<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0121-0793</journal-id>
<journal-title><![CDATA[Iatreia]]></journal-title>
<abbrev-journal-title><![CDATA[Iatreia]]></abbrev-journal-title>
<issn>0121-0793</issn>
<publisher>
<publisher-name><![CDATA[Universidad de Antioquia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0121-07932023000300377</article-id>
<article-id pub-id-type="doi">10.17533/udea.iatreia.190</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Neoplasia endocrina múltiple tipo 2B: un enfoque de diagnóstico genotipo-fenotipo y abordaje terapéutico]]></article-title>
<article-title xml:lang="en"><![CDATA[Multiple endocrine neoplasia type 2B: A genotype-phenotype diagnostic and therapeutic approach]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Agredo-Delgado]]></surname>
<given-names><![CDATA[Valentina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gutiérrez-Restrepo]]></surname>
<given-names><![CDATA[Johnayro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Román-González]]></surname>
<given-names><![CDATA[Alejandro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad de Antioquia  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<volume>36</volume>
<numero>3</numero>
<fpage>377</fpage>
<lpage>392</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0121-07932023000300377&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0121-07932023000300377&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0121-07932023000300377&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La neoplasia endocrina múltiple tipo 2B (MEN 2B) es un síndrome genético poco común, con una prevalencia estimada de 0,9 a 1,6 por millón de individuos y una incidencia de 1,4 a 2,6 por millón de nacidos vivos por año. Es causada por una mutación en el protooncogén RET y se caracteriza por la presencia de carcinoma medular de tiroides (CMT) en el 100 % de los casos, feocromocitoma hasta en el 50 % y características extraendocrinas como un fenotipo marfanoide, alteraciones musculoesqueléticas y dentales, neuromas mucosos, alacrimia y ganglioneuromatosis intestinal difusa con síntomas de dismotilidad esofágica y gastrointestinal. Este síndrome se asocia con una calidad de vida deteriorada y una supervivencia deficiente, debidas principalmente a la agresividad del CMT, enfermedad que usualmente es de aparición temprana o metastásica. El diagnóstico generalmente se realiza de forma tardía, lo cual retrasa el enfoque integral, que incluye tipificación genética, tratamiento quirúrgico temprano del CMT y terapias específicas como inhibidores de tirosina quinasa (ITK) en caso de CMT avanzado. La cirugía es el tratamiento principal y la única posibilidad de cura, aunque el advenimiento de las terapias dirigidas parece estar mejorando la supervivencia libre de progresión en los casos avanzados.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Multiple endocrine neoplasia type 2B (MEN 2B) is a rare genetic syndrome with an estimated prevalence of 0.9 to 1.6 per million individuals and an annual incidence of 1.4 to 2.6 per million live births. It is caused by a mutation in the RET proto-oncogene and is characterized by the presence of medullary thyroid carcinoma (MTC) in 100% of cases, pheochromocytoma in up to 50%, and extraendocrine features such as a marfanoid phenotype, musculoskeletal and dental abnormalities, mucosal neuromas, alacrimia, and diffuse intestinal ganglioneuromatosis with symptoms of esophageal and gastrointestinal dysmotility. This syndrome is associated with a diminished quality of life and poor survival, primarily due to the aggressiveness of MTC, which often presents at an early or metastatic stage. Diagnosis is often delayed, resulting in a delayed comprehensive approach that includes genetic profiling, early surgical management of MTC, and specific therapies such as tyrosine kinase inhibitors (TKIs) for advanced MTC. Surgery remains the mainstay and the only potential cure, although the advent of targeted therapies appears to improve progression-free survival in advanced cases]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Carcinoma Medular]]></kwd>
<kwd lng="es"><![CDATA[Feocromocitoma]]></kwd>
<kwd lng="es"><![CDATA[Nanopartículas]]></kwd>
<kwd lng="es"><![CDATA[Neoplasia Endocrina Múltiple Tipo 2b]]></kwd>
<kwd lng="es"><![CDATA[Neuroma]]></kwd>
<kwd lng="en"><![CDATA[Carcinoma]]></kwd>
<kwd lng="en"><![CDATA[Medullary]]></kwd>
<kwd lng="en"><![CDATA[Nanoparticles]]></kwd>
<kwd lng="en"><![CDATA[Pheochromocytoma]]></kwd>
<kwd lng="en"><![CDATA[Multiple Endocrine Neoplasia Type 2b]]></kwd>
<kwd lng="en"><![CDATA[Neuroma]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sipple]]></surname>
<given-names><![CDATA[JH]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The association of pheochromocytoma with carcinoma of the thyroid gland]]></article-title>
<source><![CDATA[The American journal of medicine]]></source>
<year>1961</year>
<volume>31</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>163-6</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Steiner]]></surname>
<given-names><![CDATA[AL]]></given-names>
</name>
<name>
<surname><![CDATA[Goodman]]></surname>
<given-names><![CDATA[AD]]></given-names>
</name>
<name>
<surname><![CDATA[Powers]]></surname>
<given-names><![CDATA[SR]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing&#8217;s disease: multiple endocrine neoplasia, type 2]]></article-title>
<source><![CDATA[Medicine (Baltimore)]]></source>
<year>1968</year>
<volume>47</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>371-409</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Williams]]></surname>
<given-names><![CDATA[ED]]></given-names>
</name>
<name>
<surname><![CDATA[Pollock]]></surname>
<given-names><![CDATA[DJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple mucosal neuroma with endocrine tumours: a syndrome allied to von Recklinghausen&#8217;s disease]]></article-title>
