Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma
Background: Medullary thyroid carcinoma (MTC) is characterized by the synthesis and secretion of calcitonin (Ct). MTC without Ct secretion has been reported on rare occasions. The aim of this study was to analyze the prevalence and clinical spectrum of nonsecretory MTC in two tertiary centers that c...
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| Veröffentlicht in: | Thyroid (New York, N.Y.) N.Y.), 2013-03, Vol.23 (3), p.294-300 |
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| creator | Frank-Raue, Karin Machens, Andreas Leidig-Bruckner, Gudrun Rondot, Susanne Haag, Christine Schulze, Egbert Lorenz, Angela Kreissl, Michael C. Dralle, Henning Raue, Friedhelm Schmid, Kurt W. |
| description | Background:
Medullary thyroid carcinoma (MTC) is characterized by the synthesis and secretion of calcitonin (Ct). MTC without Ct secretion has been reported on rare occasions. The aim of this study was to analyze the prevalence and clinical spectrum of nonsecretory MTC in two tertiary centers that cared for 839 patients with sporadic MTC.
Methods:
Clinical, biochemical, histological, and immunohistological findings, and somatic
RET
mutations were analyzed, and long-term follow-up was documented.
Results:
Seven patients with nonsecretory MTC were identified among 839 patients with sporadic MTC; thus, the prevalence rate of nonsecretory MTC was 0.83%. In these seven patients, Ct and carcinoembryonic antigen (CEA) levels were normal when MTC was initially diagnosed in the patients, despite advanced tumor stage. Ct and CEA levels remained undetectable in four patients; recurrence was indicated in one patient after 10 years of follow-up by routine anatomic imaging and increased CEA levels, and Ct levels became slightly elevated during follow-up, despite massive tumor load, in the remaining two patients. The diagnosis of MTC was confirmed by positive immunohistochemistry for Ct, CEA, and chromogranin A. A high Ki67 proliferation index (PI) (three patients) and a high proportion of
RET
918-mutated cells (four patients), as well as poorly differentiated histology, were associated with aggressive biological behavior of the MTC. The prognosis for nonsecretory MTC varied between long-term survival (12.5 years) and rapid progression leading to death within 1.75 years after diagnosis.
Conclusions:
The prevalence of nonsecretory MTC was low (0.83% of patients with MTC). Diagnosis was often made at a clinically advanced tumor stage. The histological and immunohistological characteristics and the clinical course and prognosis of nonsecretory MTC are markedly heterogeneous. A high Ki-67 PI and a large proportion of cells with
RET
918 mutations are associated with a poor prognosis. |
| doi_str_mv | 10.1089/thy.2012.0236 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1315630416</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1315630416</sourcerecordid><originalsourceid>FETCH-LOGICAL-c337t-c311b789c6bdb5b8f9823a1099f237c21209466eec0991f93f4ce3601a05202a3</originalsourceid><addsrcrecordid>eNqFkclOwzAQhi0EYj9yRT5ySfHSOPERVWwSS6WWc-Q4E9UosYvtgPoaPDGOChzhYo9G33wa-0fojJIJJaW8jKvNhBHKJoRxsYMOaZ4XmSRFsZtqkpOsYLk4QEchvBJCRVnwfXTAmJyKaSkO0efcw7vqwGrAyjZ41hlrtOrwYg06-qHHrsVPzgbQHqLzG_wIzdB1KlXL1cY7k2aU18a6XmFjscIL8AbCOFdyiecqGrAx4A8TV8nqvGqM_stygvZa1QU4_b6P0cvN9XJ2lz08397Prh4yzXkR00lpXZRSi7qp87psZcm4okTKlvFCM8pIeqQA0KlFW8nbqQYuCFUkZ4Qpfowutt61d28DhFj1JmhIW1lwQ6gop7ngZEpFQrMtqr0LwUNbrb3p0_YVJdUYQ5ViqMYYqjGGxJ9_q4e6h-aX_vn3BPAtMLaVtZ2BGnz8R_sFBsaVpg</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1315630416</pqid></control><display><type>article</type><title>Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma</title><source>MEDLINE</source><source>Alma/SFX Local Collection</source><creator>Frank-Raue, Karin ; Machens, Andreas ; Leidig-Bruckner, Gudrun ; Rondot, Susanne ; Haag, Christine ; Schulze, Egbert ; Lorenz, Angela ; Kreissl, Michael C. ; Dralle, Henning ; Raue, Friedhelm ; Schmid, Kurt W.</creator><creatorcontrib>Frank-Raue, Karin ; Machens, Andreas ; Leidig-Bruckner, Gudrun ; Rondot, Susanne ; Haag, Christine ; Schulze, Egbert ; Lorenz, Angela ; Kreissl, Michael C. ; Dralle, Henning ; Raue, Friedhelm ; Schmid, Kurt W.</creatorcontrib><description>Background:
Medullary thyroid carcinoma (MTC) is characterized by the synthesis and secretion of calcitonin (Ct). MTC without Ct secretion has been reported on rare occasions. The aim of this study was to analyze the prevalence and clinical spectrum of nonsecretory MTC in two tertiary centers that cared for 839 patients with sporadic MTC.
