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PubMed1980

New fine structural features of cardiac rhabdomyoma: report of a case.

Electron microscopy of a cardiac rhabdomyoma revealed two varieties of cells: (1) typical "spider cells' characterized by few myofibrils and large aggregates of glycogen particles free in the cytoplasm; and (2) cells with more myofibrils, less glycogen, and glycogen within membrane-limited vacuoles. These differences in myofibril development and glycogen content represent two succeeding stages in the early maturation of normal cardiocytes. The vacuoles containing glycogen were interpreted as autophagic and, consequently, active in the glycogen decrease typical of normal maturing cardiocytes. Rhabdomyoma cells did not show the structures, e.g., T system, characteristic of the late normal heart development. This study supports the theory that arrested cardiocyte maturation is a factor in the pathogenesis of cardiac rhabdomyoma.

Female↗
PubMed1986

Extracardiac rhabdomyoma: a report of two cases.

Two case reports of extracardiac rhabdomyoma (adult type) are presented. The rare occurrence of extracardiac rhabdomyoma and its relatively benign nature makes a histological diagnosis very necessary, though often difficult to make. In both cases reported here, a diagnosis of extracardiac rhabdomyoma was given and treatment with local excision yielded good results. The importance of a histological diagnosis and treatment by only a local excision is stressed.

Adult↗
PubMed1978

[Intracristal linear inclusions in mitochondria of human rhabdomyoma cells (author's transl)].

Electron microscopic study of a rhabdomyoma of the soft palate of a 50-year-old woman revealed linear structures within the intracristal spaces of mitochondria. In the center of most cristae, an electron-dense line was found located in parallel arrangement to the limiting cristal membrane. The linear inclusion was separated from the cristal membrane by a narrow space of lower electron-density. Thus, when sectioned longitudinally, the abnormal cristae exhibited a pentalaminar appearance. Additionally, they were characterized by a greater diameter than that of normal cristae and a "rigid" configuration. Higher magnification showed that the intracristal line consisted of a row of periodically arranged, electron-dense dots. Sometimes, these seemed to be connected with the wall of the intracristal space by fine, electron-dense cross-striations. In some mitochondria of rhabdomyoma cells, crystals with a linear substructure were observed in both intra- and extracristal spaces. The crystalline bodies located within cristae had probably been formed by accumulation of linear inclusions. The extracristal bodies seemed to have arisen from fusion of pentalaminar cristae. It is supposed that the intracristal linear inclusions in mitochondria of rhabdomyoma cells represent crystallized proteins of mitochondrial enzymes.

Female↗
PubMed1989

Rhabdomyoma of the larynx recurring after 12 years: immunocytochemistry and differential diagnosis.

Rhabdomyoma is a rare tumor of skeletal muscle origin which can occur in different localizations. The head and neck region is a site of predilection for the adult type. Its absolutely benign behavior makes correct diagnosis crucial. In this study, a case of an extracardiac rhabdomyoma of the larynx was found to recur 12 years after initial treatment. Following its surgical removal, histology showed typical patterns of an adult type of rhabdomyoma with focal cross-striations. Immunocytochemically, all applied muscular markers were positive. Electron microscopy revealed muscular filaments with cross-striations and glycogen granules. These findings showed that immunocytochemistry plays an important role in differential diagnosis and the need for possible further treatment.

Diagnosis, Differential↗
PubMed1997

Perianal fetal rhabdomyoma: case report.

A 4-week-old female was referred with a firm, enlarging perianal mass present since birth. At surgery, the lesion appeared to be arising from the subcutaneous external sphincter. Local excision was successful with preservation of the external sphincter. Histologic examination showed a mixture of undifferentiated spindle cells as well as immature and maturing myofibers consistent with a fetal rhabdomyoma. This report represents the second case of a fetal rhabdomyoma arising in the perianal location. All pediatric cases of fetal rhabdomyoma in the literature are reviewed.

Anal Canal↗
PubMed1990

Prenatal ultrasound diagnosis of multiple cardiac rhabdomyomas.

