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At least 199 records · Page 11Linked to original sources
PubMed1996

Crystal-storing histiocytosis associated with a lymphoplasmacytic neoplasm mimicking adult rhabdomyoma: a case report and review of the literature.

Massive accumulation of crystals within histiocytes in association with a lymphoplasmacytic neoplasm causing histologic features closely mimicking adult rhabdomyoma is a rare occurrence. We report the case of a 49-year-old man who presented with a left posterior cervical mass. Histologic examination demonstrated sheets of large, elongated, and polygonal cells with prominent eosinophilic cytoplasm surrounding scattered dense lymphoplasmacytic collections. Evidence of the reactive histiocytic nature of the eosinophilic cells was provided by an immunohistochemical reaction that was positive with KP-1 (CD68), but negative with common muscle actin, desmin, and myoglobin. The ultrastructural finding of elongated and rhomboid, membrane-bound, cytoplasmic crystals further supported the reactive histiocytic nature of these cells. B-cell kappa monoclonality of the lymphoplasmacytic component was proven by gene rearrangement studies. A recurrence involving the right parotid gland, 18 months after removal of the cervical mass, confirmed the aggressive nature of this B-cell lymphoma. Misdiagnosis of crystal-storing histiocytosis as adult rhabdomyoma can be avoided if the following features are recognized: lack of cytoplasmic cross striations, frequent multinucleation of the histiocytes, and prominence of the atypical lymphoplasmacytic component.

Antigens, CD↗
PubMedDate not supplied

Multiple cutaneous rhabdomyomas in a child.

We recently encountered a 4 year-old male patient with congenital, multiple, corporeal, cutaneous rhabdomyomas. Pathological examination of the excised tumors showed lobular structures in the dermis. The tumor cells contained spherical and polygonal eosinophilic cytoplasm with peripherally dislocated nuclei, and were positive for myogloblin staining. Extra-cardiac rhabdomyoma is an extremely rare condition, and our case might be considered the first of its kind in Japan.

Child, Preschool↗
PubMedDate not supplied

Splenic involvement in a stillborn fetus with tuberous sclerosis and multiple cardiac rhabdomyomas.

Multiple cardiac rhabdomyomas are frequently associated with tuberous sclerosis (TSC). However, splenic involvement in TSC is very rare. Histiocytoid cells in the spleen have been previously reported in only seven neonates and one fetus. We report an unusual case of multiple cardiac rhabdomyomas in a stillborn fetus with TSC who had clusters of histiocytoid cells in the spleen. These large cells had abundant eosinophilic cytoplasm and were positive with CD 68; ultrastructurally, they contained many membrane-bound bodies. It has been suggested that these cells are histiocytes. To the best of our knowledge, the present case is the second stillborn fetus who had splenic involvement with TSC.

Brain↗
PubMed1992

[Cardiac rhabdomyoma in tuberous sclerosis. A report of five cases and review of the literature].

Five children with tuberous sclerosis (TS), a polysystemic disease, had cardiac tumors detected by B-mode echocardiography. In a fetus, sonography performed at the eighth month showed the presence of a cardiac mass and of polycystic kidneys: the diagnosis of TS was confirmed postnatally because of the presence of calcified cerebral nodules. A newborn dichorial twin had paroxysmal tachycardia at ten days. B-mode echocardiography showed the presence of seven intracardiac tumors, and cerebral CT the presence of multifocal periventricular calcifications. An absence in a nine month old baby prompted an electrocardiogram that proved abnormal; a B-mode echocardiography showed large apical cardiac tumor; again cerebral CT showed periventricular calcified nodules. Two girls, 9 and 10 y.o. respectively, affected by TS, with normal electrocardiograms, both presented a small, asymptomatic, intracardiac mass demonstrated sonographically. In recent years, prenatal sonography and B-mode echocardiography in patients with TS demonstrated with increasing frequency the association of cardiac tumors (rhabdomyomas) with TS. Tumor detection often precedes the appearance of the cutaneous and neurological signs typical of the disease. This paper emphasizes the role of echocardiography in detecting cardiac tumors, as an early sign for the diagnosis of tuberous sclerosis, and to depict the natural history of cardiac rhabdomyoma with its variable clinical presentation and prognosis.

Child↗
PubMedDate not supplied

[Cardiac rhabdomyoma surgically treated with success. Review of literature].

