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PubMed1976

Nonepithelial tumors of the nasal cavity paranasal sinuses, and nasopharynx: a clinicopathologic study. V. Skeletal muscle tumors (rhabdomyoma and rhabdomyosarcoma).

In a study of nonepithelial tumors involving the nasal cavity, paranasal sinuses, and nasopharynx, 19 lesions (three rhabdomyomas, 16 rhabdomyosarcomas) showed skeletal muscle differentiation. The clinical findings associated with these neoplasms are reviewed, the histologic features are illustrated, results of treatment are presented, and clinicopathologic correlations are discussed. The rhabdomyomas grew as localized masses in the nasopharynx, were excised locally, and behaved clinically like benign tumors. The rhabdomyosarcomas were found predominantly in children and were associated with a poor prognosis; most of the patients died within 2 years following diagnosis.

Adolescent↗
PubMed1978

Mediastinal rhabdomyoma.

An 80-year-old black male with gastric adenocarcinoma, liver metastases, and parathyroid adenomas also had a rhabdomyoma of the anterior-superior mediastinum. This appears to be the first reported case of mediastinal rhabdomyoma in the literature. The ultrastructural demonstration of haphazardly arranged myofilaments with prominent Z bands and the absence of demosomes indicates that it is of extra-cardiac type. The probable origin of this tumor from the myoid cells of the thymus is discussed.

Adenocarcinoma↗
PubMed2003

Fine-needle aspiration of adult rhabdomyoma: a case report with review of the literature.

The cytologic features of adult rhabdomyoma, a rare benign tumor of skeletal muscle origin, have been infrequently reported in the literature. We present here a case of a rhabdomyoma involving the floor of the mouth of a 78-year-old man initially diagnosed by fine-needle aspiration cytology. Cytologic features seen on Papanicolaou-stained smear preparation included cohesive clusters of skeletal muscle cells having abundant eosinophilic cytoplasm and often peripherally located nuclei. Although cross-striations and elongated intracytoplasmic inclusions were not identified in the smears, they were noted in the cell block preparation of the aspirated specimen and in the touch preparation and histologic sections of the surgically resected specimen. The cytologic differential diagnosis of this tumor is discussed.

Aged↗
PubMed1988

Fine-needle aspiration cytology of an adult rhabdomyoma of the head and neck.

The cytologic features of a multifocal adult rhabdomyoma of the head and neck, as observed in a fine-needle aspiration specimen, are described. Cross-striations and characteristic cytoplasmic "crystals" were conspicuous in the cytologic preparations and also during ultrastructural examination. The cytologic features of adult rhabdomyoma are distinctive, allowing a rapid specific diagnosis.

Aged↗
PubMed1990

Recurrent multifocal adult rhabdomyoma diagnosed by fine-needle aspiration cytology: report of a case and review of the literature.

The fine needle aspiration (FNA) cytology of a recurrent multifocal extracardiac adult rhabdomyoma is described, and the literature is reviewed. The patient presented with dysphagia and bilateral palpable neck masses 21 yr after resection of a rhabdomyoma of the tongue. The clinical differential diagnoses included ptotic submandibular glands and lymphadenopathy. The aspiration smears and cytospin preparations contained large polygonal cells with abundant granular cytoplasm with indistinct borders and uniform, peripherally located nuclei. Cross-striations were identified within the cytoplasm of some cells on Papanicolaou and modified Wright-Giemsa stains. This case represents only the fourth description of the cytology of this entity and the first reported case of a recurrence diagnosed by FNA. The characteristic cytomorphologic features enabled a definitive diagnosis to be made 21 yr after the original resection, sparing a poor-risk patient a debilitating surgical procedure for a benign, slow-growing neoplasm.

Aged↗
PubMed2003

Rhabdomyoma of the head and neck: clinicopathologic features of two cases.

