Rhabdomyoma medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Rhabdomyoma}} | {{Rhabdomyoma}} | ||
{{CMG}}; {{AE}} {{Nnasiri}} | |||
==Overview== | |||
Rhabdomyoma is a [[benign]] [[tumor]] of [[Muscle|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]]. | Rhabdomyoma is a [[benign]] [[tumor]] of [[Muscle|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]]. | ||
== Treatment == | |||
=== Medical Therapy === | |||
*Treatment for rhabdomyoma is supportive care.<ref name="pmid21464184">{{cite journal| author=Tiberio D, Franz DN, Phillips JR| title=Regression of a cardiac rhabdomyoma in a patient receiving everolimus. | journal=Pediatrics | year= 2011 | volume= 127 | issue= 5 | pages= e1335-7 | pmid=21464184 | doi=10.1542/peds.2010-2910 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21464184 }} </ref><ref name="pmid26199144">{{cite journal| author=Wagner R, Riede FT, Seki H, Hornemann F, Syrbe S, Daehnert I et al.| title=Oral Everolimus for Treatment of a Giant Left Ventricular Rhabdomyoma in a Neonate-Rapid Tumor Regression Documented by Real Time 3D Echocardiography. | journal=Echocardiography | year= 2015 | volume= 32 | issue= 12 | pages= 1876-9 | pmid=26199144 | doi=10.1111/echo.13015 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26199144 }} </ref> <ref name="pmid26339757">{{cite journal |vauthors=Öztunç F, Atik SU, Güneş AO |title=Everolimus treatment of a newborn with rhabdomyoma causing severe arrhythmia |journal=Cardiol Young |volume=25 |issue=7 |pages=1411–4 |date=October 2015 |pmid=26339757 |doi=10.1017/S1047951114002261 |url=}}</ref> | |||
*patients with rhabdomyoma should be monitored with [[echocardiography]] and [[electrocardiography]]. | |||
====Adult rhabdomyoma==== | |||
*Patients with [[laryngeal]] rhabdomyoma need immediate care such as [[nasal]] oxygen, [[intravenous fluids]] and if respiratory distress develops intubation before admission for surgery and surgical excision. <ref name="pmid24288631">{{cite journal |vauthors=Amelia Souza A, de Araújo VC, Passador Santos F, Ferreira Martinez E, de Menezes Filho JF, Soares de Araujo N, Soares AB |title=Intraoral adult rhabdomyoma: a case report |journal=Case Rep Dent |volume=2013 |issue= |pages=741548 |date=2013 |pmid=24288631 |pmc=3833031 |doi=10.1155/2013/741548 |url=}}</ref> | |||
*Patients with adult rhabdomyoma and shortness of breath should restrain from activities which exacerbate their breathing difficulty. | |||
====Cardiac rhabdomyoma==== | |||
*[[Pharmacological]] treatment available for [[fetal]] cardiac rhabdomyoma is [[everolimus]].<ref name="pmid29759095">{{cite journal |vauthors=Martínez-García A, Michel-Macías C, Cordero-González G, Escamilla-Sánchez KI, Aguinaga-Ríos M, Coronado-Zarco A, Cardona-Pérez JA |title=Giant left ventricular rhabdomyoma treated successfully with everolimus: case report and review of literature |journal=Cardiol Young |volume=28 |issue=7 |pages=903–909 |date=July 2018 |pmid=29759095 |doi=10.1017/S1047951118000598 |url=}}</ref> | |||
*Patients with [[arrhythmia]]s are treated with [[antiarrhythmic medications]]. | |||
*Restriction on physical activities in those patients with [[cardiac]] clinical symptoms. | |||
*[[Asymptomatic]] children with [[TSC]] and a rhabdomyoma should have [[echocardiography]] every one to three years until regression of cardiac rhabdomyoma is documented. | |||
====Genital rhabdomyoma==== | |||
*In patients with [[genital]] rhabdomyoma, urinary tract obstruction may happen, therefore [[catheterization]] use may be necessary in order to avoid [[Urinary tract infections|urinary tract infection]]. | |||
*Pregnant patients need to be monitored closely, as they may require a [[cesarean delivery]].<ref name="pmid21992817">{{cite journal |vauthors=Lu DY, Chang S, Cook H, Alizadeh Y, Karam AK, Moatamed NA, Dry SM |title=Genital rhabdomyoma of the urethra in an infant girl |journal=Hum. Pathol. |volume=43 |issue=4 |pages=597–600 |date=April 2012 |pmid=21992817 |doi=10.1016/j.humpath.2011.06.012 |url=}}</ref><ref name="pmid3031168">{{cite journal |vauthors=Dodat H, Paulhac JB, Macabeo V, Bouvier R |title=[Benign tumors of the posterior urethra in children. Apropos of an unusual case of rhabdomyoma of fetal type] |language=French |journal=J Urol (Paris) |volume=93 |issue=1 |pages=43–6 |date=1987 |pmid=3031168 |doi= |url=}}</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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{{WS}} | {{WS}} | ||
[[Category: | [[Category:Oncology]] | ||
[[Category: | [[Category:Rhabdomyoma]] | ||
Latest revision as of 22:04, 10 January 2019
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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.
