1/60. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/60. adult rhabdomyoma in the masticatory area. New case presentation and review of the literature.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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/60. Cardiac rhabdomyoma in intrauterine life: clinical features and natural history. A case series and review of published reports.BACKGROUND: Fetal cardiac rhabdomyoma is very rare; despite the fact that many cases and series have been reported, the clinical presentation, the natural history and the frequency with which this pathology is associated with tuberous sclerosis complex are not well determined. The aim of this investigation was to study the clinical features and the natural history of cardiac rhabdomyoma when diagnosed during prenatal life. methods: Nine cases of cardiac rhabdomyoma detected among 5276 fetal echocardiograms recorded over a 10-year period in a single center were retrospectively reviewed. medical records and echocardiograms were studied to determine the prenatal and postnatal course and outcome. RESULTS: The incidence of cardiac rhabdomyoma in our center was 0.17%. The gestational age at diagnosis ranged from 27 to 36 weeks. The most common reason for fetal echocardiography was an abnormal obstetric ultrasound scan (6/9 cases). In no case was there a family history of tuberous sclerosis. In one case, the tumor was single whereas in 8 cases multiple tumors were diagnosed. During prenatal life the majority of tumors were clinically silent. One fetus died of hydrops and arrhythmia. Four children presented with arrhythmia postnatally and one required surgery. At a mean follow-up of 47 months, total or partial regression was observed in 7 patients. Seven patients developed postnatal clinical signs of tuberous sclerosis. CONCLUSIONS: Fetal cardiac rhabdomyomas are often benign and have a tendency to regress, but their prognosis is guarded due to very frequent association with arrhythmias and tuberous sclerosis. During prenatal counseling, it is of utmost importance to inform the future parents of the virtually constant perspective of tuberous sclerosis complex.- - - - - - - - - - ranking = 0.10851298647918keywords = complex (Clic here for more details about this article) |
4/60. rhabdomyoma of the orbit.To our knowledge, this is the first fully documented case of an adult-type orbital rhabdomyoma in an 8-year-old white boy, which originated in the belly of the medial rectus muscle and grew into the anterior orbit to incorporate the trochlea. Incomplete excision resulted in recurrence; the lesion was misinterpreted as a rhabdomyosarcoma and exenteration was performed. The patient was free of disease 25 years later. Reexamination of original pathologic material showed the typical histology of an adult-type rhabdomyoma. adult-type rhabdomyoma, the most differentiated tumor in a spectrum of benign and malignant striated muscle neoplasms, should be distinguished from fetal rhabdomyoma (also benign) and pleomorphic rhabdomyosarcoma, the malignant striated muscle tumor most likely to be confused with rhabdomyoma. An awareness of the benign nature of rhabdomyoma (rhabdomyoma never evolves into a sarcoma) should prevent unnecessary radical surgery. The tumor can be adequately managed by a local excision that includes all the lobulations of tumor within a margin of normal tissue.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/60. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/60. tuberous sclerosis in a 19-week fetus: immunohistochemical and molecular study of hamartin and tuberin.tuberous sclerosis complex (TSC) is a genetically heterogeneous disease caused by mutations of TSC1 or TSC2 genes. It involves multiple organ systems resulting in mild to lethal hamartoma formation due to gene mutation in the germ line and loss of heterozygosity (LOH) in somatic cells. Hamartin (TSC1) and tuberin (TSC2) are expressed broadly. However, little is known about tissue susceptibility to hamartomas when equal or similar amounts of TSC gene expression are present. In this study, we present a 19-week gestational age fetus with pathological features of TSC, which was confirmed by finding LOH of TSC2 in a cardiac rhabdomyoma. Developmental expression of hamartin and tuberin in the TSC fetus, an age-matched non-TSC fetus, and a 26-week gestational age non-TSC fetus were analyzed by immunohistochemistry. We found that in addition to the differential expression of the TSC genes in some normal tissues compared with that in the TSC-affected fetus, the cellular localization and distribution of hamartin and tuberin were dramatically different in different tissues. In general, hamartin and tuberin are mainly expressed in epithelial cells, myocytes, and neural tissues. By comparing the incidence of the hamartomas in early childhood and gene expression in tissues, it appears that tissues with co-expression of hamartin and tuberin are prone to a higher incidence of hamartomas than those expressing only one protein, or two proteins but in different patterns of cellular localization.- - - - - - - - - - ranking = 0.054256493239589keywords = complex (Clic here for more details about this article) |
7/60. adult cellular rhabdomyoma of the heart: a report of 3 cases.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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/60. Mediastinal rhabdomyoma: a case report and review of the literature.An incidental anterior-superior mediastinal rhabdomyoma is reported in a 68-year-old man who died of hypovolemic shock as a result of massive blood loss due to transection of aorta after being hit by a moving motor vehicle. This is the third reported case of mediastinal rhabdomyoma in the literature. The immunohistochemical evidence of expression of muscle-specific markers supports the rhabdomyomatous nature of this neoplasm, and electron microscopic demonstration of haphazardly arranged myofilaments with prominent Z bands, "jack-straws" in the mitochondria, and the absence of desmosomes is supportive of extracardiac origin of this rhabdomyoma. The possible histogenesis of extracardiac adult rhabdomyoma (EAR) in the anterior-superior mediastinum from the thymic myoid cells is also discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/60. Foetal rhabdomyoma. Case report of a patient with two tumours.A case report of a girl with two foetal rhabdomyomas is presented. One tumour was a small cutaneous lesion present at birth in the left thigh and the other was located in the chest wall deeply in the subcutaneous tissue and attached to the adjacent intercostal muscle. The patient also had the naevoid basal cell carcinoma syndrome which lends credence to the opinion that foetal rhabdomyoma is a malformation rather than a true neoplasm.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/60. Paroxysmal supraventricular tachycardia in neonatal tuberous sclerosis complex and cardiac rhabdomyoma: report of one case.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.- - - - - - - - - - ranking = 0.37979545267712keywords = complex (Clic here for more details about this article) |
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