Nerve rhabdomyomas are exceedingly rare benign tumors of the peripheral nerves consisting of well-differentiated striated muscle fibers admixed with parental nerve fibers. Only one case of intracranial nerve rhabdomyoma has been described, which affected the trigeminal nerve. This report presents the detailed neuropathological description of a nerve rhabdomyoma arising in the schwannian portion of the facial nerve root in a 41-year-old Caucasian man. The nerve fibers were arranged chaotically as in a traumatic neuroma. Because of the intimate intermingling of this slow-growing tumor with the parental nerve fibers, complete excision should be avoided.
A cardiac rhabdomyoma is described in a 6-wk-old captive fallow deer (Dama dama) that died suddenly without previous clinical signs. The tumor was characterized by multiple nodules composed of large atypical vacuolated myoblastic cells. As previously reported in humans and other animal species, there is compelling evidence that the cardiac rhabdomyoma is a congenital developmental anomaly rather than a true neoplasm. To our knowledge, this is the first report of a cardiac tumor and a rhabdomyoma in a cervid species.
Adult rhabdomyoma is a rare neoplasm of mesenchymal origin which represents approximately 2% of tumors with skeletal muscle differentiation. There are only about 100 cases reported in the literature. It is a benign tumor occurring most frequently in the head and neck region, which affects predominantly male population of a mean age ranging from 54 to 60. The purpose of this article is to present a new case of adult rhabdomyoma located in the masticatory area, arising as a slow-growing mass bulging in the left-side jugal mucosa and temporal region. The most common location for adult rhabdomyoma is the pharyngeal cavity. This type of tumor appears as a solitary mass, though occasionally may be multifocal. This tumor being of low occurrence, correct diagnosis can prevent aggressive surgery.
Rhabdomyoma in adults is a rare benign neoplasm of the skeletal muscles generally arising in the pharyngolaryngeal region. These slow-growing tumors remain asymptomatic for a long period. We report a case of basilingual rhabdomyoma. Computed tomography and magnetic resonance imaging demonstrated a well-encapsulated tumor with a line of cleavage between the base of the tongue muscles. Deep transmucosal biopsies were obtained. Well-differentiated skeletal muscle cells with large eosinophilic granulular cytoplasm and cross striations gave the histologic diagnosis. Complete endoscopic resection was successful. The differential diagnoses of rhabdomyoma in adults are myoblastoma or Abrikossof tumor, rhabdomyosarcoma and hibernoma. The risk of late recurrence implies long-term clinical and radiologic follow-up.
Cardiac rhabdomyoma, the most common cardiac tumor of infant and child, has been known to be associated with tuberous sclerosis complex. However, tuberous sclerosis complex with fetal supraventricular tachycardia is rarely reported. Herein, we report a female neonate diagnosed as tuberous sclerosis complex with cardiac rhabdomyoma presenting as supraventricular tachycardia. The newborn infant was initially treated with vagal maneuver, adenosine push, and digoxin. Although the tachycardia converted into normal sinus rhythm, frequent recurrence of PSVT was noted. At 16 days of age, the recurrent supraventricular tachycardia was refractory to vagal maneuver and drug therapy. Direct-current synchronized cardioversion converted the arrhythmia to normal sinus rhythm and oral digoxin effectively controlled the condition of the patient without further PSVT attacks. Subsequent 9-month follow-up revealed no further attack of arrhythmia. Regression of cardiac rhabdomyoma has not been visualized on echocardiogram at 9 months old.
BACKGROUND: Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. Follow-up studies of changes in electrocardiographic findings are also lacking. METHODS: We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings. RESULTS: Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour. CONCLUSIONS: The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might- not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.