Rhabdomyoma
| Rhabdomyoma | |
|---|---|
 | |
| Surgically excised cardiac rhabdomyoma (unfixed) | |
| Specialty | Oncology |
Arhabdomyomais abenigntumorofstriated muscle.Rhabdomyomas may be either cardiac or extracardiac (occurring outside the heart). Extracardiac forms of rhabdomyoma are sub-classified into three distinct types: adult type, fetal type, and genital type.
Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. It has an association withtuberous sclerosis.[1]In those with tuberous sclerosis, the tumor may regress and disappear completely, or remain consistent in size.[2]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 thetongue,[3]andheart,[4]but can also occur in other locations, such as thevagina.[5]
Malignantskeletal muscle tumors are referred to asrhabdomyosarcoma.Only rare cases of possible malignant change have been reported infetal rhabdomyoma.The differential diagnosis in the tongue includesectomesenchymal chondromyxoid tumor.[6]
Cardiac Rhabdomyoma
[edit]It is much more common to find metastasis from an alternate site than a primary heart tumor.[7]However, primary cardiac tumors do occur. One of these is the cardiac rhabdomyoma. Approximately 80-90% of these tumors are found in patients withtuberous sclerosis,a genetic condition causing multiple tumors, with most found prior to the age of one.[8]Although these tumors are most commonly treated with resection, symptomatic tumors in fetuses have been shown to decrease in size after maternalsicrolimusadministration.[9]If clinically silent, they can be watched with routine imaging as the tumor will likely spontaneously regress.[2]
Clinical Picture
[edit]The specific clinical picture of a patient with a cardiac rhabdomyoma is determined by its location in the heart.[10]However, if symptomatic, most patients will present withheart failureor anarrhythmiasuch asventricular tachycardiaorheart block.[11]The severity of the symptoms will vary based on the size of the mass.
Detection
[edit]
Cardiac rhabdomyomas are usually found in theventricular wallsor on thevalves of the heart.With imaging techniques such asultrasoundandmagnetic resonance imaging (MRI),these tumors are being detected with increased frequency and even in the prenatal period. Routine surveillance of children with tuberous sclerosis for cardiac rhabdomyoma or other cardiovascular manifestations of their disease may includeelectrocardiogram (EKG)andechocardiography.[12]
Additional images
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References
[edit]- ^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.PMID14615733.
- ^abSmythe, John F.; Dyck, John D.; Smallhorn, Jeffrey F.; Freedom, Robert M. (November 1990)."Natural history of cardiac rhabdomyoma in infancy and childhood".The American Journal of Cardiology.66(17): 1247–1249.doi:10.1016/0002-9149(90)91109-j.ISSN0002-9149.PMID2239731.
- ^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.PMID11115314.
- ^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.PMID16247210.
- ^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
- ^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.PMID7726361.S2CID21161742.
- ^Salcedo, E (February 1992)."Cardiac tumors: Diagnosis and management".Current Problems in Cardiology.17(2): 77–137.doi:10.1016/0146-2806(92)90025-J.PMID1735241.
- ^MDa, Maurice Beghetti; Gow, Robert M.; Haney, Isabel; Mawson, John; Williams, William G.; Freedom, Robert M. (December 1997)."Pediatric primary benign cardiac tumors: A 15-year review".American Heart Journal.134(6): 1107–1114.doi:10.1016/S0002-8703(97)70032-2.PMID9424072.
- ^Duan, Menghao; Sundararaghavan, Sreekanthan; Koh, Ai Ling; Soh, Shui Yen (March 2022)."Neonatal rhabdomyoma with cardiac dysfunction: favourable response to sirolimus".BMJ Case Reports.15(3): e244915.doi:10.1136/bcr-2021-244915.ISSN1757-790X.PMC8948400.PMID35332001.
- ^Vander Salm, Thomas J. (April 2000)."Unusual Primary Tumors Of The Heart".Seminars in Thoracic and Cardiovascular Surgery.12(2): 89–100.doi:10.1053/ct.2000.5080.PMID10807431.
- ^Jacobs, Jeffrey P.; Konstantakos, Anastasios K.; Holland, Fred W.; Herskowitz, Kenneth; Ferrer, Peter L.; Perryman, Richard A. (November 1994)."Surgical treatment for cardiac rhabdomyomas in children".The Annals of Thoracic Surgery.58(5): 1552–1555.doi:10.1016/0003-4975(94)91963-1.PMID7979700.
- ^Northrup, Hope; Aronow, Mary E.; Bebin, E. Martina; Bissler, John; Darling, Thomas N.; de Vries, Petrus J.; Frost, Michael D.; Fuchs, Zoë; Gosnell, Elizabeth S.; Gupta, Nishant; Jansen, Anna C.; Jóźwiak, Sergiusz; Kingswood, J. Chris; Knilans, Timothy K.; McCormack, Francis X. (October 2021)."Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations".Pediatric Neurology.123:50–66.doi:10.1016/j.pediatrneurol.2021.07.011.PMID34399110.