Congenital Rhabdomyoma Research Articles

Excision of Congenital Rhabdomyoma of the Left Outflow Tract through the Left Atrium and Mitral Valve

Primary rhabdomyomas obstructing the right or left outflow tract are uncommon findings in the perinatal period. The presenting symptom may be arrhythmia, cardiac murmur, complete or variable atrioventricular block, pericardial effusion, cardiomegaly, cardiac failure, or sudden death. The variety of symptoms can be explained on the basis of obstruction of blood flow, myocardial involvement, and disturbance of the cardiac rhythm. Commonly, rhabdomyoma spontaneously regresses in the majority of cases, and neonatal surgery is advocated only in case of severe left ventricular outflow tract (LVOT) obstruction or the development of arrhythmias. Herein, we describe the pathologic and clinical characteristics of neonatal presentation of a cardiac rhabdomyoma with a nearly obstructive mass in the LVOT, discovered during pregnancy and operated 4 months after delivery.

Spontaneous regression of a large rhabdomyoma of the interventricular septum

We report the case of a large congenital rhabdomyoma of the interventricular septum diagnosed prenatally. The foetus was strictly monitored with ultrasound throughout the gestation period showing that the mass had increased in size until delivery. Despite the size of the mass, which appeared to occupy the right ventricle, the baby presented no symptoms both in utero and after birth. Serial echocardiography was used to document the regression of the mass in childhood.

Congenital rhabdomyoma of the heart : K. D. Bhoola. S Afr Med J 49:936–938 (May), 1975

Congenital rhabdomyoma of the heart : K. D. Bhoola. S Afr Med J 49:936–938 (May), 1975

Congenital rhabdomyoma of the heart: Presentation of two cases

Congenital rhabdomyoma of the heart: Presentation of two cases

The monster Purkinje-cell nature of so-called “congenital rhabdomyoma of heart” A forme fruste of tuberous sclerosis

So-called “congenital rhabdomyoma of heart” is not a neoplasm. Microscopic observations show that it is an abnormal differentiation of embryonal myocardium at random, and prematurely, into a monster type of Purkinje cell. Close mimicry of all types of human Purkinje cells is illustrated. The evolution of such cardiac nodules and cell gigantisms corresponds with the other disturbances in organogenesis in tuberous sclerosis, of which so-called cardiac rhabdomyoma is but a spectacular facet. It would appear that tuberous sclerosis, a heredofamilial condition, is generalized and one in which chromosomal abnormalities may be expected.

Rhabdomyoma of the heart in both of a set of twins.

SUMMARYA case of congenital multiple rhabdomyoma of the heart in both of a set of twins of like sex is presented. A separated diamnionic dichorionic placenta was found. However because both twins had the same sex, the same color of eyes, the same blood group and very similar plantar prints pattern they were considered MZ. Twin A died after 10 days becouse of intractable heart failure. Twin B died one hour after birth of hydrops fetalis. The autopsies showed the presence of multiple rabdomyoma in the walls of both hearts and in Twin B a widely patent ductus arteriosus. No other extracardiac anomalies were noted. The diagnosis of rhabdomyoma of the heart, as is usual, was not made. It should be suspected in any infant who shows evidence of cardiac disease. On the other hand, this first case, known so far in the world literature of congenital rhabdomyoma of the heart in both twins could be an important contribution to the knowledge of the nature of this so discussed lesion.

Congenital rhabdomyoma of the heart; case report with histochemical study of tumor polysaccharide.