BACKGROUND: Extracardiac rhabdomyomas are rare benign tumors of the head and neck. They are frequently misdiagnosed and possibly overtreated when confused with other aggressive tumors. METHODS: This article reports on the clinicopathologic features of two such tumors affecting adult patients and initially seen as slowly-growing, indolent neoplasms. RESULTS: The tumors were of fibrous consistency, mobile, with well-demarcated borders and evident lines of cleavage. Both patients were treated by conservative surgery and remained disease-free after prolonged clinical observation. Histologically, both tumors were composed of large eosinophilic cells intermingled with polyhedral clear cells. Both cell types contained abundant glycogen accumulations. CONCLUSIONS: The differential diagnosis is with granular cell tumor and with sarcomas, the former being easily distinguishable morphologically, and the latter be seen with more rapid growth and adherence to the adjacent tissues. On the basis of the preceding features, rhabdomyomas can be suspected at a preoperative stage and adequately managed with enucleation or simple excision.

Female↗
PubMed2006

Adult extracardiac rhabdomyoma: light and immunohistochemical studies of two cases in the parapharyngeal space.

BACKGROUND: We present two cases of adult rhabdomyoma in the parapharyngeal space. They are rare benign tumors with a characteristic histologic appearance. METHODS: The tumors were studied by light and immunohistochemical analysis using stains characteristic of striated muscle fibers. RESULTS: Cross-striation was demonstrated by phosphotungstic acid hematoxylin (PTAH), muscle specific actin, desmin, and myoglobin while dystrophin was expressed in the cell membranes. Clonal origin was confirmed by expression of myosin heavy chain-fast only. Expression of myosin-neonatal and myogenin proved slight proliferation with incipient differentiation in an otherwise mature tumor. CONCLUSION: The head and neck area harbors 90% of adult rhabdomyomas and should be considered in a differential diagnosis in this region. Immunohistochemistry confirms that the tumors are almost totally mature neoplasms of clonal origin.

Aged↗
PubMed1993

Uterine mixed embryonal rhabdomyosarcoma and fetal rhabdomyoma.

A polypoid uterine tumor, occurring in a 31-year-old woman, with histopathologic features of both embryonal rhabdomyosarcoma and fetal rhabdomyoma is reported. The clinical and pathologic differences between pure fetal rhabdomyoma and embryonal rhabdomyosarcoma are detailed and theories concerning the histogenesis of such a mixed, or intermediate, tumor are discussed. Most likely, this neoplasm represents one example in a spectrum of tumors with varying proportions of immature and mature skeletal muscle elements. Its good prognosis is in keeping with that of uterine (cervical) embryonal rhabdomyosarcomas in general, although the presence of mature skeletal muscle elements may signify an even better prognostic group of tumors.

Adult↗
PubMedDate not supplied

Heart transplantation in an infant with rhabdomyoma.

Rhabdomyoma is the most common primary cardiac tumor in infants and children and is often associated with tuberous sclerosis. Surgical resection may be indicated and, if so, is usually curative. We describe a rhabdomyoma in an infant who presented with severe myocardial ischemia necessitating orthotopic heart transplantation.

Diagnosis, Differential↗
PubMed1980

Solitary rhabdomyoma of the heart. Clinically silent case with sudden, unexpected death in an 11-month-old boy.

We report a case of cardiac rhabdomyoma leading to sudden, unexpected death in an 11-month-old child. Autopsy disclosed a firm nodular tumor located in the anterior part of the interventricular septum and anterior wall of the left chamber. The tumor bulged into the cavity of the ventricle and caused appreciable narrowing of the outflow tract. Histologically, the typical "spider cells" characteristic for cardiac rhabdomyoma could be found. Sections cut from alcohol-fixed tissues and stained in water-free PAS showed that these cells contained massive amounts of diastase-digestible PAS-positive material, presumably glycogen. No evidence of tuberous sclerosis was detected. Sudden death was attributed to cardiac arrest due to irritation of the conducting system by the tumor.

Autopsy↗
PubMed2003

Cardiac rhabdomyoma and tuberous sclerosis: prenatal diagnosis and follow-up.