Methods:
Clinical, biochemical, histological, and immunohistological findings, and somatic
RET
mutations were analyzed, and long-term follow-up was documented.
Results:
Seven patients with nonsecretory MTC were identified among 839 patients with sporadic MTC; thus, the prevalence rate of nonsecretory MTC was 0.83%. In these seven patients, Ct and carcinoembryonic antigen (CEA) levels were normal when MTC was initially diagnosed in the patients, despite advanced tumor stage. Ct and CEA levels remained undetectable in four patients; recurrence was indicated in one patient after 10 years of follow-up by routine anatomic imaging and increased CEA levels, and Ct levels became slightly elevated during follow-up, despite massive tumor load, in the remaining two patients. The diagnosis of MTC was confirmed by positive immunohistochemistry for Ct, CEA, and chromogranin A. A high Ki67 proliferation index (PI) (three patients) and a high proportion of
RET
918-mutated cells (four patients), as well as poorly differentiated histology, were associated with aggressive biological behavior of the MTC. The prognosis for nonsecretory MTC varied between long-term survival (12.5 years) and rapid progression leading to death within 1.75 years after diagnosis.
Conclusions:
The prevalence of nonsecretory MTC was low (0.83% of patients with MTC). Diagnosis was often made at a clinically advanced tumor stage. The histological and immunohistological characteristics and the clinical course and prognosis of nonsecretory MTC are markedly heterogeneous. A high Ki-67 PI and a large proportion of cells with
RET
918 mutations are associated with a poor prognosis.</description><identifier>ISSN: 1050-7256</identifier><identifier>EISSN: 1557-9077</identifier><identifier>DOI: 10.1089/thy.2012.0236</identifier><identifier>PMID: 22946486</identifier><language>eng</language><publisher>United States: Mary Ann Liebert, Inc</publisher><subject>Aged ; Biomarkers, Tumor - metabolism ; Carcinoma, Medullary - diagnosis ; Carcinoma, Medullary - epidemiology ; Carcinoma, Neuroendocrine ; Cell Proliferation ; Disease Progression ; Female ; Humans ; Ki-67 Antigen - metabolism ; Male ; Middle Aged ; Mutation ; Prevalence ; Prognosis ; Proto-Oncogene Proteins c-ret - metabolism ; Thyroid Cancer and Nodules ; Thyroid Neoplasms - diagnosis ; Thyroid Neoplasms - epidemiology ; Treatment Outcome</subject><ispartof>Thyroid (New York, N.Y.), 2013-03, Vol.23 (3), p.294-300</ispartof><rights>2013, Mary Ann Liebert, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c337t-c311b789c6bdb5b8f9823a1099f237c21209466eec0991f93f4ce3601a05202a3</citedby><cites>FETCH-LOGICAL-c337t-c311b789c6bdb5b8f9823a1099f237c21209466eec0991f93f4ce3601a05202a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22946486$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Frank-Raue, Karin</creatorcontrib><creatorcontrib>Machens, Andreas</creatorcontrib><creatorcontrib>Leidig-Bruckner, Gudrun</creatorcontrib><creatorcontrib>Rondot, Susanne</creatorcontrib><creatorcontrib>Haag, Christine</creatorcontrib><creatorcontrib>Schulze, Egbert</creatorcontrib><creatorcontrib>Lorenz, Angela</creatorcontrib><creatorcontrib>Kreissl, Michael C.</creatorcontrib><creatorcontrib>Dralle, Henning</creatorcontrib><creatorcontrib>Raue, Friedhelm</creatorcontrib><creatorcontrib>Schmid, Kurt W.</creatorcontrib><title>Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma</title><title>Thyroid (New York, N.Y.)</title><addtitle>Thyroid</addtitle><description>Background:
Medullary thyroid carcinoma (MTC) is characterized by the synthesis and secretion of calcitonin (Ct). MTC without Ct secretion has been reported on rare occasions. The aim of this study was to analyze the prevalence and clinical spectrum of nonsecretory MTC in two tertiary centers that cared for 839 patients with sporadic MTC.
Methods:
Clinical, biochemical, histological, and immunohistological findings, and somatic
RET
mutations were analyzed, and long-term follow-up was documented.