We report on a neonate with multiple cardiac rhabdomyomas, including a huge rhabdomyoma of the left ventricular posterior wall. Prenatal ultrasonography performed because of supraventricular tachycardia led to the diagnosis in the 28th week of gestation. Postnatal echocardiography confirmed the prenatal diagnosis. The tumor could not be removed surgically and the child died as a result of intractable arrhythmia at the age of 5 days. The diagnosis of multiple cardiac rhabdomyomas was confirmed by autopsy.

Female↗
PubMed2000

Bilateral recurrent adult rhabdomyomas of the pharyngeal wall.

Adult rhabdomyomas of the head and neck are uncommon benign skeletal muscle tumors. Only a few cases occurring in the pharyngeal wall have been described in the world literature. We present a case of recurrent bilateral rhabdomyomas in the pharynx and discuss the clinicopathological features of this lesion, comparing it to those of other neoplasms from which it must be differentiated. To our knowledge, bilaterality of this type of lesion has not been described previously. Although adult rhabdomyomas have a distinct histology, they often are mistaken for a variety of other lesions, particularly granular cell tumor.

Adult↗
PubMed2001

[Adult multilocular rhabdomyoma as etiology of a space-occupying lesion in the area of the neck].

Real rhabdomyomas belong to the rarest tumors in the human body. The origin of these benign neoplasms is in the cardiac or skeletal muscle, in the beginning, there are no significant symptoms and the tumor grows very slowly. Extracardiac rhabdomyomas seldom occur and are mostly located in the head and neck region. A multilocular occurrence represents a rarity. We describe a 77-year-old female patient with a multilocular adult rhabdomyoma in the parapharyngeal and paratracheal space. The tumor was accidentally found; the patient had not complained of any specific symptoms. After histological examination, complete resection was carried out. On discharge the patient showed no functional deficits. Long-term follow-up is necessary because local recurrences have been described.

Aged↗
PubMed2006

Mediastinal rhabdomyoma: case report and review of the literature.

Rhabdomyomas are benign tumors in which at least some cells are differentiated as skeletal muscle cells with cytoplasmic cross-striations. Extracardiac adult rhabdomyoma is an extremely uncommon benign neoplasm that usually involves the head and neck region. Rare cases have been reported to involve other sites of the body including mediastinum. We report the fourth case of mediastinal adult rhabdomyoma.

Actins↗
PubMedDate not supplied

Localized nodular hypertrophy mimicking rhabdomyoma in the fetal heart: prenatal sonographic and pathology findings.

Multiple intracardiac masses (ICM) are considered to be diagnostic of rhabdomyoma often associated with tuberous sclerosis. We describe a fetus with multiple ICM detected by fetal sonography at 18.7 wk gestation. The appearance and number were consistent with leading diagnosis of rhabdomyoma. Due to complications of pregnancy and extreme prematurity, the fetus did not survive. Autopsy showed the cardiac masses to be localized nodular hypertrophy (LNH) of the myocardium. No features of rhabdomyoma or tuberous sclerosis were present. In a review of the literature, similar lesions were reported in a child and two adults, perhaps as localized variants of hypertrophic cardiomyopathy. Our case does not, however, show the histopathologic features of hypertrophic cardiomyopathy. Isolated developmental abnormalities, such as in this case, can have a mass effect mimicking cardiac tumors. At the time of autopsy, the largest mass in the anterior wall of the right ventricle extended to and obstructed the right ventricular outflow tract.

Adult↗
PubMed1990

Natural history of cardiac rhabdomyoma in infancy and childhood.

Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis is still considered to be poor and surgery continues to be indicated. The experience with rhabdomyoma diagnosed in live infants over a 20-year period was reviewed. Diagnosis by angiography or echocardiography was accepted only if multiple tumors were present or if tuberous sclerosis was also diagnosed. Nine patients (3 diagnosed prenatally and the remaining 6 at age less than 8 months) were identified as having a total of 24 tumors. Measurements in 2 planes demonstrated at least some evidence of regression in 24 patients (100%), with 20 of 24 having complete resolution. One patient required delayed surgery for excision of a subaortic ridge that appeared at the site of a resolved tumor. Our findings suggest that pediatric cardiac rhabdomyoma is most often a benign condition in which spontaneous regression is the rule. Surgery is recommended only for patients with refractory dysrhythmias or severe hemodynamic compromise.

