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PubMed1992

Pharyngeal rhabdomyoma: a clinico-pathological study.

Adult extracardiac rhabdomyomas are rare with only 33 head and neck cases being reported in the world literature. In this select group, only nine cases have been found in the pharynx. We present two cases of pharyngeal rhabdomyoma focusing on the imaging findings, surgical approaches and more specifically on the histopathological and electron microscopic diagnosis of this interesting lesion. It is important to differentiate this tumor from other neoplasms, including granular cell myoblastoma, rhabdomyosarcomas, hamartomas, and fetal rhabdomyomas.

Biopsy↗
PubMed1992

Recurrent parapharyngeal rhabdomyoma. Evidence of neoplastic nature of the tumor from cytogenetic study.

A 64-year-old Caucasian male with a left parapharyngeal mass had a past medical history that was significant for excision of a benign rhabdomyoma of the soft palate 30 years previously. Then 25 years ago, the tumor recurred in the palate and retropharyngeal space on the left and was reexcised. Histologic examination of all three excisions showed adult rhabdomyoma. Ultrastructural and histochemical studies of the second excision of this tumor have been published previously. The present study included histologic, ultrastructural, immunohistochemical, and cytogenetic analyses. The histologic and ultrastructural features of the tumor were identical to those reported 25 years ago. Immunohistochemical studies demonstrated that the tumor cells were desmin and myoglobin positive and vimentin negative. Focal positivity for CD56 was also present. Intracellular inclusions in the tumor cells were strongly positive for desmin. Cytogenetic examination of short-term cultures of the tumor cells demonstrated clonal chromosome abnormalities in 60% of metaphases. The majority of cells showed a reciprocal translocation between chromosomes 15 and 17 as the sole abnormality. A minor clone was characterized by abnormalities of the long arm of chromosome 10. The presence of clonal structural chromosome abnormalities in extracardiac adult rhabdomyoma lends strong support to the idea that these rare tumors are true neoplasms rather than hamartomatous or regenerative lesions.

Chromosomes, Human, Pair 15↗
PubMed2004

Nevoid basal cell carcinoma syndrome and fetal rhabdomyoma: a case study.

Fetal rhabdomyoma is not generally considered part of nevoid basal cell carcinoma syndrome. However, a review of the literature revealed five patients with this syndrome who also had fetal rhabdomyomas in various locations. We report the first patient with nevoid basal cell carcinoma syndrome and a fetal rhabdomyoma of the tongue. We recommend that embryonal rhabdomyosarcoma be ruled out to avoid overly aggressive treatment of these patients.

Basal Cell Nevus Syndrome↗
PubMed1991

Incidental cardiac rhabdomyomas: a significant finding necessitating additional investigation at the time of autopsy.

Cardiac rhabdomyomas are rare lesions forming part of the tuberous sclerosis complex that may be responsible for sudden death. As well as remaining clinically occult for variable periods of time, they may, along with other manifestations of tuberous sclerosis, be quite difficult to detect clinically and pathologically. A patient is described in whom multiple cardiac rhabdomyomas were an incidental finding at autopsy following fatal potassium fluoride poisoning. Other gross pathological lesions typical of tuberous sclerosis were present but were quite subtle in appearance. Awareness of the association of cardiac rhabdomyomas with tuberous sclerosis is important so that full examination of organ systems for characteristic lesions can be undertaken during the autopsy, and so that fresh and frozen tissue can be obtained at the time of dissection for further investigation.

Cerebral Cortex↗
PubMed1989

Cardiac rhabdomyoma presenting as sudden infant death syndrome.

A case of cardiac rhabdomyoma presenting as sudden infant death in a four-and-one-half month-old infant is reported. The child was the product of an essentially uncomplicated pregnancy and enjoyed good health before his unexpected, sudden death. Autopsy examination revealed the presence of multiple cardiac lesions which histologically were diagnosed as rhabdomyomas. Death was attributed to fatal cardiac arrhythmia caused by the tumor. To the authors' knowledge this represents the first reported case in the forensic science literature of death as a result of cardiac rhabdomyoma presenting as sudden infant death syndrome (SIDS).

Arrhythmias, Cardiac↗
PubMed1988

[Cardiac rhabdomyoma in a newborn infant with tuberous sclerosis. Value of 2-dimensional and Doppler echocardiography].

Cardiac rhabdomyomas are the most frequent primary cardiac tumors in infancy and childhood and in about 50% of cases are associated with tuberous sclerosis. They are often responsible for a high mortality rate because of their critical location and extent of involvement. This report describes a patient with tuberous sclerosis and multiple cardiac rhabdomyomas, both intramyocardial and intracavitary--diagnosed by two dimensional and Doppler echocardiography at birth. The infant was followed till nine months of age. To date, the tumors have not increased their size and don't provoke moderate or severe obstruction of blood flow. The child is, at present, free from cardiac signs and symptoms, and does not take digitalis or diuretics. We have not detected any arrhythmias. Two-dimensional and Doppler echocardiography are very useful non-invasive techniques in the diagnosis and longitudinal follow-up of patients with cardiac rhabdomyomas.

