Carcinoma of the Thyroid With Ewing Family Tumor Elements and Favorable Prognosis

Eloy, Catarina; Oliveira, Manuel Castanheira de; Vieira, Joana; Teixeira, Manuel R.; Cruz, João; Sobrinho-Simões, Manuel

doi:10.1177/1066896913486696

Cited by 26 publications

(36 citation statements)

References 21 publications

(72 reference statements)

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“…(10, 13, 14) Only recently similar cases were documented in the head and neck. (16–20) This study is the largest series (n=7) of adamantinoma-like EFT to date and focuses specifically on head and neck due to its challenging differential diagnoses encountered in these locations. Similar to classic EFT, head and neck adamantinoma-like EFT appears to generally affect young patients and may arise in a wide range of anatomic subsites including periorbital soft tissues, thyroid gland, parotid gland, and even mucosal sites like the sinonasal tract.…”

Section: Discussionmentioning

confidence: 99%

Adamantinoma-like Ewing Family Tumors of the Head and Neck

Bishop

Alaggio

Zhang

et al. 2015

American Journal of Surgical Pathology

141

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The Ewing sarcoma family of tumors (EFTs) of the head and neck are rare and may be difficult to diagnose as they display significant histologic overlap with other more common undifferentiated small blue round cell malignancies. Occasionally, EFTs may exhibit overt epithelial differentiation in the form of diffuse cytokeratin immunoexpression or squamous pearls, resembling the so-called adamantinoma-like EFTs and being challenging to distinguish from bona-fide carcinomas. Furthermore, the presence of EWSR1 gene rearrangement correlated with strong keratin expression may suggest a myoepithelial carcinoma. Herein we analyze a series of 7 adamantinoma-like EFTs of the head and neck, most of them being initially misdiagnosed as carcinomas due to their anatomic location and strong cytokeratin immunoexpression, and subsequently reclassified as EFT by molecular techniques. The tumors arose in the sinonasal tract (n=2), parotid gland (n=2), thyroid gland (n=2), and orbit (n=1), in patients ranging from 7 to 56 years (mean, 31). Microscopically they departed from the typical EFT morphology by growing as nests with peripheral nuclear palisading and prominent interlobular fibrosis, imparting a distinctly basaloid appearance. Moreover, two cases exhibited overt keratinization in the form of squamous pearls and one sinonasal tumor demonstrated areas of intraepithelial growth. All cases were positive for CD99, pan-cytokeratin, and p40. A subset of cases showed synaptophysin, S100 protein, and/or p16 reactivity, further confounding the diagnosis. FISH assays showed EWSR1 and FLI1 rearrangements in all cases. Our results reinforce that a subset of head and neck EFT may show strong cytokeratin expression or focal keratinization, and thus indistinguishable from more common true epithelial neoplasms. Thus CD99 should be included in the immunopanel of a round cell malignancy regardless of strong cytokeratin expression or anatomic location, and a strong and diffuse CD99 positivity should prompt molecular testing for the presence of EWSR1 gene rearrangements.

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Section: Discussionmentioning

confidence: 99%

Adamantinoma-like Ewing Family Tumors of the Head and Neck

Bishop

Alaggio

Zhang

et al. 2015

American Journal of Surgical Pathology

141

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“…Such entities have been recently described and discussed in the 2014 -AFIP book on Thyroid and Parathyroid Tumours (8) and will not be addressed in the present review. The only exception concerns the group of Small cell carcinomas/Carcinoma of the thyroid with Ewing Family Tumour Elements (CEFTE) because it represents a particularly interesting end result of new developments in the immunohistochemical and molecular study of peculiar thyroid tumour that raise difficult differential diagnostic problems (9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

“…The neoplastic cells of CEFTE diffusely express cytokeratins, p63, E-cadherin and CD99 in the absence of vimentin expression (9)(10)(11). In Table 3 we summarized the immunohistochemical features of CEFTE.…”

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confidence: 99%

Poorly differentiated and undifferentiated thyroid carcinomas

Eloy

Ferreira²,

Salgado³

et al. 2015

TJPATH

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Thyroid cancer is the most common endocrine malignancy and its incidence goes on increasing worldwide. The majority of thyroid tumours comprise well-differentiated (papillary and follicular) thyroid carcinomas that usually carry an excellent prognosis, while a minority progress to poorly differentiated carcinoma (PDTC) and, ultimately, to the highly aggressive and lethal undifferentiated carcinoma (UTC). Recently, some major advances have been made on the histologic and imunohistochemical identification, as well as on the molecular characterization of PDTC and UTC. In this review we summarize the most recent immunohistochemical and molecular findings in PDTC and UTC, giving a particular emphasis to the diagnostic and prognostic meaning of the genetic alterations.

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“…The two cases reported by Cruz et al [6], Eloy et al [7], and Eloy and Oliveira et al [8] had good outcomes, with the patients showing no evidence of disease at 38 months and 13 years, respectively. Ongkeko et al [9] reported a thyroid ALES that histologically showed a basaloid malignancy with focal peripheral palisading composed of nests of monotonous round cells with scant cytoplasm with areas of follicular colonization.…”

Section: Discussionmentioning

confidence: 98%

“…Cruz et al [6] and Eloy et al [7,8] initially reported two thyroid small round blue cell tumors that showed diffuse expression of p63, pankeratin and CD99 and harbored EWSR1-FLI1 fusions under the name "carcinoma of the thyroid with Ewing family tumor elements." Both of these cases showed essentially a small round blue cell tumor with scant cytoplasm growing in solid and insular patterns with epidermoid foci noted in one case that was initially diagnosed as a small cell carcinoma [6].…”

Section: Discussionmentioning

confidence: 99%

Adamantinoma-Like Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

Morlote

Harada

Lindeman

et al. 2019

Head and Neck Pathol

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Currently considered a variant of Ewing sarcoma, adamantinoma-like Ewing sarcoma is a rare malignancy that shows classic Ewing sarcoma-associated gene fusions but also epithelial differentiation. Here we present the 6th reported case of adamantinoma-like Ewing sarcoma involving the thyroid gland. Sections of the thyroid tumor from a 20-year old woman showed sheets, lobules and trabeculae of primitive, uniform, small round blue cells that diffusely expressed pankeratin, p40 and CD99. Fluorescent in situ hybridization revealed an EWSR1 gene rearrangement and an EWSR1-FLI1 fusion was detected by RT-PCR. Neck lymph nodes were not involved, and the patient was treated with a Ewing sarcoma chemotherapy protocol and radiation and is disease free 7 months after surgery. The unusual histology and immunohistochemical profile of adamantinoma-like Ewing sarcoma makes diagnosis and classification very challenging. We also present a literature review of adamantinoma-like Ewing sarcoma involving the thyroid.

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Carcinoma of the Thyroid With Ewing Family Tumor Elements and Favorable Prognosis

Cited by 26 publications

References 21 publications

Adamantinoma-like Ewing Family Tumors of the Head and Neck

Adamantinoma-like Ewing Family Tumors of the Head and Neck

Poorly differentiated and undifferentiated thyroid carcinomas

Adamantinoma-Like Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

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