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{{Rhabdomyoma}}
{{Rhabdomyoma}}
{{CMG}}{{AE}}{{Simrat}}
{{CMG}}{{AE}}{{Nnasiri}}
==Overview==
==Overview==
Enteropathy-associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell [[Non-Hodgkin lymphoma|non-hodgkin lymphoma]] that affects the [[small intestine]], it is composed of large [[lymphoid]] cells. Enteropathy-associated T-cell lymphoma has two subtypes, type I enteropathy-associated T-cell lymphoma which has a strong association with [[celiac disease]] and it is more common in western countries and type II enteropathy-associated T-cell lymphoma which is mostly found among the Asian population. [[Genes]] involved in the [[pathogenesis]] of this disease include 8q24, [[T-cell receptor]] (TCR) beta and gamma, and 16q genes. On gross [[pathology]], multiple [[intestinal]] ulcers are characteristic findings of EATL. On microscopic [[histopathological]] analysis, monotonous cells, round or angulated vesicular [[nuclei]], and prominent [[nucleoli]] are characteristic findings of enteropathy-associated T-cell lymphoma. There are no established causes for enteropathy-associated T-cell lymphoma. EATL must be differentiated from other diseases such as [[peptic ulcer]], poorly-differentiated [[adenocarcinoma]], [[MALT lymphoma]], [[diffuse large B cell lymphoma]], and [[mantle cell lymphoma]].
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.
 
==Natural history and complications==
*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]].
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Latest revision as of 17:49, 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.

Natural history and complications

References

  1. 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.
  2. 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.
  3. 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.

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