Rhabdomyoma

Rhabdomyomas (Greek ῥαβδομύωμα, ravthomíomma, rhabdos of " bar " in terms of histological transverse striations of skeletal muscle and mys " muscle " and the ending ~ om for " tumor " ) are among the rarest tumors of the human body. Have their origin in these tumors of striated muscle ( skeletal or cardiac muscle).

Rhabdomyomas are very rare and occur mainly in the head and neck region - in children as so-called " fetal rhabdomyomas ," in adults as " adult rhabdomyoma ." The extracardiac forms which occur preferentially in the head and neck region, are much less frequent than cardiac rhabdomyomas.

Rhabdomyomas of the heart are frequently occurring in autosomal, dominantly inherited or incurred as a spontaneous mutation tuberous sclerosis. They occur from about the second trimester of pregnancy (from about the 20th week of pregnancy ) on, then often grow up to the birth and decline thereafter, especially during the first four years of life, then more slowly. Until the age of 20 they are as good as gone forever. According to previous experiences come new Rhabdomyomas latest after the infancy not to rebuild it. Rhabdomyomas of the heart caused about 80% as part of a tuberous sclerosis complex (TSC ), in about 20% of those affected is no TSC ago. Children with TSC have over 50% of rhabdomyomas.

Rhabdomyomas make predominantly no clinical complaints and have a good clinical prognosis. However, they can cause heart rhythm disturbances, but were observed in the TSC without detectable rhabdomyomas. Why Rhabdomyomas shrink after birth is not known. Deaths due to cardiac rhabdomyomas are extremely rare, affecting experience to date apparently only newborns. If rhabdomyomas of the heart do not make complaints in the neonatal or childhood, operational measures are almost never necessary. Moreover, the number and size of rhabdomyomas do not allow any conclusions about the severity of other organ manifestations in TSC.