Diagnostic and surgical dilemmas in hereditary medullary thyroid carcinoma
Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia t...
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| Veröffentlicht in: | The Laryngoscope 2009-07, Vol.119 (7), p.1303-1311 |
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| container_title | The Laryngoscope |
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| creator | Allen, Shawn M. Bodenner, Donald Suen, James Y. Richter, Gresham T. |
| description | Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC. Laryngoscope, 2009 |
| doi_str_mv | 10.1002/lary.20299 |
| format | Article |
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The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC. Laryngoscope, 2009</description><identifier>ISSN: 0023-852X</identifier><identifier>EISSN: 1531-4995</identifier><identifier>DOI: 10.1002/lary.20299</identifier><identifier>PMID: 19444888</identifier><identifier>CODEN: LARYA8</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Biological and medical sciences ; Biopsy, Fine-Needle ; Calcitonin - blood ; Carcinoma, Medullary - diagnosis ; Carcinoma, Medullary - genetics ; Carcinoma, Medullary - surgery ; diagnosis ; Diagnosis, Differential ; dilemmas ; Endocrinopathies ; familial medullar thyroid carcinoma ; Genetic Predisposition to Disease ; hereditary medullary thyroid carcinoma ; Humans ; Male ; Malignant tumors ; Medical sciences ; Medullary thyroid carcinoma ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a - diagnosis ; Multiple Endocrine Neoplasia Type 2a - genetics ; Multiple Endocrine Neoplasia Type 2a - surgery ; Non tumoral diseases. Target tissue resistance. Benign neoplasms ; Otorhinolaryngology. Stomatology ; Proto-Oncogene Proteins c-ret - genetics ; therapy ; Thyroid Neoplasms - diagnosis ; Thyroid Neoplasms - genetics ; Thyroid Neoplasms - surgery ; Thyroid. Thyroid axis (diseases) ; Thyroidectomy</subject><ispartof>The Laryngoscope, 2009-07, Vol.119 (7), p.1303-1311</ispartof><rights>Copyright © 2009 The American Laryngological, Rhinological, and Otological Society, Inc.</rights><rights>2009 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3959-d8eb2e3454ed7896a18659e19a2654ef03f3c4329092fcc08a420073ae6152b93</citedby><cites>FETCH-LOGICAL-c3959-d8eb2e3454ed7896a18659e19a2654ef03f3c4329092fcc08a420073ae6152b93</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%2Flary.20299$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Flary.20299$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=21709454$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19444888$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Allen, Shawn M.</creatorcontrib><creatorcontrib>Bodenner, Donald</creatorcontrib><creatorcontrib>Suen, James Y.</creatorcontrib><creatorcontrib>Richter, Gresham T.</creatorcontrib><title>Diagnostic and surgical dilemmas in hereditary medullary thyroid carcinoma</title><title>The Laryngoscope</title><addtitle>The Laryngoscope</addtitle><description>Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC. Laryngoscope, 2009</description><subject>Biological and medical sciences</subject><subject>Biopsy, Fine-Needle</subject><subject>Calcitonin - blood</subject><subject>Carcinoma, Medullary - diagnosis</subject><subject>Carcinoma, Medullary - genetics</subject><subject>Carcinoma, Medullary - surgery</subject><subject>diagnosis</subject><subject>Diagnosis, Differential</subject><subject>dilemmas</subject><subject>Endocrinopathies</subject><subject>familial medullar thyroid carcinoma</subject><subject>Genetic Predisposition to Disease</subject><subject>hereditary medullary thyroid carcinoma</subject><subject>Humans</subject><subject>Male</subject><subject>Malignant tumors</subject><subject>Medical sciences</subject><subject>Medullary thyroid carcinoma</subject><subject>Middle Aged</subject><subject>Multiple Endocrine Neoplasia Type 2a - diagnosis</subject><subject>Multiple Endocrine Neoplasia Type 2a - genetics</subject><subject>Multiple Endocrine Neoplasia Type 2a - surgery</subject><subject>Non tumoral diseases. Target tissue resistance. Benign neoplasms</subject><subject>Otorhinolaryngology. Stomatology</subject><subject>Proto-Oncogene Proteins c-ret - genetics</subject><subject>therapy</subject><subject>Thyroid Neoplasms - diagnosis</subject><subject>Thyroid Neoplasms - genetics</subject><subject>Thyroid Neoplasms - surgery</subject><subject>Thyroid. 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Target tissue resistance. Benign neoplasms</topic><topic>Otorhinolaryngology. Stomatology</topic><topic>Proto-Oncogene Proteins c-ret - genetics</topic><topic>therapy</topic><topic>Thyroid Neoplasms - diagnosis</topic><topic>Thyroid Neoplasms - genetics</topic><topic>Thyroid Neoplasms - surgery</topic><topic>Thyroid. Thyroid axis (diseases)</topic><topic>Thyroidectomy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Allen, Shawn M.</creatorcontrib><creatorcontrib>Bodenner, Donald</creatorcontrib><creatorcontrib>Suen, James Y.</creatorcontrib><creatorcontrib>Richter, Gresham T.</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</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>The Laryngoscope</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Allen, Shawn M.</au><au>Bodenner, Donald</au><au>Suen, James Y.</au><au>Richter, Gresham T.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Diagnostic and surgical dilemmas in hereditary medullary thyroid carcinoma</atitle><jtitle>The Laryngoscope</jtitle><addtitle>The Laryngoscope</addtitle><date>2009-07</date><risdate>2009</risdate><volume>119</volume><issue>7</issue><spage>1303</spage><epage>1311</epage><pages>1303-1311</pages><issn>0023-852X</issn><eissn>1531-4995</eissn><coden>LARYA8</coden><abstract>Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC. Laryngoscope, 2009</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>19444888</pmid><doi>10.1002/lary.20299</doi><tpages>9</tpages></addata></record> |
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| subjects | Biological and medical sciences Biopsy, Fine-Needle Calcitonin - blood Carcinoma, Medullary - diagnosis Carcinoma, Medullary - genetics Carcinoma, Medullary - surgery diagnosis Diagnosis, Differential dilemmas Endocrinopathies familial medullar thyroid carcinoma Genetic Predisposition to Disease hereditary medullary thyroid carcinoma Humans Male Malignant tumors Medical sciences Medullary thyroid carcinoma Middle Aged Multiple Endocrine Neoplasia Type 2a - diagnosis Multiple Endocrine Neoplasia Type 2a - genetics Multiple Endocrine Neoplasia Type 2a - surgery Non tumoral diseases. Target tissue resistance. Benign neoplasms Otorhinolaryngology. Stomatology Proto-Oncogene Proteins c-ret - genetics therapy Thyroid Neoplasms - diagnosis Thyroid Neoplasms - genetics Thyroid Neoplasms - surgery Thyroid. Thyroid axis (diseases) Thyroidectomy |
| title | Diagnostic and surgical dilemmas in hereditary medullary thyroid carcinoma |
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