<source><![CDATA[J Pathol Bacteriol]]></source>
<year>1966</year>
<volume>91</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>71-80</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Chong]]></surname>
<given-names><![CDATA[GC]]></given-names>
</name>
<name>
<surname><![CDATA[Beahrs]]></surname>
<given-names><![CDATA[OH]]></given-names>
</name>
<name>
<surname><![CDATA[Sizemore]]></surname>
<given-names><![CDATA[GW]]></given-names>
</name>
<name>
<surname><![CDATA[Woolner]]></surname>
<given-names><![CDATA[LH]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Medullary carcinoma of the thyroid gland]]></article-title>
<source><![CDATA[Cancer]]></source>
<year>1975</year>
<volume>35</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>695-704</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Gagel]]></surname>
<given-names><![CDATA[RF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Genetic testing in endocrinology: lessons learned from experience with multiple endocrine neoplasia type 2 (MEN2)]]></article-title>
<source><![CDATA[Growth Horm IGF Res]]></source>
<year>2004</year>
<numero>14</numero>
<issue>14</issue>
<page-range>150-7</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Znaczko]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Donnelly]]></surname>
<given-names><![CDATA[DE]]></given-names>
</name>
<name>
<surname><![CDATA[Morrison]]></surname>
<given-names><![CDATA[PJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Epidemiology, clinical features, and genetics of multiple endocrine neoplasia type 2B in a complete population]]></article-title>
<source><![CDATA[Oncologist]]></source>
<year>2014</year>
<volume>19</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1284-6</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mathiesen]]></surname>
<given-names><![CDATA[JS]]></given-names>
</name>
<name>
<surname><![CDATA[Kroustrup]]></surname>
<given-names><![CDATA[JP]]></given-names>
</name>
<name>
<surname><![CDATA[Vestergaard]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Madsen]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Stochholm]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Poulsen]]></surname>
<given-names><![CDATA[PL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Incidence and prevalence of multiple endocrine neoplasia 2B in Denmark: a nationwide study]]></article-title>
<source><![CDATA[Endocr Relat Cancer]]></source>
<year>2017</year>
<volume>24</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>L39-42</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8.</label><nlm-citation citation-type="">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mathiesen]]></surname>
<given-names><![CDATA[JS]]></given-names>
</name>
<name>
<surname><![CDATA[Effraimidis]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Rossing]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Rasmussen]]></surname>
<given-names><![CDATA[ÅK]]></given-names>
</name>
<name>
<surname><![CDATA[Hoejberg]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Bastholt]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<source><![CDATA[Cancer Biol]]></source>
<year>2022</year>
<volume>79</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>163-79</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Takahashi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ritz]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Cooper]]></surname>
<given-names><![CDATA[GM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Activation of a novel human transforming gene, ret, by DNA rearrangement]]></article-title>
<source><![CDATA[Cell]]></source>
<year>1985</year>
<volume>42</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>581-8</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[McDonnell]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Gild]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
<name>
<surname><![CDATA[Clifton-Bligh]]></surname>
<given-names><![CDATA[RJ]]></given-names>
</name>
<name>
<surname><![CDATA[Robinson]]></surname>
<given-names><![CDATA[BG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia: an update]]></article-title>
<source><![CDATA[Intern Med J]]></source>
<year>2019</year>
<volume>49</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>954-61</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Carlson]]></surname>
<given-names><![CDATA[KM]]></given-names>
</name>
<name>
<surname><![CDATA[Dou]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Chi]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Scavarda]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Toshima]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Jackson]]></surname>
<given-names><![CDATA[CE]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[Proc Natl Acad Sci U S A]]></source>
<year>1994</year>
<volume>91</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>1579-83</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hu]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Guan]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Shi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Song]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[ZP]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A narrative review of multiple endocrine neoplasia syndromes: genetics, clinical features, imaging findings, and diagnosis]]></article-title>
<source><![CDATA[Ann Transl Med]]></source>
<year>2021</year>
<volume>9</volume>
<numero>11</numero>
<issue>11</issue>
</nlm-citation>
</ref>
<ref id="B13">
<label>13.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Machens]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Lorenz]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Bjøro]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Varhaug]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Dralle]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Surgical curability of medullary thyroid cancer in multiple endocrine neoplasia 2B: a changing perspective]]></article-title>
