dyshormonogenetic goiter
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Definition: Thyroid lesions in thyroid dyshormonogenesis in inborm errors of thyroid metabolism. Dyshormonogenetic goiters (DG) are genetically determined thyroid hyperplasias due to enzyme defects in thyroid-hormone synthesis.
Synopsis
enlarged thyroid (up to 600g)
multinodular thyroid
hypercellular thyroid nodules
foci of thyroid cystic degeneration
thyroid hemorrhages
myxoid changes
architectural patterns
- solid growth pattern
- trabecular growth pattern
- microfollicular growth pattern
- papillary growth pattern
- insular growth pattern
- microfollicles or macrofollicles
thyroid fibrosis
- irregularities at the edge of the nodules simulating capsular invasion
nuclear anomalies
- nuclear atypia
- large, irregularly shaped hyperchromatic nuclei
- vesicular nuclei
minimal amount of colloid
papilary hyperplasia
follicular hyperplasia
cellular atypia
Differential diagnosis
antithyroidal agents iatrogenic goiter
thyroid follicular carcinoma
- thyroid papillary carcinoma
- thyroid medullary carcinoma
- thryoid undifferentiated carcinoma
Etiology
thyroid dyshormonogenesis
- iodine transport defetcs
- defective organification of iodine
- iodotyrosine coupling defect
- iodotyrosine dehalogenase defect
- abnormal secretion of iodoproteins
Associations
thyroid papillary microcarcinoma
thyroid papillary carcinoma (3450511)
See also
thyroid metabolism diseases (inherited disorders of thyroid metabolism)
References
Ghossein RA, Rosai J, Heffess C. Related Articles, Links Dyshormonogenetic Goiter: A Clinicopathologic Study of 56 Cases. Endocr Pathol. 1997 Winter;8(4):283-292. PMID: 12114789
Camargo RY, Gross JL, Silveiro SP, Knobel M, Medeiros-Neto G. Pathological Findings in Dyshormonogenetic Goiter with Defective Iodide Transport. Endocr Pathol. 1998 Autumn;9(3):225-233. PMID: 12114713