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Mature and immature variants recapitulate their gonadal counterparts (predilection for infants\/children, triphasic germ layer differentiation, and favorable outcome). On the other hand, the so-called malignant teratomas affect predominantly adults and elderly, are highly aggressive, and, according to a few published cases, harbor<jats:italic>DICER1<\/jats:italic>mutations. We describe three highly aggressive sporadic malignant teratoid thyroid tumors in 2 females (17 and 45\u00a0years) and one male (17\u00a0years). Histology showed triphasic neoplasms composed of solid nests of small primitive monomorphic cells embedded in a cellular stroma with primitive immature rhabdomyosarcoma-like (2) or pleomorphic sarcoma-like (1) phenotype. The third component was represented by TTF1+\/PAX8+ primitive teratoid epithelial tubules reminiscent of primitive thyroid follicles and\/or Wilms tumor, admixed with scattered respiratory- or enteric-type tubules, neuroepithelial rosettes, and fetal-type squamoid nests. Foci of cartilage were seen in two cases, but none contained mature organoid adult-type tissue or skin adnexa. SALL4 was expressed in the small cell (2) and stromal (1) component. Other germ cell markers were negative. Molecular testing revealed a known \u201chotspot\u201d pathogenic<jats:italic>DICER1<\/jats:italic>mutation in two cases. In addition, case 1 had a missense<jats:italic>TP53<\/jats:italic>variant. This type of thyroid malignancy is distinct from genuine teratomas. The immunoprofile suggests primitive thyroid- or branchial cleft-like differentiation. Given that \u201cblastoma\u201d is a well-accepted terminology in the spectrum of DICER1-associated malignancies, the term \u201cthyroblastoma\u201d might be more convenient for these malignant teratoid tumors of the thyroid gland. Relationship of thyroblastoma to the DICER1 syndrome remains to be addressed.<\/jats:p>","DOI":"10.1007\/s00428-020-02853-1","type":"journal-article","created":{"date-parts":[[2020,6,7]],"date-time":"2020-06-07T05:02:18Z","timestamp":1591506138000},"page":"787-798","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":79,"title":["Malignant teratoid tumor of the thyroid gland: an aggressive primitive multiphenotypic malignancy showing organotypical elements and frequent DICER1 alterations\u2014is the term \u201cthyroblastoma\u201d more appropriate?"],"prefix":"10.1007","volume":"477","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0445-8161","authenticated-orcid":false,"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leora","family":"Witkowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph Christopher Castillo","family":"Cuenca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos Alberto","family":"Gonz\u00e1lez-Muller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfred","family":"Br\u00fctting","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"B\u00e4hrle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Mantsopoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randa M. 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