Rhabdomyoma natural history, complications and prognosis: Difference between revisions
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{{Rhabdomyoma}} | {{Rhabdomyoma}} | ||
===Natural History=== | |||
*The majority of cases regress spontaneously(partial or complete),however there are reported cases which [[surgical]] interventions were necessary,especially if tumor causes [[cardiac]] symptoms such as [[arrhythmia]] or [[obstruction]]. <ref name="pmid29742370">{{cite journal |vauthors=Barnes BT, Procaccini D, Crino J, Blakemore K, Sekar P, Sagaser KG, Jelin AC, Gaur L |title=Maternal Sirolimus Therapy for Fetal Cardiac Rhabdomyomas |journal=N. Engl. J. Med. |volume=378 |issue=19 |pages=1844–1845 |date=May 2018 |pmid=29742370 |pmc=6201692 |doi=10.1056/NEJMc1800352 |url=}}</ref><ref name="pmid10865004">{{cite journal| author=Becker AE| title=Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. | journal=Pediatr Cardiol | year= 2000 | volume= 21 | issue= 4 | pages= 317-23 | pmid=10865004 | doi=10.1007/s002460010071 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10865004 }} </ref><ref name="pmid2239731">{{cite journal| author=Smythe JF, Dyck JD, Smallhorn JF, Freedom RM| title=Natural history of cardiac rhabdomyoma in infancy and childhood. | journal=Am J Cardiol | year= 1990 | volume= 66 | issue= 17 | pages= 1247-9 | pmid=2239731 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2239731 }} </ref> | |||
**Early clinical features include [[heart failure]], [[cardiac murmur]], and [[arrhythmia]]. | |||
**Tumors larger in diameter are more likely to cause [[arrhythmia]]s or [[hemodynamic]] instability, which are associated with an increased risk of death. | |||
**The majority of patients with [[cardiac]] rhabdomyoma remain asymptomatic; however, some affected patients become symptomatic in the [[perinatal period]]. | |||
== | ===Complications=== | ||
{{ | *Common complications of [[cardiac]] rhabdomyoma include development of [[cardiac arrhythmias]], [[ventricular outflow tract]] obstruction, and [[congestive heart failure]].<ref name="pmid25424575">{{cite journal |vauthors=Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK |title=Cardiovascular manifestations of tuberous sclerosis complex and summary of the revised diagnostic criteria and surveillance and management recommendations from the International Tuberous Sclerosis Consensus Group |journal=J Am Heart Assoc |volume=3 |issue=6 |pages=e001493 |date=November 2014 |pmid=25424575 |pmc=4338742 |doi=10.1161/JAHA.114.001493 |url=}}</ref> | ||
{{ | ===Prognosis=== | ||
{{ | *prognosis is generally good; the [[survival rate]] of patients with benign rhabdomyoma is excellent, depending on location of tumor, prognosis may change.<ref name="pmid25870340">{{cite journal |vauthors=Linnemeier L, Benneyworth BD, Turrentine M, Rodefeld M, Brown J |title=Pediatric cardiac tumors: a 45-year, single-institution review |journal=World J Pediatr Congenit Heart Surg |volume=6 |issue=2 |pages=215–9 |date=April 2015 |pmid=25870340 |doi=10.1177/2150135114563938 |url=}}</ref> | ||
*Rhabdomyomas on [[mitral]] or [[Tricuspid valve|tricuspid]] valves can lead to regurgitation or obstruction of outflow tract thus poor prognosis.<ref name="pmid26891966">{{cite journal |vauthors=Wang Y, Wang X, Xiao Y |title=Surgical treatment of primary cardiac valve tumor: early and late results in eight patients |journal=J Cardiothorac Surg |volume=11 |issue= |pages=31 |date=February 2016 |pmid=26891966 |pmc=4759914 |doi=10.1186/s13019-016-0406-2 |url=}}</ref> | |||
[[ | *The long-term prognosis of [[cardiac]] rhabdomyoma is affected by the [[neurologic]] manifestations associated with [[tuberous sclerosis]].<ref name="pmid28811058">{{cite journal |vauthors=Chung CWT, Lawson JA, Sarkozy V, Riney K, Wargon O, Shand AW, Cooper S, King H, Kennedy SE, Mowat D |title=Early Detection of Tuberous Sclerosis Complex: An Opportunity for Improved Neurodevelopmental Outcome |journal=Pediatr. Neurol. |volume=76 |issue= |pages=20–26 |date=November 2017 |pmid=28811058 |doi=10.1016/j.pediatrneurol.2017.05.014 |url=}}</ref> | ||
[[ | *The prognosis of patients with rhabdomyomas depends mainly on the size, location, number of the lesions, associated anomalies such as [[tuberous sclerosis]]. | ||
*[[Metastases]] have not been associated with rhabdomyoma. | |||
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Natural History
- The majority of cases regress spontaneously(partial or complete),however there are reported cases which surgical interventions were necessary,especially if tumor causes cardiac symptoms such as arrhythmia or obstruction. [1][2][3]
- Early clinical features include heart failure, cardiac murmur, and arrhythmia.
