Rhabdomyoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]
Overview
Rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas are rare and can be classified into cardiac type and extracardiac type.The most common primary benign pediatric tumor of the heart is cardiac rhabdomyoma which can be seen mainly in fetal life and children, second most common primary benign cardiac tumor in children is fibroma. Most tumors regress spontaneously, prognosis depends on the location of tumor and size. Cardiac rhabdomyoma is strongly associated with tuberous sclerosis. Cardiac rhabdomyoma is seen almost always in the pediatric age group and is associated with tuberous sclerosis, neurofibromatosis, and sebaceous adenomas. Extracardiac rhabdomyoma can be divided into three groups (adult, fetal, and genital types) with distinct clinical and morphological differences. The adult type is a slowly growing mass which typically involves the head and neck. Fetal type is seen in the head and neck region. The genital type is almost always found in the vulvovaginal region of older women. Treatment of adult type rhabdomyoma of head and neck depends upon the severity of clinical symptoms. Conservative management or surgery may be necessary depending upon the type and tumor stage.
Natural History, complications and prognosis
- 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.
- Common complications of cardiac rhabdomyoma include development of cardiac arrhythmias, ventricular outflow tract obstruction, and congestive heart failure.[4]
- 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.
Refrences
- ↑ 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)