Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A
Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies. Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreporte...
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| Veröffentlicht in: | Pediatric blood & cancer 2010-12, Vol.55 (6), p.1213-1216 |
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| container_title | Pediatric blood & cancer |
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| creator | Jones, Ashley E. Albano, Edythe A. Lovell, Mark A. Hunger, Stephen P. |
| description | Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies. Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreported association of MEN‐2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options. The high penetrance of malignancy in patients with MEN warrants a heightened suspicion for the development of nonendocrine malignancies. The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients. Pediatr Blood Cancer. 2010;55:1213–1216. © 2010 Wiley‐Liss, Inc. |
| doi_str_mv | 10.1002/pbc.22591 |
| format | Article |
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Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreported association of MEN‐2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options. The high penetrance of malignancy in patients with MEN warrants a heightened suspicion for the development of nonendocrine malignancies. The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients. Pediatr Blood Cancer. 2010;55:1213–1216. © 2010 Wiley‐Liss, Inc.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.22591</identifier><identifier>PMID: 20533522</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>genetic syndrome ; Humans ; Infant ; Male ; medullary thyroid carcinoma ; MEN-2A ; Multiple Endocrine Neoplasia ; Multiple Endocrine Neoplasia Type 2a - pathology ; Multiple Endocrine Neoplasia Type 2a - therapy ; pediatric oncology ; Prognosis ; rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar - secondary ; Rhabdomyosarcoma, Alveolar - therapy</subject><ispartof>Pediatric blood & cancer, 2010-12, Vol.55 (6), p.1213-1216</ispartof><rights>Copyright © 2010 Wiley‐Liss, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3621-6352eba6d6fde302b9c6a181d64455c0737ae858831c9dd27173a6a2080405483</citedby><cites>FETCH-LOGICAL-c3621-6352eba6d6fde302b9c6a181d64455c0737ae858831c9dd27173a6a2080405483</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fpbc.22591$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fpbc.22591$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,777,781,1412,27905,27906,45555,45556</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/20533522$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Jones, Ashley E.</creatorcontrib><creatorcontrib>Albano, Edythe A.</creatorcontrib><creatorcontrib>Lovell, Mark A.</creatorcontrib><creatorcontrib>Hunger, Stephen P.</creatorcontrib><title>Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A</title><title>Pediatric blood & cancer</title><addtitle>Pediatr. Blood Cancer</addtitle><description>Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies. Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreported association of MEN‐2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options. The high penetrance of malignancy in patients with MEN warrants a heightened suspicion for the development of nonendocrine malignancies. The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients. Pediatr Blood Cancer. 2010;55:1213–1216. © 2010 Wiley‐Liss, Inc.</description><subject>genetic syndrome</subject><subject>Humans</subject><subject>Infant</subject><subject>Male</subject><subject>medullary thyroid carcinoma</subject><subject>MEN-2A</subject><subject>Multiple Endocrine Neoplasia</subject><subject>Multiple Endocrine Neoplasia Type 2a - pathology</subject><subject>Multiple Endocrine Neoplasia Type 2a - therapy</subject><subject>pediatric oncology</subject><subject>Prognosis</subject><subject>rhabdomyosarcoma</subject><subject>Rhabdomyosarcoma, Alveolar - secondary</subject><subject>Rhabdomyosarcoma, Alveolar - therapy</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kMtOwzAQRS0E4lFY8AMoO8Qi1I_YSZZQoIDKQ6jA0po4U2FwmmCnQP-eQGl3rGYWZ-7MHEL2GT1mlPJ-U5hjzmXO1sg2k4mMJWXp-qqn-RbZCeG1QxWV2SbZ4lQKITnfJqMbbCG00FoTgfvA2oGP_AsUZV3N6wDe1BVEdhpVM9faxmGE07I23k4xmmLdOAgWonbeYMRPdsnGBFzAvb_aI48X5-PBZTy6G14NTkaxEYqzWHWrsQBVqkmJgvIiNwpYxkqVJFIamooUMJNZJpjJy5KnLBWggNOMJlQmmeiRw0Vu4-v3GYZWVzYYdA66k2ZBp4pRwYVkHXm0II2vQ_A40Y23Ffi5ZlT_uNOdO_3rrmMP_lJnRYXlilzK6oD-Avi0Duf_J-n708EyMl5M2NDi12oC_JtW3ZdSP98O9ZMY59dnoxv9IL4BUHeGjw</recordid><startdate>20101201</startdate><enddate>20101201</enddate><creator>Jones, Ashley E.</creator><creator>Albano, Edythe A.</creator><creator>Lovell, Mark A.</creator><creator>Hunger, Stephen P.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20101201</creationdate><title>Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A</title><author>Jones, Ashley E. ; Albano, Edythe A. ; Lovell, Mark A. ; Hunger, Stephen P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3621-6352eba6d6fde302b9c6a181d64455c0737ae858831c9dd27173a6a2080405483</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><topic>genetic syndrome</topic><topic>Humans</topic><topic>Infant</topic><topic>Male</topic><topic>medullary thyroid carcinoma</topic><topic>MEN-2A</topic><topic>Multiple Endocrine Neoplasia</topic><topic>Multiple Endocrine Neoplasia Type 2a - pathology</topic><topic>Multiple Endocrine Neoplasia Type 2a - therapy</topic><topic>pediatric oncology</topic><topic>Prognosis</topic><topic>rhabdomyosarcoma</topic><topic>Rhabdomyosarcoma, Alveolar - secondary</topic><topic>Rhabdomyosarcoma, Alveolar - therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Jones, Ashley E.</creatorcontrib><creatorcontrib>Albano, Edythe A.</creatorcontrib><creatorcontrib>Lovell, Mark A.</creatorcontrib><creatorcontrib>Hunger, Stephen P.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric blood & cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Jones, Ashley E.</au><au>Albano, Edythe A.</au><au>Lovell, Mark A.</au><au>Hunger, Stephen P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A</atitle><jtitle>Pediatric blood & cancer</jtitle><addtitle>Pediatr. Blood Cancer</addtitle><date>2010-12-01</date><risdate>2010</risdate><volume>55</volume><issue>6</issue><spage>1213</spage><epage>1216</epage><pages>1213-1216</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies. Multiple Endocrine Neoplasia (MEN) type 2A is an autosomal dominant syndrome associated with near universal development of medullary thyroid carcinoma. We describe a previously unreported association of MEN‐2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options. The high penetrance of malignancy in patients with MEN warrants a heightened suspicion for the development of nonendocrine malignancies. The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients. 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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | genetic syndrome Humans Infant Male medullary thyroid carcinoma MEN-2A Multiple Endocrine Neoplasia Multiple Endocrine Neoplasia Type 2a - pathology Multiple Endocrine Neoplasia Type 2a - therapy pediatric oncology Prognosis rhabdomyosarcoma Rhabdomyosarcoma, Alveolar - secondary Rhabdomyosarcoma, Alveolar - therapy |
| title | Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A |
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