Treatment
Medical Therapy
- Treatment for rhabdomyoma is supportive care.[1][2] [3]
- patients with rhabdomyoma should be monitored with echocardiography and electrocardiography.
Adult rhabdomyoma
- Patients with laryngeal rhabdomyoma need immediate care such as nasal oxygen, intravenous fluids and if respiratory distress develops intubation before admission for surgery and surgical excision. [4]
- Patients with adult rhabdomyoma and shortness of breath should restrain from activities which exacerbate their breathing difficulty.
Cardiac rhabdomyoma
- Pharmacological treatment available for fetal cardiac rhabdomyoma is everolimus.[5]
- Patients with arrhythmias are treated with antiarrhythmic medications.
- Restriction on physical activities in those patients with cardiac clinical symptoms.
- Asymptomatic children with TSC and a rhabdomyoma should have echocardiography every one to three years until regression of cardiac rhabdomyoma is documented.
Genital rhabdomyoma
- In patients with genital rhabdomyoma, urinary tract obstruction may happen, therefore catheterization use may be necessary in order to avoid urinary tract infection.
- Pregnant patients need to be monitored closely, as they may require a cesarean delivery.[6][7]
References
- ↑ Tiberio D, Franz DN, Phillips JR (2011). "Regression of a cardiac rhabdomyoma in a patient receiving everolimus". Pediatrics. 127 (5): e1335–7. doi:10.1542/peds.2010-2910. PMID 21464184.
- ↑ Wagner R, Riede FT, Seki H, Hornemann F, Syrbe S, Daehnert I; et al. (2015). "Oral Everolimus for Treatment of a Giant Left Ventricular Rhabdomyoma in a Neonate-Rapid Tumor Regression Documented by Real Time 3D Echocardiography". Echocardiography. 32 (12): 1876–9. doi:10.1111/echo.13015. PMID 26199144.
- ↑ Öztunç F, Atik SU, Güneş AO (October 2015). "Everolimus treatment of a newborn with rhabdomyoma causing severe arrhythmia". Cardiol Young. 25 (7): 1411–4. doi:10.1017/S1047951114002261. PMID 26339757.
- ↑ Amelia Souza A, de Araújo VC, Passador Santos F, Ferreira Martinez E, de Menezes Filho JF, Soares de Araujo N, Soares AB (2013). "Intraoral adult rhabdomyoma: a case report". Case Rep Dent. 2013: 741548. doi:10.1155/2013/741548. PMC 3833031. PMID 24288631.
- ↑ Martínez-García A, Michel-Macías C, Cordero-González G, Escamilla-Sánchez KI, Aguinaga-Ríos M, Coronado-Zarco A, Cardona-Pérez JA (July 2018). "Giant left ventricular rhabdomyoma treated successfully with everolimus: case report and review of literature". Cardiol Young. 28 (7): 903–909. doi:10.1017/S1047951118000598. PMID 29759095.
- ↑ Lu DY, Chang S, Cook H, Alizadeh Y, Karam AK, Moatamed NA, Dry SM (April 2012). "Genital rhabdomyoma of the urethra in an infant girl". Hum. Pathol. 43 (4): 597–600. doi:10.1016/j.humpath.2011.06.012. PMID 21992817.
- ↑ Dodat H, Paulhac JB, Macabeo V, Bouvier R (1987). "[Benign tumors of the posterior urethra in children. Apropos of an unusual case of rhabdomyoma of fetal type]". J Urol (Paris) (in French). 93 (1): 43–6. PMID 3031168.