Rhabdomyoma

A rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas may be either "cardiac" or "extra cardiac" (occurring outside the heart). Extracardiac forms of rhabdomyoma are sub classified into three distinct types: adult type, fetal type, and genital type.

Rhabdomyoma
Surgically excised cardiac rhabdomyoma (unfixed)
SpecialtyOncology 

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. A common histological feature is the presence of Spider Cells, which are cardiac myocytes with enlarged glycogen vacuoles separated by eosinophilic strands, resembling the legs of a spider.

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. The differential diagnosis in the tongue includes ectomesenchymal chondromyxoid tumor.[5]

Additional images
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 ed.). Elsevier. p. 537. Tumour Type: Rhabdomyoma; Location: Heart, also tongue and vagina; Comment: Benign heart tumour associated with tuberous sclerosis
  5. Smith BC, Ellis GL, Meis-Kindblom JM, Williams SB (May 1995). "Ectomesenchymal chondromyxoid tumor of the anterior tongue. Nineteen cases of a new clinicopathologic entity". Am J Surg Pathol. 19 (5): 519–30. doi:10.1097/00000478-199505000-00003. PMID 7726361. S2CID 21161742.


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