<source><![CDATA[Annals of surgery]]></source>
<year>2014</year>
<volume>259</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>800-6</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hansford]]></surname>
<given-names><![CDATA[JR]]></given-names>
</name>
<name>
<surname><![CDATA[Mulligan]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia type 2 and RET: from neoplasia to neurogenesis]]></article-title>
<source><![CDATA[Journal of Medical Genetics]]></source>
<year>2000</year>
<volume>37</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>817-27</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lairmore]]></surname>
<given-names><![CDATA[TC]]></given-names>
</name>
<name>
<surname><![CDATA[Howe]]></surname>
<given-names><![CDATA[JR]]></given-names>
</name>
<name>
<surname><![CDATA[Korte]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Dilley]]></surname>
<given-names><![CDATA[WG]]></given-names>
</name>
<name>
<surname><![CDATA[Aine]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Aine]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Familial medullary thyroid carcinoma and multiple endocrine neoplasia type 2B map to the same region of chromosome 10 as multiple endocrine neoplasia type 2A]]></article-title>
<source><![CDATA[Genomics]]></source>
<year>1991</year>
<volume>9</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>181-92</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hofstra RM]]></surname>
<given-names><![CDATA[Landsvater RM]]></given-names>
</name>
<name>
<surname><![CDATA[Ceccherini I]]></surname>
<given-names><![CDATA[Stulp RP]]></given-names>
</name>
<name>
<surname><![CDATA[Stelwagen T]]></surname>
<given-names><![CDATA[Luo]]></given-names>
</name>
<name>
<surname><![CDATA[Y]]></surname>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A mutation in the RET protooncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma]]></article-title>
<source><![CDATA[Nature]]></source>
<year>1994</year>
<volume>367</volume>
<numero>6461</numero>
<issue>6461</issue>
<page-range>375-6</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gujral]]></surname>
<given-names><![CDATA[TS]]></given-names>
</name>
<name>
<surname><![CDATA[Mulligan]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Molecular implications of RET mutations for pheochromocytoma risk in multiple endocrine neoplasia 2]]></article-title>
<source><![CDATA[Annals of the New York Academy of Sciences]]></source>
<year>2006</year>
<volume>1073</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>234-40</page-range></nlm-citation>
</ref>
<ref id="B18">
<label>18.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Plaza-Menacho]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Barnouin]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Goodman]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Martínez-Torres]]></surname>
<given-names><![CDATA[RJ]]></given-names>
</name>
<name>
<surname><![CDATA[Borg]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Murray-Rust]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Oncogenic RET kinase domain mutations perturb the autophosphorylation trajectory by enhancing substrate presentation in trans]]></article-title>
<source><![CDATA[Mol Cell]]></source>
<year>2014</year>
<volume>53</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>738-51</page-range></nlm-citation>
</ref>
<ref id="B19">
<label>19.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mathiesen]]></surname>
<given-names><![CDATA[JS]]></given-names>
</name>
<name>
<surname><![CDATA[Habra]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Bassett]]></surname>
<given-names><![CDATA[JHD]]></given-names>
</name>
<name>
<surname><![CDATA[Choudhury]]></surname>
<given-names><![CDATA[SM]]></given-names>
</name>
<name>
<surname><![CDATA[Balasubramanian]]></surname>
<given-names><![CDATA[SP]]></given-names>
</name>
<name>
<surname><![CDATA[Howlett]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Risk profile of the RET A883F germline mutation: an international collaborative study]]></article-title>
<source><![CDATA[J Clin Endocrinol Metab]]></source>
<year>2017</year>
<volume>102</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>2069-74</page-range></nlm-citation>
</ref>
<ref id="B20">
<label>20.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Salvatore D]]></surname>
<given-names><![CDATA[Santoro M]]></given-names>
</name>
<name>
<surname><![CDATA[Schlumberge]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The importance of the RET gene in thyroid cancer and therapeutic implications.]]></article-title>
<source><![CDATA[Nat Rev Endocrinol]]></source>
<year>2021</year>
<volume>17</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>296-306</page-range></nlm-citation>
</ref>
<ref id="B21">
<label>21.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Roman-Gonzalez]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Zapata]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
<name>
<surname><![CDATA[Mejia]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Inhibidores de la tirosina-cinasa en cáncer de tiroides]]></article-title>
<source><![CDATA[Revista Colombiana de Endocrinología, Diabetes &amp; Metabolismo]]></source>
<year>2021</year>
<volume>7</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>170-6</page-range></nlm-citation>
</ref>
<ref id="B22">
<label>22.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mise]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Drosten]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Racek]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Tannapfel]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Putzer]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Evaluation of potential mechanisms underlying genotype-phenotype correlations in multiple endocrine neoplasia type 2]]></article-title>