Echocardiography, Doppler↗
PubMed1987

Cardiac rhabdomyoma associated with tuberous sclerosis. An autopsy case of newborn infant died of cardiac failure.

An autopsy case of cardiac rhabdomyoma associated with tuberous sclerosis in a 27-day-old infant is presented. He was born with severe cyanosis. Echocardiogram revealed the presence of multiple mass lesions, some of which protruded into the left ventricle at the level of subaortic valve. From the age of 4 days, cardiac arrhythmia developed and lasted until his death. The arrhythmia started as WPW syndrome and atrial extrasystoles and then additional paroxysmal supraventricular tachycardia, ventricular fibrillation, sinus arrest, and S-A block occurred. Computed tomographic scanning of the brain revealed the presence of symmetric high-density spots around the central part of lateral ventricle. Before his death paroxysmal supraventricular tachycardia occurred frequently which changed to ventricular fibrillation and he collapsed without urination and then died. Autopsy examination revealed the presence of generalized congestion, multiple nodules of cardiac rhabdomyoma, some of which causing subaortic stenosis, and tuberous sclerosis in the brain. From the clinical and autopsy findings, the direct cause of death was attributable to the cardiac rhabdomyoma.

Heart Neoplasms↗
PubMed1983

Intraoral multifocal adult rhabdomyoma. Report of a case and review of the literature.

Adult rhabdomyomas are uncommon, benign neoplasms of the head and neck region. They are usually solitary, but may rarely be multifocal. We report the clinical and morphologic features of a multifocal adult rhabdomyoma apparently present in the floor of the mouth of an elderly woman for 20 years. At the time of exploratory surgery, the surgeon believed there was diffuse multinodular enlargement of both sublingual glands. The differential diagnosis of a biopsy specimen that was taken for frozen section included salivary gland oncocytosis, a reactive process. On examination of permanent sections, however, the lesion was found to be a multifocal rhabdomyoma. No evidence of salivary gland tissue was found. Ultrastructural study demonstrated the characteristic attempts by this tumor to recapitulate its origin from skeletal muscle by the formation of abnormal contractile elements.

Aged↗
PubMed1984

Nasopharyngeal rhabdomyoma. Report of case (light and electron microscopic studies) and review of the literature.

Rhabdomyoma is an extremely rare benign tumor originating from the skeletal or cardiac muscle cells. Only 87 cases of rhabdomyoma have been reported to date, 70% occurring in the head and neck areas. The authors present the fourth case of rhabdomyoma of the nasopharynx. The tumor appeared in an 18 year old woman with hypacusis. Examination of the epipharynx showed a 2 x 1,5 cm tumor, extending from torus tubarius down to the soft palate. Light microscopy disclosed areas and islands of polymorphous large cells with abundant eosinophilic cytoplasm. Cross striations were focally visible with special PTAH staining. Electron microscopy revealed parallel and haphazardly arranged fibrillar bands (myofibrils) with irregularly and periodically arranged densities corresponding to Z lines. The pecularities of our case were abundant cystically dilated rough endoplasmatic reticulum and the absence of cytoplasmatic glycogen.

Adolescent↗
PubMed1984

Cardiac rhabdomyoma: surgical treatment in the neonate.

Primary cardiac tumors are rare. Of the benign lesions, rhabdomyoma predominates in infants and children. The natural history of patients with cardiac rhabdomyoma is poor. About 50% die in the first month of life and 80% by 1 year of age. We report on the two youngest infants ever operated upon successfully for removal of symptomatic ventricular cardiac rhabdomyoma. The operations were performed through a left ventriculotomy in one and through a right ventriculotomy in the other during the first day of life.

Cardiac Output, Low↗
PubMed1982

[Rhabdomyoma of the heart: three case report (author's transl)].

Authors described three infants under three months of age with cardiac rhabdomyoma observed during a period of eight years. There was no clinical or pathological evidence of tuberous sclerosis. During this period of time they found two cases of tuberous sclerosis, without associated rhabdomyoma, among 2,457 necropsies. On the other hand none of 11 children with tuberous sclerosis under observation in this centre had any clinical evidence of rhabdomyoma. Presenting symptoms of the three cases were cyanosis, heart failure, heart murmurs and alterations of heart rhythm. Diagnosis was suspected on the bases of eco and angiocardiographic studies and was finally confirmed by necropsy. Surgical exeresis of the tumor was attempted in one of the cases, but was not possible because it was too large.

Cardiac Catheterization↗
PubMed1982

Rhabdomyoma: ultrastructural features and distribution of desmin, muscle type of intermediate filament protein.