Results:
Seven patients with nonsecretory MTC were identified among 839 patients with sporadic MTC; thus, the prevalence rate of nonsecretory MTC was 0.83%. In these seven patients, Ct and carcinoembryonic antigen (CEA) levels were normal when MTC was initially diagnosed in the patients, despite advanced tumor stage. Ct and CEA levels remained undetectable in four patients; recurrence was indicated in one patient after 10 years of follow-up by routine anatomic imaging and increased CEA levels, and Ct levels became slightly elevated during follow-up, despite massive tumor load, in the remaining two patients. The diagnosis of MTC was confirmed by positive immunohistochemistry for Ct, CEA, and chromogranin A. A high Ki67 proliferation index (PI) (three patients) and a high proportion of
RET
918-mutated cells (four patients), as well as poorly differentiated histology, were associated with aggressive biological behavior of the MTC. The prognosis for nonsecretory MTC varied between long-term survival (12.5 years) and rapid progression leading to death within 1.75 years after diagnosis.
Conclusions:
The prevalence of nonsecretory MTC was low (0.83% of patients with MTC). Diagnosis was often made at a clinically advanced tumor stage. The histological and immunohistological characteristics and the clinical course and prognosis of nonsecretory MTC are markedly heterogeneous. A high Ki-67 PI and a large proportion of cells with
RET
918 mutations are associated with a poor prognosis.</description><subject>Aged</subject><subject>Biomarkers, Tumor - metabolism</subject><subject>Carcinoma, Medullary - diagnosis</subject><subject>Carcinoma, Medullary - epidemiology</subject><subject>Carcinoma, Neuroendocrine</subject><subject>Cell Proliferation</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Humans</subject><subject>Ki-67 Antigen - metabolism</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Mutation</subject><subject>Prevalence</subject><subject>Prognosis</subject><subject>Proto-Oncogene Proteins c-ret - metabolism</subject><subject>Thyroid Cancer and Nodules</subject><subject>Thyroid Neoplasms - diagnosis</subject><subject>Thyroid Neoplasms - epidemiology</subject><subject>Treatment Outcome</subject><issn>1050-7256</issn><issn>1557-9077</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkclOwzAQhi0EYj9yRT5ySfHSOPERVWwSS6WWc-Q4E9UosYvtgPoaPDGOChzhYo9G33wa-0fojJIJJaW8jKvNhBHKJoRxsYMOaZ4XmSRFsZtqkpOsYLk4QEchvBJCRVnwfXTAmJyKaSkO0efcw7vqwGrAyjZ41hlrtOrwYg06-qHHrsVPzgbQHqLzG_wIzdB1KlXL1cY7k2aU18a6XmFjscIL8AbCOFdyiecqGrAx4A8TV8nqvGqM_stygvZa1QU4_b6P0cvN9XJ2lz08397Prh4yzXkR00lpXZRSi7qp87psZcm4okTKlvFCM8pIeqQA0KlFW8nbqQYuCFUkZ4Qpfowutt61d28DhFj1JmhIW1lwQ6gop7ngZEpFQrMtqr0LwUNbrb3p0_YVJdUYQ5ViqMYYqjGGxJ9_q4e6h-aX_vn3BPAtMLaVtZ2BGnz8R_sFBsaVpg</recordid><startdate>20130301</startdate><enddate>20130301</enddate><creator>Frank-Raue, Karin</creator><creator>Machens, Andreas</creator><creator>Leidig-Bruckner, Gudrun</creator><creator>Rondot, Susanne</creator><creator>Haag, Christine</creator><creator>Schulze, Egbert</creator><creator>Lorenz, Angela</creator><creator>Kreissl, Michael C.</creator><creator>Dralle, Henning</creator><creator>Raue, Friedhelm</creator><creator>Schmid, Kurt W.</creator><general>Mary Ann Liebert, Inc</general><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>20130301</creationdate><title>Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma</title><author>Frank-Raue, Karin ; Machens, Andreas ; Leidig-Bruckner, Gudrun ; Rondot, Susanne ; Haag, Christine ; Schulze, Egbert ; Lorenz, Angela ; Kreissl, Michael C. ; Dralle, Henning ; Raue, Friedhelm ; Schmid, Kurt W.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c337t-c311b789c6bdb5b8f9823a1099f237c21209466eec0991f93f4ce3601a05202a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Aged</topic><topic>Biomarkers, Tumor - metabolism</topic><topic>Carcinoma, Medullary - diagnosis</topic><topic>Carcinoma, Medullary - epidemiology</topic><topic>Carcinoma, Neuroendocrine</topic><topic>Cell Proliferation</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Humans</topic><topic>Ki-67 Antigen - metabolism</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Mutation</topic><topic>Prevalence</topic><topic>Prognosis</topic><topic>Proto-Oncogene Proteins c-ret - metabolism</topic><topic>Thyroid Cancer and Nodules</topic><topic>Thyroid Neoplasms - diagnosis</topic><topic>Thyroid Neoplasms - epidemiology</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Frank-Raue, Karin</creatorcontrib><creatorcontrib>Machens, Andreas</creatorcontrib><creatorcontrib>Leidig-Bruckner, Gudrun</creatorcontrib><creatorcontrib>Rondot, Susanne</creatorcontrib><creatorcontrib>Haag, Christine</creatorcontrib><creatorcontrib>Schulze, Egbert</creatorcontrib><creatorcontrib>Lorenz, Angela</creatorcontrib><creatorcontrib>Kreissl, Michael C.