Multifocality in Sporadic Medullary Thyroid Carcinoma: An International Multicenter Study

Background: Current surgical standard of care in sporadic medullary thyroid carcinoma (sMTC) consists of a minimum of total thyroidectomy with central neck dissection. Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequ...

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Veröffentlicht in:Thyroid (New York, N.Y.) N.Y.), 2016-11, Vol.26 (11), p.1563-1572
Hauptverfasser: Essig, Garth F., Porter, Kyle, Schneider, David, Arpaia, Debora, Lindsey, Susan C., Busonero, Giulia, Fineberg, Daniel, Fruci, Barbara, Boelaert, Kristien, Smit, Johannes W., Meijer, Johannes Arnoldus Anthonius, Duntas, Leonidas H., Sharma, Neil, Costante, Giuseppe, Filetti, Sebastiano, Sippel, Rebecca S., Biondi, Bernadette, Topliss, Duncan J., Pacini, Furio, Maciel, Rui M.B., Walz, Patrick C., Kloos, Richard T.
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container_end_page 1572
container_issue 11
container_start_page 1563
container_title Thyroid (New York, N.Y.)
container_volume 26
creator Essig, Garth F.
Porter, Kyle
Schneider, David
Arpaia, Debora
Lindsey, Susan C.
Busonero, Giulia
Fineberg, Daniel
Fruci, Barbara
Boelaert, Kristien
Smit, Johannes W.
Meijer, Johannes Arnoldus Anthonius
Duntas, Leonidas H.
Sharma, Neil
Costante, Giuseppe
Filetti, Sebastiano
Sippel, Rebecca S.
Biondi, Bernadette
Topliss, Duncan J.
Pacini, Furio
Maciel, Rui M.B.
Walz, Patrick C.
Kloos, Richard T.
description Background: Current surgical standard of care in sporadic medullary thyroid carcinoma (sMTC) consists of a minimum of total thyroidectomy with central neck dissection. Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983–2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8% demonstrated a negative family history and no manifestations of MEN2 syndromes other than MTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p  
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Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983–2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8% demonstrated a negative family history and no manifestations of MEN2 syndromes other than MTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p  &lt; 0.001). No geographic differences in focality were identified. Conclusions: The 5.6% prevalence of bilateral foci in sMTC suggests that total thyroidectomy should remain the standard of care for initial surgery, as less complete thyroid surgery may fail to address fully the primary site of disease. Whether ipsilateral tumor focality should be an independent factor determining the need for completion thyroidectomy when sMTC is diagnosed after hemithyroidectomy remains to be determined.</description><identifier>ISSN: 1050-7256</identifier><identifier>EISSN: 1557-9077</identifier><identifier>DOI: 10.1089/thy.2016.0255</identifier><identifier>PMID: 27604949</identifier><language>eng</language><publisher>United States: Mary Ann Liebert, Inc</publisher><subject>Adult ; Aged ; Aged, 80 and over ; Biopsy ; Carcinoma, Medullary - epidemiology ; Carcinoma, Medullary - pathology ; Carcinoma, Medullary - prevention &amp; control ; Carcinoma, Medullary - surgery ; Carcinoma, Neuroendocrine - epidemiology ; Carcinoma, Neuroendocrine - pathology ; Carcinoma, Neuroendocrine - prevention &amp; control ; Carcinoma, Neuroendocrine - surgery ; Cohort Studies ; Humans ; Middle Aged ; Neoplasm Recurrence, Local - epidemiology ; Neoplasm Recurrence, Local - pathology ; Neoplasm Recurrence, Local - prevention &amp; control ; Neoplasm Staging ; Practice Guidelines as Topic ; Prevalence ; Retrospective Studies ; Thyroid Cancer and Nodules ; Thyroid Gland - pathology ; Thyroid Gland - surgery ; Thyroid Neoplasms - epidemiology ; Thyroid Neoplasms - pathology ; Thyroid Neoplasms - prevention &amp; control ; Thyroid Neoplasms - surgery ; Thyroidectomy - adverse effects ; Tumor Burden ; Young Adult</subject><ispartof>Thyroid (New York, N.Y.), 2016-11, Vol.26 (11), p.1563-1572</ispartof><rights>2016, Mary Ann Liebert, Inc.</rights><rights>Copyright 2016, Mary Ann Liebert, Inc. 2016</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c501t-581b4e7ad0874154738707ff67ff8ab0cf23a758ff7822e8c8c8dc0502e375383</citedby><cites>FETCH-LOGICAL-c501t-581b4e7ad0874154738707ff67ff8ab0cf23a758ff7822e8c8c8dc0502e375383</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,776,780,881,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27604949$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Essig, Garth F.</creatorcontrib><creatorcontrib>Porter, Kyle</creatorcontrib><creatorcontrib>Schneider, David</creatorcontrib><creatorcontrib>Arpaia, Debora</creatorcontrib><creatorcontrib>Lindsey, Susan C.