Child↗
PubMed1983

Fetal rhabdomyoma of the tongue, with a discussion of the two histologic variants of this tumor.

Fetal rhabdomyomas are rare benign tumors of skeletal muscle that must be distinguished histologically from various sarcomas, including rhabdomyosarcoma. They have recently been subdivided into two histologic variants myxoid and cellular fetal rhabdomyomas, respectively. This article describes the sixth fetal rhabdomyoma to be reported in the oral regions, illustrates the histologic features of the two types, and discusses the means of distinguishing these lesions from various other conditions with which they may be confused.

Diagnosis, Differential↗
PubMed1983

Multifocal adult rhabdomyoma.

It is not well recognized that a small but significant proportion of extracardiac adult rhabdomyomas have occurred multifocally. The present example of a 60-year-old man with adult rhabdomyomas of the larynx and submandibular region is the seventh multifocal case out of approximately sixty adult rhabdomyomas reported in the literature. Previously published cases are summarized in this article.

Head and Neck Neoplasms↗
PubMed1996

Multiple cardiac rhabdomyoma with exclusively histological manifestation.

Rhabdomyomas of the heart are rare lesions and usually exhibit macroscopic tumour nodules at autopsy. The uncommon death of an apparently healthy 2 1/2-month-old female infant is described. Post-mortem examination revealed normal gross cardiac morphology and exclusively histological manifestation of multifocal rhabdomyoma. The microscopic and immunohistochemical features are demonstrated. Tuberous sclerosis, frequently associated with this type of hamartoma was not present. The case confirms again the importance of additional histological investigations in unexpected fatalities in infancy: beside the clarification of death, autopsy may obtain a prophylactic function in the detection of possibly genetically determined diseases such as cardiac rhabdomyoma.

Female↗
PubMed2005

Ovarian serous cystadenoma with mural nodules of genital rhabdomyoma.

We present an extremely rare case of ovarian serous cystadenoma with mural nodules of rhabdomyoma. The patient, a 48-year-old woman, was admitted to our hospital with left lower abdominal pain and vaginal bleeding. A unilocular cystic tumor, measuring 13 x 10 x 10 cm, was found in her left ovary and was removed. The tumor contained clear serous fluid, approximately 600 mL, and 2 mural nodules, up to 7.5 x 5.5 x 4.5 cm. The internal cystic wall was thin for the most part and lined with ciliated cuboidal epithelium without any malignant feature. The mural part was composed of mainly more mature muscle fibers with easily discernible cross-striations, set in abundant myxoid to fibromyxoid stroma, similar to clinical and microscopic manifestations of genital rhabdomyomas reported in other sites. Because extracardiac rhabdomyoma has never been described occurring in the ovary, especially arising in serous cystadenoma, to our knowledge, this is the first case reported in the English literature.

Cystadenoma, Serous↗
PubMed1975

Rhabdomyoma (adult type) of the larynx.

A rhabdomyoma successfully removed from the right false cord of a 52-year-old woman, who had been disphonic for 3 years, is reported. Histologically the tumor showed the features of the adult type rhabdomyoma. The pathologic appearance and the biologic behavior of the rhabdomyoma have been well established in the recent literature.

Female↗
PubMed2003

Paratesticular rhabdomyoma.

A case of rhabdomyoma of the spermatic cord in an infant with cryptorchidism is reported. This extremely rare tumor has not been described in an extrascrotal position or at such a young age. Differential diagnosis includes malignant rhabdomyosarcoma. Although fetal-type rhabdomyoma of the head and neck generally is a benign tumor, the prognosis of paratesticular fetal-type rhabdomyoma is unknown. Long-term follow-up is recommended.

Genital Neoplasms, Male↗
PubMed1987

Adult rhabdomyoma of the esophagus.

Rhabdomyoma is a rare benign tumor of striated muscle origin. Two distinct histologic groups, adult and fetal type, are recognized. So far only 66 cases of extracardiac rhabdomyomas (37 fetal and 29 adult) have been documented in the literature reviewed. The present report details a case of adult rhabdomyoma of the esophagus in an 8-year-old boy.

Child↗