- Tumors larger in diameter are more likely to cause arrhythmias or hemodynamic instability, which are associated with an increased risk of death.
- The majority of patients with cardiac rhabdomyoma remain asymptomatic; however, some affected patients become symptomatic in the perinatal period.
Complications
- Common complications of cardiac rhabdomyoma include development of cardiac arrhythmias, ventricular outflow tract obstruction, and congestive heart failure.[4]
Prognosis
- prognosis is generally good; the survival rate of patients with benign rhabdomyoma is excellent, depending on location of tumor, prognosis may change.[5]
- Rhabdomyomas on mitral or tricuspid valves can lead to regurgitation or obstruction of outflow tract thus poor prognosis.[6]
- The long-term prognosis of cardiac rhabdomyoma is affected by the neurologic manifestations associated with tuberous sclerosis.[7]
- The prognosis of patients with rhabdomyomas depends mainly on the size, location, number of the lesions, associated anomalies such as tuberous sclerosis.
- Metastases have not been associated with rhabdomyoma.
- ↑ Barnes BT, Procaccini D, Crino J, Blakemore K, Sekar P, Sagaser KG, Jelin AC, Gaur L (May 2018). "Maternal Sirolimus Therapy for Fetal Cardiac Rhabdomyomas". N. Engl. J. Med. 378 (19): 1844–1845. doi:10.1056/NEJMc1800352. PMC 6201692. PMID 29742370.
- ↑ Becker AE (2000). "Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians". Pediatr Cardiol. 21 (4): 317–23. doi:10.1007/s002460010071. PMID 10865004.
- ↑ Smythe JF, Dyck JD, Smallhorn JF, Freedom RM (1990). "Natural history of cardiac rhabdomyoma in infancy and childhood". Am J Cardiol. 66 (17): 1247–9. PMID 2239731.
- ↑ Hinton RB, Prakash A, Romp RL, Krueger DA, Knilans TK (November 2014). "Cardiovascular manifestations of tuberous sclerosis complex and summary of the revised diagnostic criteria and surveillance and management recommendations from the International Tuberous Sclerosis Consensus Group". J Am Heart Assoc. 3 (6): e001493. doi:10.1161/JAHA.114.001493. PMC 4338742. PMID 25424575.
- ↑ Linnemeier L, Benneyworth BD, Turrentine M, Rodefeld M, Brown J (April 2015). "Pediatric cardiac tumors: a 45-year, single-institution review". World J Pediatr Congenit Heart Surg. 6 (2): 215–9. doi:10.1177/2150135114563938. PMID 25870340.
- ↑ Wang Y, Wang X, Xiao Y (February 2016). "Surgical treatment of primary cardiac valve tumor: early and late results in eight patients". J Cardiothorac Surg. 11: 31. doi:10.1186/s13019-016-0406-2. PMC 4759914. PMID 26891966.
- ↑ Chung C, Lawson JA, Sarkozy V, Riney K, Wargon O, Shand AW, Cooper S, King H, Kennedy SE, Mowat D (November 2017). "Early Detection of Tuberous Sclerosis Complex: An Opportunity for Improved Neurodevelopmental Outcome". Pediatr. Neurol. 76: 20–26. doi:10.1016/j.pediatrneurol.2017.05.014. PMID 28811058. Vancouver style error: initials (help)