</creatorcontrib><creatorcontrib>Busonero, Giulia</creatorcontrib><creatorcontrib>Fineberg, Daniel</creatorcontrib><creatorcontrib>Fruci, Barbara</creatorcontrib><creatorcontrib>Boelaert, Kristien</creatorcontrib><creatorcontrib>Smit, Johannes W.</creatorcontrib><creatorcontrib>Meijer, Johannes Arnoldus Anthonius</creatorcontrib><creatorcontrib>Duntas, Leonidas H.</creatorcontrib><creatorcontrib>Sharma, Neil</creatorcontrib><creatorcontrib>Costante, Giuseppe</creatorcontrib><creatorcontrib>Filetti, Sebastiano</creatorcontrib><creatorcontrib>Sippel, Rebecca S.</creatorcontrib><creatorcontrib>Biondi, Bernadette</creatorcontrib><creatorcontrib>Topliss, Duncan J.</creatorcontrib><creatorcontrib>Pacini, Furio</creatorcontrib><creatorcontrib>Maciel, Rui M.B.</creatorcontrib><creatorcontrib>Walz, Patrick C.</creatorcontrib><creatorcontrib>Kloos, Richard T.</creatorcontrib><title>Multifocality in Sporadic Medullary Thyroid Carcinoma: An International Multicenter Study</title><title>Thyroid (New York, N.Y.)</title><addtitle>Thyroid</addtitle><description>Background: Current surgical standard of care in sporadic medullary thyroid carcinoma (sMTC) consists of a minimum of total thyroidectomy with central neck dissection. Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983–2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8% demonstrated a negative family history and no manifestations of MEN2 syndromes other than MTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p  &lt; 0.001). No geographic differences in focality were identified. Conclusions: The 5.6% prevalence of bilateral foci in sMTC suggests that total thyroidectomy should remain the standard of care for initial surgery, as less complete thyroid surgery may fail to address fully the primary site of disease. Whether ipsilateral tumor focality should be an independent factor determining the need for completion thyroidectomy when sMTC is diagnosed after hemithyroidectomy remains to be determined.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Biopsy</subject><subject>Carcinoma, Medullary - epidemiology</subject><subject>Carcinoma, Medullary - pathology</subject><subject>Carcinoma, Medullary - prevention &amp; control</subject><subject>Carcinoma, Medullary - surgery</subject><subject>Carcinoma, Neuroendocrine - epidemiology</subject><subject>Carcinoma, Neuroendocrine - pathology</subject><subject>Carcinoma, Neuroendocrine - prevention &amp; control</subject><subject>Carcinoma, Neuroendocrine - surgery</subject><subject>Cohort Studies</subject><subject>Humans</subject><subject>Middle Aged</subject><subject>Neoplasm Recurrence, Local - epidemiology</subject><subject>Neoplasm Recurrence, Local - pathology</subject><subject>Neoplasm Recurrence, Local - prevention &amp; control</subject><subject>Neoplasm Staging</subject><subject>Practice Guidelines as Topic</subject><subject>Prevalence</subject><subject>Retrospective Studies</subject><subject>Thyroid Cancer and Nodules</subject><subject>Thyroid Gland - pathology</subject><subject>Thyroid Gland - surgery</subject><subject>Thyroid Neoplasms - epidemiology</subject><subject>Thyroid Neoplasms - pathology</subject><subject>Thyroid Neoplasms - prevention &amp; control</subject><subject>Thyroid Neoplasms - surgery</subject><subject>Thyroidectomy - adverse effects</subject><subject>Tumor Burden</subject><subject>Young Adult</subject><issn>1050-7256</issn><issn>1557-9077</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc1PwyAYxonROJ0evRqOXjr5KIV6MDGLH0s0HpwHT4RR6jAdTEpN-t9L3TR6MoRAXn55eN73AeAEowlGojyPy35CEC4miDC2Aw4wYzwrEee76Y4YyjhhxQgctu0bSpjgdB-MCC9QXublAXh56Jpoa69VY2MPrYNPax9UZTV8MFXXNCr0cL7sg7cVnKqgrfMrdQGvHJy5aIJT0XqnGvilo81Qg0-xq_ojsFerpjXH23MMnm-u59O77P7xdja9us80QzhmTOBFbriqkOA5ZjmngiNe10XaQi2QrglVnIm65oIQI3RalU6NEUM5o4KOweVGd90tVqYaLATVyHWwq-RdemXl3xdnl_LVf8giZzRP8xiDs61A8O-daaNc2Vab1LozvmslFjQvS1agIqHZBtXBt20w9c83GMkhDpnikEMccogj8ae_vf3Q3_NPAN0AQ1k511izMCH-I_sJnl2ZUA</recordid><startdate>20161101</startdate><enddate>20161101</enddate><creator>Essig, Garth F.</creator><creator>Porter, Kyle</creator><creator>Schneider, David</creator><creator>Arpaia, Debora</creator><creator>Lindsey, Susan C.</creator><creator>Busonero, Giulia</creator><creator>Fineberg, Daniel</creator><creator>Fruci, Barbara</creator><creator>Boelaert, Kristien</creator><creator>Smit, Johannes W.</creator><creator>Meijer, Johannes Arnoldus Anthonius</creator><creator>Duntas, Leonidas H.</creator><creator>Sharma, Neil</creator><creator>Costante, Giuseppe</creator><creator>Filetti, Sebastiano</creator><creator>Sippel, Rebecca S.</creator><creator>Biondi, Bernadette</creator><creator>Topliss, Duncan J.</creator><creator>Pacini, Furio</creator><creator>Maciel, Rui M.B.