Rare Tumors of the Thyroid Gland by José Manuel Cameselle-Teijeiro Catarina Eloy & Manuel Sobrinho-Simões

Author:José Manuel Cameselle-Teijeiro, Catarina Eloy & Manuel Sobrinho-Simões
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

Fig. 4.12Carcinoma of the thyroid with Ewing family tumour elements (CEFTE) . The small cell component is characterized by cells with scant cytoplasm, monotonous, roundish nuclei with low mitotic index and foci of necrosis (a). The small cell component coexists with PTC foci (disclosing a follicular organization and typical PTC-like nuclei) and squamoid nests (b, c)

Immunoreactivity for TTF-1, thyroglobulin, calcitonin , CK5, CK7, actin, chromogranin A , synaptophysin , NSE, GCDFP-15, HMB-45 and S100 protein was not detected. Later on, during the study of a second case with similar features, the expression CD99 , together with the presence of EWSR1/FLI1 rearrangement, was detected in this and in the previous case [14, 15]. Both patients are alive and well after more than 6 years of follow-up after being treated with total thyroidectomy and, in the second case, followed by radioactive iodine. In summary, CEFTE is a special type of small cell thyroid carcinoma with primary extraskeletal Ewing family tumour features, foci of papillary thyroid carcinoma and apparently good prognosis. There is another case of CEFTE with neuroendocrine differentiation (Fig. 4.13), a feature that can also be detected in primary extraskeletal Ewing family tumour (PEEFT).

Fig. 4.13Immunohistochemical features of a case of CEFTE. The small cells express CAM5.2 (a), p63 (b) and CD99 (c), while the PTC cells express CK7 (d), thyroglobulin (e) and TTF-1 (f)

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