A case of adult type rhabdomyoma in the neck region of a 77-year-old man is presented. In electron microscopy, some tumour cells showed irregularly orientated sarcomere formation with distinct Z-bands. All cells contained rod-like inclusions with attached thin cytoplasmic filaments. When stained with antibodies against desmin many tumour cells showed a diffuse peroxidase reaction, but the tumour also contained cells with cross striations. After phosphotungstic acid hematoxylin staining, dense cytoplasmic inclusions were seen; these structures showed strong affinity to antibodies against desmin. Our results apparently confirm the suggestion that the rod-like inclusions in rhabdomyoma cells are modified Z-bands. The desmin staining patterns in the present tumour may represent different stages in disorderly proliferation and degeneration of rhabdomyoma cells.

Aged↗
PubMed1980

[Congenital multiple rhabdomyoma of the heart].

An observation of a rare congenital multiple tumor of the heart with histological structure of rhabdomyoma is described. Of interest is the presence in the child with congenital rhabdomyoma of the heart of dysplastic changes in the kidneys in the form of few small cysts and in the brain in the form of angiomatous structures. Such combination confirms dysontogenetic origin of rhabdomyoma.

Heart Neoplasms↗
PubMed1994

[Natural history of cardiac rhabdomyoma. Presentation of 2 cases with immunohistochemical study and review of the literature].

Two cases of cardiac rhabdomyoma have recently been observed in foetuses. The cardiac and muscular nature of the cells was confirmed by immunohistochemistry using monoclonal antibodies. A review of the literature shows that cardiac rhabdomyomas are usually considered to be hamartomas which are composed of primitive myocardial cells. Their spontaneous regression is often observed and apoptosis has been proposed as a possible mechanism of such a phenomenon. We propose that cardiac rhabdomyomas should be better considered as developmental vestiges rather than as true hamartomas. These vestigial structures would result either from a defect in differentiation, or from a defect in regression of primitive myocardial cells.

Abortion, Therapeutic↗
PubMed1993

An infant with rhabdomyoma prenatally diagnosed in a mother with tuberous sclerosis.

We report on an infant with rhabdomyoma diagnosed prenatally by echocardiography which revealed specific patterns of intracardiac tumors. The mother had been previously diagnosed with tuberous sclerosis. Since birth, the infant's intracardiac tumor has regressed and he has shown good growth and development without surgery in more than one year of follow-up. In tuberous sclerosis, a cardiac rhabdomyoma is the only sign that can be detected prenatally by ultrasound. Even though a cardiac rhabdomyoma occasionally induces poor outcome, the need for surgery depends on the patient's clinical presentation.

Adult↗
PubMed1993

Adult-type rhabdomyoma of the submandibular region.

Adult-type rhabdomyoma is a rare benign tumor which occurs mainly in the head and neck region of middle-aged patients. We present a case of rhabdomyoma of the submandibular region. Clinical and histologic features are discussed as well as differential diagnosis and proper treatment. Finally, all previous reports of adult rhabdomyoma cases are reviewed.

Cell Nucleus↗
PubMedDate not supplied

Fetal rhabdomyoma: two instances of recurrence.

A congenital fetal rhabdomyoma was removed from the neck of a male infant on the second day of life. The lesion recurred 10 years later with histological features suggestive of increased differentiation and no evidence of malignant transformation. An unrelated patient of 3 years suffered from a recurrent fetal rhabdomyoma 4 months after the initial resection. The possibility of early or late recurrence must be considered when a diagnosis of fetal-type rhabdomyoma is made.

Child, Preschool↗
PubMed1998

[Rhabdomyoma of the right ventricle associated with Behcet disease simulating the presence of a malignant tumor].

OBJECTIVE OF THE STUDY: To present the clinical case of a patient suffering from a rhabdomyoma associated to Behçet's disease. PATIENTS: A 35-year-old man with a history of high fever associated to oral ulcers, sinus tachycardia, leukocytosis and a high erythrocyte sedimentation rate. Physical examination, chest x-ray, blood chemistry, serology and rheumatic tests were normal. Seriate sputum cultures, urocultures and blood cultures were negative. The echocardiogram showed a non-obstructive mass in the right ventricle, which was confirmed by magnetic resonance imaging. INTERVENTIONS: Resection of the tumor under cardiopulmonary bypass. After the post-operative recurrence of the fever and the appearance of genital ulcers, the diagnosis of Behçet's disease was made. Two and a half years after the operation the patient remains asymptomatic under treatment with colchicine. CONCLUSIONS: The clinical setting suggested a malignant tumor of the right ventricle but the pathological findings showed an adult rhabdomyoma. The rarity of finding a rhabdomyoma in an adult is stressed, as well as its association with Behçet's disease.

Adult↗