The primary cardiac tumors are inusual, the incidence varies in all the ages between 0.005 to 0.05%. In pediatrics patients the incidence is 0.27%. The more frequent tumors during the childhood are the cardiac rhabdomyomas. These tumors are considered benigns. The clinical expression is wide, in the most the cases, the patients are asymptomatic and are detected by murmurs. In the prenatal age are manifested by arrhythmias or hydrops fetalis. The neonates and children may be show cardiac arrhythmias, low cardiac index and sudden cardiac death. The association with tuberous sclerosis had been reported in 81%. We present a neonate with cardiac rhabdomyoma diagnosed in the newborn period when he was asymptomatic, however in the follow-up he developed cardiac failure by obstruction in the out flow tract of the right ventricle. He underwent open cardiac surgery to resect the obstruction. Five months after surgery, the patient remain asymptomatic.

Heart Neoplasms↗
PubMed1991

Prenatal diagnosis of intracardiac rhabdomyoma: a case report.

We report on a case of intracardiac rhabdomyoma diagnosed prenatally from two-dimensional echocardiograms. The tumor was diagnosed by intrauterine echocardiography at the 38th week of gestation. Autopsy proved that the intracardiac tumor was a rhabdomyoma. Tuberous sclerosis spots in the brain tissue were also found.

Adult↗
PubMed1991

Rhabdomyomas of female genital tract. Report on two cases.

Studies were conducted by means of optical light and electron microscopy into two cases of rhabdomyoma of a fetal type in the vagina of a patient, 42 years of age, and in the ectocervical region of a patient aged 39 years. Both cases were similar to one another for optical and ultrastructural features and revealed high maturity of the rhabdomyomatous cells in tumors. A review of the literature would reveal the cases presented to be the 20th case of a vaginal tumor and the 3rd case of ectocervical tumor so far described as rhabdomyomas of the female genital tract.

Adult↗
PubMed1991

[Cardiac rhabdomyomas].

Case report of cardiac rhabdomyoma in one two days old infant with important cyanosis and respiratory insufficiency with evolution to death in few hours. The radiologic feature was a great enlargement of the heart and the electrocardiographic study showed left branch block. The two-dimensional echocardiography study showed multiple intracavitary tumours in both ventricles and in the right atrium. These features were confirmed in the post-mortem study. The microscopic study of the tumors showed proliferation of the spider cells, and vacuolation with the presence glycogen. The diagnosis of cardiac rhabdomyoma was confirmed. The brain and the kidney were not studied for the diagnosis of tuberous sclerosis or kidney tumors. It is very important the early diagnosis by echocardiography, due to the possibility of surgical resection of the intracavitary tumors that cause severe obstruction to the blood flow.

Echocardiography↗
PubMed1991

[Laryngeal rhabdomyoma].

Adults Rhabdomyoma are benign and rare tumors of skeletal muscle. The bibliography gathers about 60 cases, sitting on the head and the neck (95%), specially on the floor of the mouth, larynx or pharynx. Two exceptional case has been reported, one in the abdominal wall, the other on the retroperitoneal space, the latter being a mixed rhabdomyoma (either foetal and adult). Although the condition shows typical morphological features, the interest of the case reported, rely, apart of its scant presentation, on the possibility of leading to a mistaken clinic-macroscopic diagnosis of malignant mesenchymal tumor, because of its outward appearance.

Adult↗
PubMed1991

Cardiac rhabdomyoma: a clinicopathologic study.

We studied the clinical and pathologic features of 17 cardiac rhabdomyomas from 13 males and four females whose ages ranged from birth to 9 yr (mean, 36 wk). Eleven were multiple, and tumors were found throughout the heart. Four patients had congenital heart disease, and three had tuberous sclerosis; of the ten sporadic cases, four were surgical resections. Three of the four patients with surgical resections survived postoperatively. Two patients presented with sudden cardiac death. Immunohistochemical stains on seven tumor revealed diffuse positivity for myoglobin, actin, desmin, and vimentin, with negative results for S-100 protein, similar to adjacent cardiac muscle. We conclude that cardiac rhabdomyomas can be sporadic, can be associated with tuberous sclerosis, or can be seen with other cardiac malformations. They usually present early in life with a variety of symptoms, including sudden death, and attempts at resection may be successful.

Autopsy↗
PubMed1991

[The spontaneous regression of a rhabdomyoma of the right ventricle].