<source><![CDATA[Oncogene]]></source>
<year>2006</year>
<volume>25</volume>
<numero>50</numero>
<issue>50</issue>
</nlm-citation>
</ref>
<ref id="B23">
<label>23.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Eng]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The RET proto-oncogene in multiple endocrine neoplasia type 2 and Hirschsprung&#8217;s disease]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>1996</year>
<volume>335</volume>
<numero>13</numero>
<issue>13</issue>
<page-range>943-51</page-range></nlm-citation>
</ref>
<ref id="B24">
<label>24.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Oczko-Wojciechowska]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Swierniak]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Krajewska]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Kowalska]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Kowal]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Stokowy]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Differences in the transcriptome of medullary thyroid cancer regarding the status and type of RET gene mutations]]></article-title>
<source><![CDATA[Sci Rep]]></source>
<year>2017</year>
<volume>7</volume>
<numero>1</numero>
<issue>1</issue>
</nlm-citation>
</ref>
<ref id="B25">
<label>25.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Cadena]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[De Francisco]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Garavito]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Guevara]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neoplasia endocrina múltiple tipo 2: un ejemplo para la prevención del cáncer]]></article-title>
<source><![CDATA[Rev Col Cancerol]]></source>
<year>2005</year>
<volume>9</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>31-7</page-range></nlm-citation>
</ref>
<ref id="B26">
<label>26.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wells Jr]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Asa]]></surname>
<given-names><![CDATA[SL]]></given-names>
</name>
<name>
<surname><![CDATA[Dralle]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Elisei]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Evans]]></surname>
<given-names><![CDATA[DB]]></given-names>
</name>
<name>
<surname><![CDATA[Gagel]]></surname>
<given-names><![CDATA[RF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma: The American Thyroid Association Guidelines Task Force on medullary thyroid carcinoma]]></article-title>
<source><![CDATA[Thyroid]]></source>
<year>2015</year>
<volume>25</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>567-610</page-range></nlm-citation>
</ref>
<ref id="B27">
<label>27.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gomez]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Varghese]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Medullary thyroid carcinoma: molecular signaling pathways and emerging therapies]]></article-title>
<source><![CDATA[J Thyroid Res]]></source>
<year>2011</year>
</nlm-citation>
</ref>
<ref id="B28">
<label>28.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hu]]></surname>
<given-names><![CDATA[MI]]></given-names>
</name>
<name>
<surname><![CDATA[Ying]]></surname>
<given-names><![CDATA[AK]]></given-names>
</name>
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Update on medullary thyroid cancer]]></article-title>
<source><![CDATA[Endocrinol Metab Clin North Am]]></source>
<year>2014</year>
<volume>43</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>423-42</page-range></nlm-citation>
</ref>
<ref id="B29">
<label>29</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[O&#8217;Riordain]]></surname>
<given-names><![CDATA[DS]]></given-names>
</name>
<name>
<surname><![CDATA[O&#8217;Brien]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Weaver]]></surname>
<given-names><![CDATA[AL]]></given-names>
</name>
<name>
<surname><![CDATA[Gharib]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Hay]]></surname>
<given-names><![CDATA[ID]]></given-names>
</name>
<name>
<surname><![CDATA[Van Heerden]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Medullary thyroid carcinoma in multiple endocrine neoplasia types 2A and 2B]]></article-title>
<source><![CDATA[Surgery]]></source>
<year>1994</year>
<volume>116</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1017-23</page-range></nlm-citation>
</ref>
<ref id="B30">
<label>30.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Modigliani]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Cohen]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Campos]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Conte-Devolx]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Maes]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Boneu]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Prognostic factors for survival and for biochemical cure in medullary thyroid carcinoma: results in 899 patients]]></article-title>
<source><![CDATA[Clinical endocrinology]]></source>
<year>1998</year>
<volume>48</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>265-73</page-range></nlm-citation>
</ref>
<ref id="B31">
<label>31.</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Miller]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
<name>
<surname><![CDATA[Ellison]]></surname>
<given-names><![CDATA[EC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia type 2B]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Clark]]></surname>
<given-names><![CDATA[OH]]></given-names>
</name>
<name>
<surname><![CDATA[Duh]]></surname>
<given-names><![CDATA[QY]]></given-names>
</name>
</person-group>
<source><![CDATA[Textbook of Endocrine Surgery]]></source>
<year>1997</year>