</creator><creator>Walz, Patrick C.</creator><creator>Kloos, Richard T.</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><scope>5PM</scope></search><sort><creationdate>20161101</creationdate><title>Multifocality in Sporadic Medullary Thyroid Carcinoma: An International Multicenter Study</title><author>Essig, Garth F. ; Porter, Kyle ; Schneider, David ; Arpaia, Debora ; Lindsey, Susan C. ; Busonero, Giulia ; Fineberg, Daniel ; Fruci, Barbara ; Boelaert, Kristien ; Smit, Johannes W. ; Meijer, Johannes Arnoldus Anthonius ; Duntas, Leonidas H. ; Sharma, Neil ; Costante, Giuseppe ; Filetti, Sebastiano ; Sippel, Rebecca S. ; Biondi, Bernadette ; Topliss, Duncan J. ; Pacini, Furio ; Maciel, Rui M.B. ; Walz, Patrick C. ; Kloos, Richard T.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c501t-581b4e7ad0874154738707ff67ff8ab0cf23a758ff7822e8c8c8dc0502e375383</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Biopsy</topic><topic>Carcinoma, Medullary - epidemiology</topic><topic>Carcinoma, Medullary - pathology</topic><topic>Carcinoma, Medullary - prevention &amp; control</topic><topic>Carcinoma, Medullary - surgery</topic><topic>Carcinoma, Neuroendocrine - epidemiology</topic><topic>Carcinoma, Neuroendocrine - pathology</topic><topic>Carcinoma, Neuroendocrine - prevention &amp; 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Some have suggested thyroid lobectomy with isthmusectomy and central neck dissection for patients with sMTC, given their lower frequency of bilateral disease, although this topic has not been thoroughly studied. This study assessed the prevalence of multifocality in sMTC via a large international multi-institutional retrospective review to quantify this prevalence, including the impact of geography, to assess more accurately the risks associated with alternative surgical approaches. Methods: A retrospective chart review of sMTC patients from 11 institutions over 29 years (1983–2011) was undertaken. Data regarding focality, extent of disease, RET germline analysis plus family and clinical history for multiple endocrine neoplasia type 2 (MEN2), and demographic data were collected and analyzed. Results: Patients from four continents and seven countries were included in the sample. Data for 313 patients with documented sMTC were collected. Of these, 81.2% were confirmed with negative RET germline testing, while the remaining 18.8% demonstrated a negative family history and no manifestations of MEN2 syndromes other than MTC. Bilateral disease was identified in 17/306 (5.6%) patients, while multifocal disease was noted in 50/312 (16.0%) sMTC patients. When only accounting for germline negative patients, these rates were not significantly different (5.6% and 17%, respectively). Among them, when disease was unifocal in the ipsilateral lobe and isthmus, bilateral disease was present in 6/212 (2.8%) cases. When disease was multifocal in the ipsilateral lobe or isthmus, then bilateral disease was present in 8/37 (21.6%) cases ( p  &lt; 0.001). No geographic differences in focality were identified. Conclusions: The 5.6% prevalence of bilateral foci in sMTC suggests that total thyroidectomy should remain the standard of care for initial surgery, as less complete thyroid surgery may fail to address fully the primary site of disease. Whether ipsilateral tumor focality should be an independent factor determining the need for completion thyroidectomy when sMTC is diagnosed after hemithyroidectomy remains to be determined.</abstract><cop>United States</cop><pub>Mary Ann Liebert, Inc</pub><pmid>27604949</pmid><doi>10.1089/thy.2016.0255</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record>
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subjects Adult
Aged
Aged, 80 and over
Biopsy
Carcinoma, Medullary - epidemiology
Carcinoma, Medullary - pathology
Carcinoma, Medullary - prevention & control
Carcinoma, Medullary - surgery
Carcinoma, Neuroendocrine - epidemiology
Carcinoma, Neuroendocrine - pathology
Carcinoma, Neuroendocrine - prevention & control
Carcinoma, Neuroendocrine - surgery
Cohort Studies
Humans
Middle Aged
Neoplasm Recurrence, Local - epidemiology
Neoplasm Recurrence, Local - pathology
Neoplasm Recurrence, Local - prevention & control
Neoplasm Staging
Practice Guidelines as Topic
Prevalence
Retrospective Studies
Thyroid Cancer and Nodules
Thyroid Gland - pathology
Thyroid Gland - surgery
Thyroid Neoplasms - epidemiology
Thyroid Neoplasms - pathology
Thyroid Neoplasms - prevention & control
Thyroid Neoplasms - surgery
Thyroidectomy - adverse effects
Tumor Burden
Young Adult
title Multifocality in Sporadic Medullary Thyroid Carcinoma: An International Multicenter Study
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