Rhabdomyoma history and symptoms: Difference between revisions
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{{Rhabdomyoma}} | {{Rhabdomyoma}} | ||
{{CMG}}; {{AE}} {{Nnasiri}} | |||
==Overview== | ==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]]. [[Cardiac]] rhabdomyoma is seen almost always in the [[pediatric]] age group and is associated with tuberous sclerosis, [[neurofibromatosis]], and [[sebaceous]] [[adenoma]]<nowiki/>s. 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. | 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]]. [[Cardiac]] rhabdomyoma is seen almost always in the [[pediatric]] age group and is associated with tuberous sclerosis, [[neurofibromatosis]], and [[sebaceous]] [[adenoma]]<nowiki/>s. 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. | ||
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**Tumors larger in diameter are more likely to cause [[arrhythmia]]s or [[hemodynamic]] instability, which are associated with an increased risk of death. | **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]]. | **The majority of patients with [[cardiac]] rhabdomyoma remain asymptomatic; however, some affected patients become symptomatic in the [[perinatal period]]. | ||
*Symptoms of adult rhabdomyoma may include:<ref name="pmid29318074">{{cite journal |vauthors=Altissimi G, Ralli M, Sementilli G, Fiorentino F, Ciofalo A, Greco A, de Vincentiis M, Corsi A, Cianfrone G |title=Adult-Type Rhabdomyoma of the Larynx: Clinicopathologic Study of an Uncommon Tumor in a Rare Location |journal=Case Rep Otolaryngol |volume=2017 |issue= |pages=7186768 |date=2017 |pmid=29318074 |pmc=5727691 |doi=10.1155/2017/7186768 |url=}}</ref> | |||
:*[[Hoarseness]] | |||
:*[[Difficulty breathing]] | |||
:*[[Difficulty swallowing]]([[dysphagia]]) | |||
:*[[laryngeal]] mass | |||
:*In a very rare occassion can present with symptoms of [[hyperparathyroidism]] if it involves parathyroid gland. <ref name="pmid30537102">{{cite journal |vauthors=Lee JP, Blake Sullivan C, Bayon R, Shearer AE, Robinson RA |title=Adult type rhabdomyoma presenting as a parathyroid adenoma |journal=Head Neck |volume= |issue= |pages= |date=December 2018 |pmid=30537102 |doi=10.1002/hed.25419 |url=}}</ref> | |||
*Symptoms of genital rhabdomyoma may include the following: | |||
:*[[Dyspareunia|Painful sexual intercourse due to medical or psychological causes]] | |||
*Symptoms of [[cardiac]] rhabdomyoma may include the following: | |||
:*[[Shortness of breath]] | |||
*In [[cardiac]] rhabdomyoma, symptoms if present, may be caused by obstruction of [[blood flow]] through the [[heart]] or consist of rhythm disturbances, such as [[heart block]] and [[ventricular tachycardia]].<ref name="pmid8863873">{{cite journal| author=Bosi G, Lintermans JP, Pellegrino PA, Svaluto-Moreolo G, Vliers A| title=The natural history of cardiac rhabdomyoma with and without tuberous sclerosis. | journal=Acta Paediatr | year= 1996 | volume= 85 | issue= 8 | pages= 928-31 | pmid=8863873 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8863873 }} </ref><ref name="pmid7979700">{{cite journal| author=Jacobs JP, Konstantakos AK, Holland FW, Herskowitz K, Ferrer PL, Perryman RA| title=Surgical treatment for cardiac rhabdomyomas in children. | journal=Ann Thorac Surg | year= 1994 | volume= 58 | issue= 5 | pages= 1552-5 | pmid=7979700 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7979700 }} </ref> | |||
==Refrences== | |||
{{reflist|2}} | |||
Latest revision as of 21:40, 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. 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.
History and symptoms
- 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.
- Symptoms of adult rhabdomyoma may include:[4]
- Hoarseness
- Difficulty breathing
- Difficulty swallowing(dysphagia)
- laryngeal mass
- In a very rare occassion can present with symptoms of hyperparathyroidism if it involves parathyroid gland. [5]
- Symptoms of genital rhabdomyoma may include the following:
- Symptoms of cardiac rhabdomyoma may include the following:
- In cardiac rhabdomyoma, symptoms if present, may be caused by obstruction of blood flow through the heart or consist of rhythm disturbances, such as heart block and ventricular tachycardia.[6][7]
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.
- ↑ Altissimi G, Ralli M, Sementilli G, Fiorentino F, Ciofalo A, Greco A, de Vincentiis M, Corsi A, Cianfrone G (2017). "Adult-Type Rhabdomyoma of the Larynx: Clinicopathologic Study of an Uncommon Tumor in a Rare Location". Case Rep Otolaryngol. 2017: 7186768. doi:10.1155/2017/7186768. PMC 5727691. PMID 29318074.
- ↑ Lee JP, Blake Sullivan C, Bayon R, Shearer AE, Robinson RA (December 2018). "Adult type rhabdomyoma presenting as a parathyroid adenoma". Head Neck. doi:10.1002/hed.25419. PMID 30537102.
- ↑ Bosi G, Lintermans JP, Pellegrino PA, Svaluto-Moreolo G, Vliers A (1996). "The natural history of cardiac rhabdomyoma with and without tuberous sclerosis". Acta Paediatr. 85 (8): 928–31. PMID 8863873.
- ↑ Jacobs JP, Konstantakos AK, Holland FW, Herskowitz K, Ferrer PL, Perryman RA (1994). "Surgical treatment for cardiac rhabdomyomas in children". Ann Thorac Surg. 58 (5): 1552–5. PMID 7979700.