<page-range>619-25</page-range><publisher-loc><![CDATA[Philadelphia ]]></publisher-loc>
<publisher-name><![CDATA[W.B. Saunders Company]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B32">
<label>32.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Roman-Gonzalez]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Mejia]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Zapata]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Nuevos tratamientos médicos para el cáncer medular de tiroides]]></article-title>
<source><![CDATA[Revista Colombiana de Endocrinología Diabetes &amp; Metabolismo]]></source>
<year>2019</year>
<volume>6</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>306-10</page-range></nlm-citation>
</ref>
<ref id="B33">
<label>33.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Moline]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Eng]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia type 2: an overview]]></article-title>
<source><![CDATA[Genet Med]]></source>
<year>2011</year>
<volume>13</volume>
<numero>9</numero>
<issue>9</issue>
</nlm-citation>
</ref>
<ref id="B34">
<label>34.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Cabanillas]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
<name>
<surname><![CDATA[Ryder]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Targeted therapy for advanced thyroid cancer: kinase inhibitors and beyond]]></article-title>
<source><![CDATA[Endocr Rev]]></source>
<year>2019</year>
<volume>40</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1573-604</page-range></nlm-citation>
</ref>
<ref id="B35">
<label>35.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Castinetti]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Moley]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Mulligan]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Waguespack]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A comprehensive review on MEN2B]]></article-title>
<source><![CDATA[Endocr Relat Cancer]]></source>
<year>2018</year>
<volume>25</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>T29-39</page-range></nlm-citation>
</ref>
<ref id="B36">
<label>36.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Castinetti]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Waguespack]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Machens]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Uchino]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Hasse-Lazar]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Sanso]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Natural history, treatment, and long-term follow up of patients with multiple endocrine neoplasia type 2B: an international, multicentre, retrospective study]]></article-title>
<source><![CDATA[Lancet Diabetes Endocrinol]]></source>
<year>2019</year>
<volume>7</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>213-20</page-range></nlm-citation>
</ref>
<ref id="B37">
<label>37.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wells-Jr]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Chi]]></surname>
<given-names><![CDATA[DD]]></given-names>
</name>
<name>
<surname><![CDATA[Toshima]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Dehner]]></surname>
<given-names><![CDATA[LP]]></given-names>
</name>
<name>
<surname><![CDATA[Coffin]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Dowton]]></surname>
<given-names><![CDATA[SB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Predictive DNA testing and prophylactic thyroidectomy in patients at risk for multiple endocrine neoplasia type 2A]]></article-title>
<source><![CDATA[Ann Surg]]></source>
<year>1994</year>
<volume>220</volume>
<numero>3</numero>
<issue>3</issue>
</nlm-citation>
</ref>
<ref id="B38">
<label>38.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Skinner]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Moley]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Dilley]]></surname>
<given-names><![CDATA[WG]]></given-names>
</name>
<name>
<surname><![CDATA[Owzar]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Debenedetti]]></surname>
<given-names><![CDATA[MK]]></given-names>
</name>
<name>
<surname><![CDATA[Wells-Jr]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>2005</year>
<volume>353</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>1105-13</page-range></nlm-citation>
</ref>
<ref id="B39">
<label>39.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Waguespack]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Rich]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Perrier]]></surname>
<given-names><![CDATA[ND]]></given-names>
</name>
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Cote]]></surname>
<given-names><![CDATA[GJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Management of medullary thyroid carcinoma and MEN2 syndromes in childhood]]></article-title>
<source><![CDATA[Nat Rev Endocrinol]]></source>
<year>2011</year>
<volume>7</volume>
<numero>10</numero>
<issue>10</issue>
</nlm-citation>
</ref>
<ref id="B40">
<label>40.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wray]]></surname>
<given-names><![CDATA[CJ]]></given-names>
</name>
<name>
<surname><![CDATA[Rich]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Waguespack]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Perrier]]></surname>
<given-names><![CDATA[ND]]></given-names>
</name>
<name>
<surname><![CDATA[Evans]]></surname>
<given-names><![CDATA[DB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Failure to recognize multiple endocrine neoplasia 2B: more common than we think?]]></article-title>
<source><![CDATA[Ann Surg Oncol]]></source>
<year>2008</year>
<volume>15</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>293-30</page-range></nlm-citation>
</ref>
<ref id="B41">
<label>41.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wells-Jr]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Robinson]]></surname>
