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The highly unusual macrofollicular variant of follicular thyroid carcinoma (MV-FTC) mirrors these attributes and is thereby misclassified by cytological examination of fine-needle aspiration biopsies. The MV-FTC diagnosis is instead suggested following histological investigation, in which malignant attributes, most commonly capsular invasion, are noted. The bulk of MV-FTCs described in the literature arise in younger female patients and carry an excellent prognosis. A recent coupling to mutations in the <jats:italic>DICER1<\/jats:italic> tumor suppressor gene has been proposed, possibly indicating aberrancies in micro-RNA (miRNA) patterns as responsible of the tumorigenic process. We describe the cytological, histological and molecular phenotype of a 35\u00a0mm large MV-FTC arising in the right thyroid lobe of a 33-year-old female with a family history of multinodular goiter. The tumor was encapsulated and strikingly inconspicuous in terms of cellularity and atypia, but nevertheless displayed multiple foci with capsular invasion. A next-generation molecular screening of tumor DNA revealed missense variants in <jats:italic>DICER1<\/jats:italic> (p. D1709N) and <jats:italic>MET<\/jats:italic> (p. T1010I), but no established fusion gene events. After sequencing of germline DNA, the <jats:italic>DICER1<\/jats:italic> mutation was confirmed as somatic, while the <jats:italic>MET<\/jats:italic> variant was constitutional. The patient is alive and well, currently awaiting radioiodine treatment. This MV-FTC mirrors previous publications, suggesting that these tumors carry a favorable prognosis and predominantly arise in younger females. Moreover, <jats:italic>DICER1<\/jats:italic> mutations should be considered a common driver event in the development of MV-FTCs.<\/jats:p>","DOI":"10.1007\/s12105-020-01208-1","type":"journal-article","created":{"date-parts":[[2020,7,25]],"date-time":"2020-07-25T18:02:23Z","timestamp":1595700143000},"page":"668-675","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Macrofollicular Variant of Follicular Thyroid Carcinoma (MV-FTC) with a Somatic DICER1 Gene Mutation: Case Report and Review of the Literature"],"prefix":"10.1007","volume":"15","author":[{"given":"L. Samuel","family":"Hellgren","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Hysek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenbugul","family":"Jatta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Zedenius","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5945-9081","authenticated-orcid":false,"given":"C. Christofer","family":"Juhlin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,25]]},"reference":[{"key":"1208_CR1","doi-asserted-by":"publisher","first-page":"1341","DOI":"10.1089\/thy.2017.0500","volume":"27","author":"ES Cibas","year":"2017","unstructured":"Cibas ES, Ali SZ. 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