CancerVolume 8, Issue 5 p. 916-920 ArticleFree Access Congenital rhabdomyoma of the heart. Case report with histochemical study of tumor polysaccharide Joseph Beaird B.S., Joseph Beaird B.S. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this authorRobert W. Mowry M.D., Robert W. Mowry M.D. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this authorJoseph A. Cunningham M.D., Joseph A. Cunningham M.D. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this author Joseph Beaird B.S., Joseph Beaird B.S. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this authorRobert W. Mowry M.D., Robert W. Mowry M.D. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this authorJoseph A. Cunningham M.D., Joseph A. Cunningham M.D. Departments of Pathology, University of Alabama Medical Center and St. Vincent's Hospital, Birmingham, AlabamaSearch for more papers by this author First published: 1955 https://doi.org/10.1002/1097-0142(1955)8:5<916::AID-CNCR2820080511>3.0.CO;2-6Citations: 16AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL References 1 Batchelor, T. M., and Maun, M. E.: Congenital glycogenic tumors of the heart. Arch. Path. 39: 67– 73, 1945. 2 Crowell, W. M., and Rice, E. C.: Congenital rhabdomyoma of the heart. Clin. Proc. Child. Hasp. 6: 292– 296, 1950. 3 Farber, S.: Congenital rhabdomyoma of the heart. Am. J. Path. 7: 105– 130, Pl. 23–26, 1931. 4 Hueper, W. C.: Rhabdomyomatosis of the heart in a guinea pig. Am. J. Path. 17: 121– 124, Pl 26, 1941. 5 Kidder, L. A.: Congenital glycogenic tumors of the heart. Arch. Path. 49: 55– 62, 1950. 6 Labate, J. S.: Congenital rhabdomyoma of the heart; report of a case. Am. J. Path. 15: 137– 150, Pl 28, 1939. 7 Mowry, R. W.: Histochemical demonstration and significance of dextran sulfate: a metachromatic, water-soluble acid polysaccharide. [Abstr.] J Histochem. & Cytochem. 2: 470– 471, 1954. 8 Mowry, R. W., and Bangle, R., JR.: Histochemically demonstrable glycogen in the human heart; with special reference to glycogen storage disease and diabetes mellitus. Am. J. Path. 27: 611– 625, 1951. 9 Mowry, R. W., and Millican, R. C.: A histochemical study of the distribution and fate of dextran in tissues in the mouse. Am. J. Path. 29: 523– 545, 1953. 10 Prichard, R. W.: Tumors of the heart; review of the subject and report of one hundred and fifty cases. A. M. A. Arch. Path. 51: 98– 128, 1951. 11 Recklinghausen Von: Ein Herz von einem Keugeborenen, welches mehrere theils nach aussen, theils nach den Höhlen prominirende Tumoren (Myomen) trug. Monatschr. J. Geburtsk. u. Frauenkr. 20: 1, 1862. 12 Vallance-Owen, J.: The histological demonstration of glycogen in necropsy material. J Path. & Bact. 60: 325– 327. 1948. Citing Literature Volume8, Issue51955Pages 916-920 ReferencesRelatedInformation

GLYCOGEN STORAGE DISEASE OF THE HEART

Glycogen storage disease of the heart is a separate disease entity with distinctive manifestations. Clinical, chemical and pathologic criteria for diagnosis are listed. In a survey of the literature 14 cases were found that fulfilled all requirements. The impression given by the available clinical data is that of an illness remarkably constant in its course and manifestations. Eleven of the 14 patients died between the ages of 4 and 8 months, and the other three at an earlier age. The electrocardiographic findings in three cases are discussed. The pathologic changes were similar in all cases. The outstanding lesions were in the myocardium, but abnormal deposition of glycogen was not limited to this site, being found in many other organs and tissues. In 6 out of 8 cases in which histologic and histochemical studies of skeletal muscles were made, a distinctive type of degeneration was found together with an abnormally great accumulation of glycogen. Analyses of blood and urine performed while the patients were still living were essentially normal. Postmortem chemical studies in six cases confirmed the morphologic demonstration of widespread distribution of glycogen throughout the body. Spontaneous glycogenolysis was shown to occur in the myocardium after incubation at 37° C. for 24 to 48 hours, although to a different degree in the three cases in which it was done. In two of these there was virtually complete disappearance of glycogen from the liver after incubation at 37° C. for 24 to 48 hours. The cause of this rare disease is presumably an inborn error of metabolism transmitted as a recessive genetic character. Whatever the mechanism the condition is incompatible with life for more than a few months, during which cardiac function continually regresses. The most plausible explanation is that the accumulation of glycogen in the myocardial fibers progresses to a point where their contraction is impaired and effective cardiac action is no longer possible. It is pointed out that the term "glycogen storage disease of the heart" is a misnomer, since the disease process is not limited to this organ. On the other hand, as tradition has sanctioned its use, the name may well be retained. A plea is entered for dropping the name "von Gierke's disease of the heart" as leading to confusion with other types of glycogen storage disease. The clinical and metabolic differences between the hepatic and cardiac types of glycogen storage disease are detailed. Other conditions characterized by abnormally great accumulation of glycogen in the myocardium, such as congenital rhabdomyoma of the heart, socalled cardiomegalia glycogenica circumscripta, and occasional cases in which death is sudden though not always explained, are described and the differences with glycogen storage disease of the heart mentioned. Differential diagnosis is discussed and it is pointed out there are no pathognomonic signs or laboratory investigations, except for the histochemical demonstration of abnormal amounts of glycogen in the skeletal muscles. It is suggested that biopsy of skeletal muscle be performed in all cases in which unexplained enlargement of the heart in infancy occurs. This is essential in order to plan ante- and postmortem pathologic and chemical studies.

Congenital rhabdomyoma of the heart associated with arrhythmia

Summary A case of rhabdomyoma of the heart in a ten-month-old girl is reported. The most important clinical finding was a cardiac arrhythmia thought to be auricular flutter. At necropsy the tumor was found to invade the conduction system. Congenital cysts of the kidneys were also present.

Congenital Rhabdomyoma of the Heart

Congenital Rhabdomyoma of the Heart