<given-names><![CDATA[BG]]></given-names>
</name>
<name>
<surname><![CDATA[Gagel]]></surname>
<given-names><![CDATA[RF]]></given-names>
</name>
<name>
<surname><![CDATA[Dralle]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Fagin]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Santoro]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Vandetanib in patients with locally advanced or metastatic medullary thyroid cancer: a randomized, double-blind phase III trial]]></article-title>
<source><![CDATA[Journal of clinical oncology]]></source>
<year>2012</year>
<volume>30</volume>
<numero>2</numero>
<issue>2</issue>
</nlm-citation>
</ref>
<ref id="B42">
<label>42.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fox]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Widemann]]></surname>
<given-names><![CDATA[BC]]></given-names>
</name>
<name>
<surname><![CDATA[Chuk]]></surname>
<given-names><![CDATA[MK]]></given-names>
</name>
<name>
<surname><![CDATA[Marcus]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Aikin]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Balis]]></surname>
<given-names><![CDATA[FM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Vandetanib in children and adolescents with multiple endocrine neoplasia type 2B associated medullary thyroid carcinoma]]></article-title>
<source><![CDATA[Clin Cancer Res]]></source>
<year>2013</year>
<volume>19</volume>
<numero>15</numero>
<issue>15</issue>
<page-range>4239-48</page-range></nlm-citation>
</ref>
<ref id="B43">
<label>43.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Elisei]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Schlumberger]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
<name>
<surname><![CDATA[Muller]]></surname>
<given-names><![CDATA[SP]]></given-names>
</name>
<name>
<surname><![CDATA[Schoffski]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Brose]]></surname>
<given-names><![CDATA[MS]]></given-names>
</name>
<name>
<surname><![CDATA[Sherman]]></surname>
<given-names><![CDATA[SI]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Cabozantinib in progressive medullary thyroid cancer]]></article-title>
<source><![CDATA[J Clin Oncol]]></source>
<year>2013</year>
<volume>31</volume>
<numero>29</numero>
<issue>29</issue>
</nlm-citation>
</ref>
<ref id="B44">
<label>44.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sherman]]></surname>
<given-names><![CDATA[SI]]></given-names>
</name>
<name>
<surname><![CDATA[Clary]]></surname>
<given-names><![CDATA[DO]]></given-names>
</name>
<name>
<surname><![CDATA[Elisei]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Schlumberger]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
<name>
<surname><![CDATA[Cohen]]></surname>
<given-names><![CDATA[EE]]></given-names>
</name>
<name>
<surname><![CDATA[Schoffski]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Correlative analyses of RET and RAS mutations in a phase 3 trial of cabozantinib in patients with progressive, metastatic medullary thyroid cancer]]></article-title>
<source><![CDATA[Cancer]]></source>
<year>2016</year>
<volume>122</volume>
<numero>24</numero>
<issue>24</issue>
<page-range>3856-64</page-range></nlm-citation>
</ref>
<ref id="B45">
<label>45.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Moley]]></surname>
<given-names><![CDATA[JF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The molecular genetics of multiple endocrine neoplasia type 2A and related syndromes]]></article-title>
<source><![CDATA[Annu Rev Med]]></source>
<year>1997</year>
<volume>48</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>409-20</page-range></nlm-citation>
</ref>
<ref id="B46">
<label>46.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Thosani]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Ayala-Ramirez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Palmer]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Hu]]></surname>
<given-names><![CDATA[MI]]></given-names>
</name>
<name>
<surname><![CDATA[Rich]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Gagel]]></surname>
<given-names><![CDATA[RF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The characterization of pheochromocytoma and its impact on overall survival in multiple endocrine neoplasia type 2]]></article-title>
<source><![CDATA[J Clin Endocrinol Metab]]></source>
<year>2013</year>
<volume>98</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>E1813-9</page-range></nlm-citation>
</ref>
<ref id="B47">
<label>47.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brunt]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Doherty]]></surname>
<given-names><![CDATA[GM]]></given-names>
</name>
<name>
<surname><![CDATA[Norton]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Soper]]></surname>
<given-names><![CDATA[NJ]]></given-names>
</name>
<name>
<surname><![CDATA[Quasebarth]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Moley]]></surname>
<given-names><![CDATA[JF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Laparoscopic adrenalectomy compared to open adrenalectomy for benign adrenal neoplasms]]></article-title>
<source><![CDATA[J Am Coll Surg]]></source>
<year>1996</year>
<volume>183</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>1-10</page-range></nlm-citation>
</ref>
<ref id="B48">
<label>48.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Scholten]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Valk]]></surname>
<given-names><![CDATA[GD]]></given-names>
</name>
<name>
<surname><![CDATA[Ulfman]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Borel]]></surname>
<given-names><![CDATA[RI]]></given-names>
</name>
<name>
<surname><![CDATA[Vriens]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Unilateral subtotal adrenalectomy for pheochromocytoma in multiple endocrine neoplasia type 2 patients: a feasible surgical strategy]]></article-title>