Rhabdomyoma is the most common intracardiac tumor in the newborn. The diagnosis is made by the clinical history and the physical examination, the electrocardiogram, chest X-ray, and the echocardiographic data. It has a poor prognosis, 80% dying in the first year of life because congestive heart failure or sudden death. Rarely, spontaneous regression has been described. We present the case of a newborn diagnosed of tuberous sclerosis, with an echocardiographic image of a rhabdomyoma in the right ventricle with a spontaneous total regression of the tumor 2 years later.

Echocardiography↗
PubMed1990

[Cardiac rhabdomyoma in children and Bourneville's tuberous sclerosis].

Cardiac rhabdomyomas are rare congenital tumours resulting from an early dysembryoplastic disorder of organogenesis. They are generally benign hamartomas which may be the first manifestation of a phakomatosis, tuberous sclerosis (TS) (Bourneville disease), present in over half of cases. The cases of 11 children with cardiac rhabdomyomas are reported. All of them also had extracardiac lesions of TS. Their ages ranged from 1 day to 6 years of age. In one case, the diagnosis was made antenatally by foetal echocardiography. The clinical expression may be very serious when there are hemodynamic disturbances resulting from an obstructive syndrome (N = 3) or arrhythmias (N = 2). Nevertheless, the cardiac evolution is usually favorable (N = 9) in contrast to the neuropsychiatric outcome which is generally catastrophic (N = 8). The diagnosis is made by two-dimensional echocardiography and magnetic resonance imaging. Surgical resection of the tumours is reserved for forms complicated by cardiac failure due to intracardiac obstruction (N = 2) of life-threatening resistant arrhythmias. Genetic counseling should be directed towards prevention of TS because it is transmitted in an autosomal dominant mode. However, most of the cases are sporadic. Echocardiography should be systematic in all children with TS and is also recommended for members of their families, even those apparently unaffected, in order not to miss paucisymptomatic forms of TS.

Adolescent↗
PubMed1990

[Adult type rhabdomyoma of the soft palate: pathology of 2 cases with review of the medical literature].

In this paper, the authors report two cases of adult type rhabdomyomas of the soft palate. This tumor is composed of striated muscle cells containing striations and intracytoplasmic rod-shaped inclusions. In specimens already fixed in aqueous fixators (formalin...), some tumor cells presented a fixation artefact and displayed the morphology of so-called "spiderweb cells". Adult type rhabdomyoma is a real tumor. Total surgical removal is sufficient to cure the patient of this tumor in most cases.

Adult↗
PubMedDate not supplied

Adult-type rhabdomyoma of the palate. Cytologic presentation of two cases with histologic and immunologic study.

The cytologic presentations of two adult-type rhabdomyomas of the soft palate are reported. Fine needle aspiration smears of the lesions contained globular tumor cells that had striations and intracytoplasmic rod-shaped inclusions. These inclusions, corresponding to hypertrophied Z bands, were pathognomonic of rhabdomyoma tissue. The cytologic diagnosis was confirmed by histologic and immunologic studies in both cases. The cytologic preparations fixed in alcohol or air dried did not show the "spiderweb cells" observed in the histologic sections of tissue fragments fixed in formaldehyde; this clearly illustrates that the morphology of the cells of this tumor depends primarily on the specimen preparation technique used. These cases suggest that aspiration cytology can establish the diagnosis of this benign tumor of striated muscle, for which total surgical removal is usually sufficient to effect a cure. The differential diagnosis is discussed in detail.

Adult↗
PubMed1989

[Spontaneous regression of a heart rhabdomyoma in tuberous sclerosis. Apropos of a case].

A case of cardiac rhabdomyoma diagnosed during the neonatal period is presented. The condition remitted spontaneously, as was observed by monitoring its course both clinically and by echocardiography. Surgical treatment of this rare condition should be assessed with care, both because of the risk involved in surgery and because of the possibility of a spontaneous cure in rhabdomyomas, which are usually grouped together, as in our case, in a general picture of tuberous sclerosis.

Echocardiography↗
PubMed1988

[Basal-cell nevomatosis associated with multifocal fetal rhabdomyoma. A case].

Nevoid basal-cell carcinoma is a hereditary syndrome. Its major features are a multiple basal-cell carcinoma which appears early in childhood, skeletal and genital abnormalities and ectopic calcifications. It may be associated with malignant schwannoma, medulloblastoma and lymphoma. We report one case of nevoid basal-cell carcinoma syndrome associated with foetal rhabdomyoma, thyroid gland polyadenoma and benign schwannoma. The first case of foetal rhabdomyoma associated with this syndrome was described in 1976.

Adult↗