This article reports 4 cases with cardiac rhabdomyomas diagnosed during intrauterine life. Echocardiographic follow-up at 9-30 months showed regression of the tumors in 3 cases (75%). Two cases (50%) developed tuberous sclerosis during that period. Fetal echocardiography promotes early diagnosis of tuberous sclerosis through prenatal detection of cardiac rhabdomyoma, and facilitates genetic counselling of families at risk.

Echocardiography↗
PubMedDate not supplied

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.

Abnormalities, Multiple↗
PubMed2002

Rhabdomyoma of the orbit: a case report.

Extracardiac rhabdomyoma is a rare benign neoplasm of skeletal muscle differentiation. It occurs most often in the head and neck, but only four cases with occurrence in the orbit have been reported in the literature. A 3-month-old infant presented with right proptosis. MRI showed an enhancing intraconal mass in the right orbit, which appeared isointense on TI-weighted images and hypointense on T2-weighted images. The mass was removed and diagnosed as rhabdomyoma. It recurred 8 months after surgery. MR imaging features can be helpful for the diagnosis.

Humans↗
PubMed1979

Intraoral rhabdomyomas.

Review of the world literature revealed 16 cases of intraoral rhabdomyomas. Thirteen additional cases of intraoral rhabdomyomas from the files of the Armed Forces Institute of Pathology are reported. The neoplasm occurred principally in adults (mean age 56 years), and the male-female ratio was greater than 2:1. The majority of cases occurred in the floor of the mouth, followed by the soft palate, tongue, and buccal mucosa. Recurrences occurred in four (30 percent) cases, and there was one multiple recurrence. One residual tumor, the result of incomplete surgical removal was noted. Although these tumors have a characteristic microscopic appearance, they were frequently misinterpreted. The size, clinical presentation, and growth pattern of these tumors may reflect their ultimate behavior and prognosis.

Adolescent↗
PubMed1994

Adult rhabdomyoma. A light microscopic, ultrastructural, virologic, and immunologic analysis.

Adult rhabdomyoma is a rare benign tumor of skeletal muscle origin that frequently arises from musculature of the head and neck region. In this study we report five cases of adult rhabdomyoma. Light and electron microscopic examination revealed the typical cellular features as described by others. The results of immunophenotyping and quantitation of nucleolar organizer regions were consistent for a benign skeletal muscle tumor. Epstein-Barr virus and human papillomavirus genomic DNA could not be detected by in situ hybridization.

Actins↗
PubMed1993

Adult intracardiac rhabdomyoma resembling the extracardiac variant.

Rhabdomyomas are benign striated muscle neoplasms that may assume a number of characteristic histologic patterns. These lesions may be classified as cardiac or extracardiac on the basis of their location and histology. We present a case of large intracardiac mass with the morphologic features of an extracardiac rhabdomyoma occurring in an adult female.

Adult↗
PubMedDate not supplied

Multifocal adult rhabdomyoma: a case report and literature review.

Adult extracardiac rhabdomyoma is an uncommon primary tumor of striated muscle origin that almost exclusively presents in the head and neck region. The occurrence of multifocality is a rare manifestation of this unusual lesion. In this study, we report a rare case of multifocal adult extracardiac rhabdomyoma in a patient presenting with an asymptomatic neck mass and provide a brief review of the literature on this entity.

Aged↗
PubMed2000

Surgical resection of an intracardiac rhabdomyoma.

There are only a few previous reports of intracardiac rhabdomyomas causing ventricular arrhythmias and near syncope. In this report we describe the successful surgical resection of an intracardiac rhabdomyoma using cardiopulmonary bypass, blood cardioplegia, and hypothermia. Preoperative evaluation consisting of echocardiography, computed tomography (CT), magnet resonance imaging (MRI), and positron emission tomography (PET) strongly suggested the presence of a symptomatic primary cardiac tumor projecting from the interventricular septum into the right ventricle.

Adult↗