<source><![CDATA[Ann Surg]]></source>
<year>2011</year>
<volume>254</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1022-7</page-range></nlm-citation>
</ref>
<ref id="B49">
<label>49.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Stock]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Stock]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Lorenz]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Sekulla]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Thanh]]></surname>
<given-names><![CDATA[PN]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Limitations of intraoperative adrenal remnant volume measurement in patients undergoing subtotal adrenalectomy]]></article-title>
<source><![CDATA[World J Surg]]></source>
<year>2008</year>
<volume>32</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>863-72</page-range></nlm-citation>
</ref>
<ref id="B50">
<label>50.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Dralle]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Funktionserhaltende Adrenalektomie bei Nebennierentumoren]]></article-title>
<source><![CDATA[Der Chirurg]]></source>
<year>2012</year>
<volume>83</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>519-27</page-range></nlm-citation>
</ref>
<ref id="B51">
<label>51.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lastra]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Franco]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Rueda]]></surname>
<given-names><![CDATA[PN]]></given-names>
</name>
<name>
<surname><![CDATA[Pradilla]]></surname>
<given-names><![CDATA[LP]]></given-names>
</name>
<name>
<surname><![CDATA[Paz]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neoplasia endocrina múltiple tipo-2B]]></article-title>
<source><![CDATA[Rev Fac Med]]></source>
<year>2004</year>
<volume>52</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>231-7</page-range></nlm-citation>
</ref>
<ref id="B52">
<label>52.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Dennehy]]></surname>
<given-names><![CDATA[PJ]]></given-names>
</name>
<name>
<surname><![CDATA[Feldman]]></surname>
<given-names><![CDATA[GL]]></given-names>
</name>
<name>
<surname><![CDATA[Kambouris]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[O&#8217;malley]]></surname>
<given-names><![CDATA[ER]]></given-names>
</name>
<name>
<surname><![CDATA[Sanders]]></surname>
<given-names><![CDATA[CY]]></given-names>
</name>
<name>
<surname><![CDATA[Jackson]]></surname>
<given-names><![CDATA[CE]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Relationship of familial prominent corneal nerves and lesions of the tongue resembling neuromas to multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[Am J Ophthalmol]]></source>
<year>1995</year>
<volume>120</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>456-61</page-range></nlm-citation>
</ref>
<ref id="B53">
<label>53.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Romeo]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Ceccherini]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Celli]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Priolo]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Betsos]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Bonardi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Association of multiple endocrine neoplasia type 2 and Hirschsprung disease]]></article-title>
<source><![CDATA[J Intern Med]]></source>
<year>1998</year>
<volume>243</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>515-20</page-range></nlm-citation>
</ref>
<ref id="B54">
<label>54.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Grobmyer]]></surname>
<given-names><![CDATA[SR]]></given-names>
</name>
<name>
<surname><![CDATA[Guillem]]></surname>
<given-names><![CDATA[JG]]></given-names>
</name>
<name>
<surname><![CDATA[O&#8217;Riordain]]></surname>
<given-names><![CDATA[DS]]></given-names>
</name>
<name>
<surname><![CDATA[Woodruff]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Shriver]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Brennan]]></surname>
<given-names><![CDATA[MF]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Colonic manifestations of multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[Dis Colon Rectum]]></source>
<year>1999</year>
<volume>42</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>1216-9</page-range></nlm-citation>
</ref>
<ref id="B55">
<label>55.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Smith]]></surname>
<given-names><![CDATA[VV]]></given-names>
</name>
<name>
<surname><![CDATA[Eng]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Milla]]></surname>
<given-names><![CDATA[PJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: implications for treatment]]></article-title>
<source><![CDATA[Gut]]></source>
<year>1999</year>
<volume>45</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>143-6</page-range></nlm-citation>
</ref>
<ref id="B56">
<label>56.</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lever]]></surname>
<given-names><![CDATA[WF]]></given-names>
</name>
<name>
<surname><![CDATA[Schaumburg-Lever]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<source><![CDATA[Histopathology of the Skin]]></source>
<year>1990</year>
<edition>7th</edition>
<publisher-name><![CDATA[Lippincott Williams and Wilkins]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B57">
<label>57.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yanoff]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Sharaby]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[Arch Ophthalmol]]></source>
<year>1996</year>
<volume>114</volume>
<numero>2</numero>
<issue>2</issue>
</nlm-citation>
</ref>
<ref id="B58">
<label>58.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Parker]]></surname>
<given-names><![CDATA[DG]]></given-names>
</name>
<name>
<surname><![CDATA[Robinson]]></surname>
<given-names><![CDATA[BG]]></given-names>
