Rhabdomyoma

Rhabdomyoma

Surgically excised cardiac rhabdomyoma (unfixed).
Classification and external resources
ICD-10 D21 (ILDS D21.M50)
ICD-9 215
ICD-O 8900/0
eMedicine med/2021
MeSH D012207

A rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas may be either "cardiac" or "extracardiac" (occurring outside of the heart). Extracardiac forms of rhabdomyoma are subclassified into three distinct types: (1) Adult type, (2) Fetal type, and (3) Genital type.

Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. It has an association with tuberous sclerosis.[1] In those with tuberous sclerosis, the tumor may regress and disappear completely, or remain consistent in size.

It is most commonly associated with the tongue[2] and heart,[3] but can also occur in other locations, such as the vagina.[4]

Malignant skeletal muscle tumors are referred to as rhabdomyosarcoma. Only rare cases of possible malignant change have been reported in fetal rhabdomyoma.

Photomicrograph of fetal-type rhabdomyoma. Note the plump, pink benign skeletal muscle cells.

References

  1. Bader RS, Chitayat D, Kelly E et al. (November 2003). "Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex". J. Pediatr. 143 (5): 620–4. doi:10.1067/S0022-3476(03)00494-3. PMID 14615733.
  2. Pérez-Alonso P, Sánchez-Simón R, Contreras F, Patrón-Romero M (December 2000). "Special feature: pathological case of the month. Denouement and discussion: fetal rhabdomyoma of the tongue (myxoid type)". Arch Pediatr Adolesc Med 154 (12): 1265–6. doi:10.1001/archpedi.154.12.1265. PMID 11115314.
  3. Sugiyama H, Naito H, Tsukano S, Echigo S, Kamiya T (November 2005). "Evaluation of cardiac tumors in children by electron-beam computed tomography: rhabdomyoma and fibroma". Circ. J. 69 (11): 1352–6. doi:10.1253/circj.69.1352. PMID 16247210.
  4. Edward F. Goljan (2009). Pathology, Third Edition. Elsevier. p. 537. Tumour Type: Rhabdomyoma; Location: Heart, also tongue and vagina; Comment: Benign heart tumour associated with tuberous sclerosis

External links