Rhabdomyom
| Classification according to ICD-10 | |
|---|---|
| D21 | Other benign neoplasms of connective and other soft tissues |
| D21.9 | Connective tissue and other soft tissue, unspecified |
| ICD-10 online (WHO version 2019) | |
Rhabdomyome ( Greek ῥαβδομύωμα , ravthomíomma , from rhabdos "stick" in the sense of the fine-tissue transverse stripes of the skeletal muscles and mys "muscle" and the ending ~ om for "tumor") are among the rarest tumors in the human body. These tumors have their origin in the striated muscles (skeletal or heart muscle ).
Rhabdomyomas are very rare and occur mainly in the head and neck area - in children as so-called "fetal rhabdomyomas", in adults as "adult rhabdomyomas". The extracardiac forms, which occur preferentially in the head and neck area, are much rarer than cardiac rhabdomyomas.
Rhabdomyomas of the heart frequently come in the autosomal , dominantly inherited or as a spontaneous mutation resulting tuberous sclerosis before. They occur from around the second trimester of pregnancy (around the 20th week of pregnancy), then often grow until birth and then shrink more slowly, especially in the first four years of life. By the age of 20 they almost always disappeared. According to previous experience, new rhabdomyomas do not reappear after infancy at the latest. About 80% of rhabdomyomas of the heart develop in the context of tuberous sclerosis (TSC), and about 20% of those affected do not have TSC. Over 50% of children with TSC have rhabdomyomas.
For the most part, rhabdomyomas cause no clinical symptoms and have a good clinical prognosis. However, they can cause cardiac arrhythmias, which were observed in TSC even without any detectable rhabdomyomas. Why rhabdomyomas shrink after childbirth is not known. Deaths from cardiac rhabdomyomas are extremely rare and, according to previous experience, appear to affect only newborns. If rhabdomyomas of the heart do not cause symptoms in newborns or children, surgical measures are almost never necessary. Furthermore, the number and size of rhabdomyomas do not allow conclusions to be drawn about the severity of other organ manifestations in TSC.