</name>
<name>
<surname><![CDATA[O&#8217;Donnell]]></surname>
<given-names><![CDATA[BA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[External ophthalmic findings in multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[Clin Exp Ophthalmol]]></source>
<year>2004</year>
<volume>32</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>420-3</page-range></nlm-citation>
</ref>
<ref id="B59">
<label>59.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Carney]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Go]]></surname>
<given-names><![CDATA[VL]]></given-names>
</name>
<name>
<surname><![CDATA[Sizemore]]></surname>
<given-names><![CDATA[GW]]></given-names>
</name>
<name>
<surname><![CDATA[Hayles]]></surname>
<given-names><![CDATA[AB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Alimentary-tract ganglioneuromatosis: a major component of the syndrome of multiple endocrine neoplasia, type 2b]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>1976</year>
<volume>295</volume>
<numero>23</numero>
<issue>23</issue>
<page-range>1287-91</page-range></nlm-citation>
</ref>
<ref id="B60">
<label>60.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Zhou]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Thosani]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Bisanz]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ayala-Ramirez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Roman-Gonzalez]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Jimenez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma]]></article-title>
<source><![CDATA[Endocrinology]]></source>
<year>2015</year>
<volume>173</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>377-87</page-range></nlm-citation>
</ref>
<ref id="B61">
<label>61.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Raue]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Zink]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical features of multiple endocrine neoplasia type 1 and type 2]]></article-title>
<source><![CDATA[Horm Res Paediatr]]></source>
<year>1992</year>
<volume>38</volume>
<numero>^sSuppl. 2</numero>
<issue>^sSuppl. 2</issue>
<supplement>Suppl. 2</supplement>
<page-range>31-5</page-range></nlm-citation>
</ref>
<ref id="B62">
<label>62.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Cohen]]></surname>
<given-names><![CDATA[MS]]></given-names>
</name>
<name>
<surname><![CDATA[Phay]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Albinson]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[DeBenedetti]]></surname>
<given-names><![CDATA[MK]]></given-names>
</name>
<name>
<surname><![CDATA[Skinner]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Lairmore]]></surname>
<given-names><![CDATA[TC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Gastrointestinal manifestations of multiple endocrine neoplasia type 2]]></article-title>
<source><![CDATA[Ann Surg]]></source>
<year>2002</year>
<volume>235</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>648-54</page-range></nlm-citation>
</ref>
<ref id="B63">
<label>63.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gibbons]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Camilleri]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Nelson]]></surname>
<given-names><![CDATA[AD]]></given-names>
</name>
<name>
<surname><![CDATA[Eckert]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Characteristics of chronic megacolon among patients diagnosed with multiple endocrine neoplasia type 2B]]></article-title>
<source><![CDATA[United European Gastroenterol J]]></source>
<year>2016</year>
<volume>4</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>449-54</page-range></nlm-citation>
</ref>
<ref id="B64">
<label>64.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Gimm]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Weiss]]></surname>
<given-names><![CDATA[CL]]></given-names>
</name>
<name>
<surname><![CDATA[Ukkat]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Sekulla]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Brauckhoff]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease]]></article-title>
<source><![CDATA[World J Surg]]></source>
<year>2004</year>
<volume>28</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1305-11</page-range></nlm-citation>
</ref>
<ref id="B65">
<label>65.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Martucciello]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Lerone]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Bricco]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Tonini]]></surname>
<given-names><![CDATA[GP]]></given-names>
</name>
<name>
<surname><![CDATA[Lombardi]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Del Rossi]]></surname>
<given-names><![CDATA[CG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasias type 2B and RET proto-oncogene]]></article-title>
<source><![CDATA[Ital J Pediatr]]></source>
<year>2012</year>
<volume>38</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>1-11</page-range></nlm-citation>
</ref>
<ref id="B66">
<label>66.</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[O&#8217;Riordain]]></surname>
<given-names><![CDATA[DS]]></given-names>
</name>
<name>
<surname><![CDATA[O&#8217;Brien]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Crotty]]></surname>
<given-names><![CDATA[TB]]></given-names>
</name>
<name>
<surname><![CDATA[Gharib]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Grant]]></surname>
<given-names><![CDATA[CS]]></given-names>
</name>
<name>
<surname><![CDATA[Van Heerden]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Multiple endocrine neoplasia type 2B: more than an endocrine disorder]]></article-title>
<source><![CDATA[Surgery]]></source>
<year>1995</year>
<volume>118</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>936-42</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
