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 diffuse hyperplasia (Grave disease)
radiation thyroiditis
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)
- defective iodide transport diseases
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#