</creatorcontrib><creatorcontrib>Dralle, Henning</creatorcontrib><creatorcontrib>Raue, Friedhelm</creatorcontrib><creatorcontrib>Schmid, Kurt W.</creatorcontrib><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>Thyroid (New York, N.Y.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Frank-Raue, Karin</au><au>Machens, Andreas</au><au>Leidig-Bruckner, Gudrun</au><au>Rondot, Susanne</au><au>Haag, Christine</au><au>Schulze, Egbert</au><au>Lorenz, Angela</au><au>Kreissl, Michael C.</au><au>Dralle, Henning</au><au>Raue, Friedhelm</au><au>Schmid, Kurt W.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma</atitle><jtitle>Thyroid (New York, N.Y.)</jtitle><addtitle>Thyroid</addtitle><date>2013-03-01</date><risdate>2013</risdate><volume>23</volume><issue>3</issue><spage>294</spage><epage>300</epage><pages>294-300</pages><issn>1050-7256</issn><eissn>1557-9077</eissn><abstract>Background:
Medullary thyroid carcinoma (MTC) is characterized by the synthesis and secretion of calcitonin (Ct). MTC without Ct secretion has been reported on rare occasions. The aim of this study was to analyze the prevalence and clinical spectrum of nonsecretory MTC in two tertiary centers that cared for 839 patients with sporadic MTC.
Methods:
Clinical, biochemical, histological, and immunohistological findings, and somatic
RET
mutations were analyzed, and long-term follow-up was documented.
Results:
Seven patients with nonsecretory MTC were identified among 839 patients with sporadic MTC; thus, the prevalence rate of nonsecretory MTC was 0.83%. In these seven patients, Ct and carcinoembryonic antigen (CEA) levels were normal when MTC was initially diagnosed in the patients, despite advanced tumor stage. Ct and CEA levels remained undetectable in four patients; recurrence was indicated in one patient after 10 years of follow-up by routine anatomic imaging and increased CEA levels, and Ct levels became slightly elevated during follow-up, despite massive tumor load, in the remaining two patients. The diagnosis of MTC was confirmed by positive immunohistochemistry for Ct, CEA, and chromogranin A. A high Ki67 proliferation index (PI) (three patients) and a high proportion of
RET
918-mutated cells (four patients), as well as poorly differentiated histology, were associated with aggressive biological behavior of the MTC. The prognosis for nonsecretory MTC varied between long-term survival (12.5 years) and rapid progression leading to death within 1.75 years after diagnosis.
Conclusions:
The prevalence of nonsecretory MTC was low (0.83% of patients with MTC). Diagnosis was often made at a clinically advanced tumor stage. The histological and immunohistological characteristics and the clinical course and prognosis of nonsecretory MTC are markedly heterogeneous. A high Ki-67 PI and a large proportion of cells with
RET
918 mutations are associated with a poor prognosis.</abstract><cop>United States</cop><pub>Mary Ann Liebert, Inc</pub><pmid>22946486</pmid><doi>10.1089/thy.2012.0236</doi><tpages>7</tpages></addata></record> |
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| identifier | ISSN: 1050-7256 |
| ispartof | Thyroid (New York, N.Y.), 2013-03, Vol.23 (3), p.294-300 |
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| source | MEDLINE; Alma/SFX Local Collection |
| subjects | Aged Biomarkers, Tumor - metabolism Carcinoma, Medullary - diagnosis Carcinoma, Medullary - epidemiology Carcinoma, Neuroendocrine Cell Proliferation Disease Progression Female Humans Ki-67 Antigen - metabolism Male Middle Aged Mutation Prevalence Prognosis Proto-Oncogene Proteins c-ret - metabolism Thyroid Cancer and Nodules Thyroid Neoplasms - diagnosis Thyroid Neoplasms - epidemiology Treatment Outcome |
| title | Prevalence and Clinical Spectrum of Nonsecretory Medullary Thyroid Carcinoma in a Series of 839 Patients with Sporadic Medullary Thyroid Carcinoma |
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