Laboratório de Endocrinologia Molecular e Translacional, Divisão de Endocrinologia, Universidade Federal de São Paulo, São Paulo, SP, Brasil.
Laboratório de Inovação Molecular e Biotecnologia, Divisão de Pós-Graduação Médica, Universidade Nove de Julho, São Paulo, SP, Brasil.
Arch Endocrinol Metab. 2022 Mar 8;66(1):112-117. doi: 10.20945/2359-3997000000439.
Thyroid cancer is the most common endocrine malignancy, and papillary thyroid carcinoma (PTC) is the main subtype. The cribriform morular variant is a histological phenotype of PTC characterized by its relationship with familial adenomatous polyposis (FAP). Description of the case: We report the genetic assessment of a 20-year-old female patient diagnosed with a cribriform-morular variant of PTC and FAP. We aimed to assess the genetic background of the reported patient, looking for variants that would help us explain the predisposition to tumorigenesis. Genomic DNA was extracted from peripheral blood lymphocytes, and whole exome sequencing was performed. We applied an overrepresentation and gene-set enrichment analysis to look for an accumulation of effects of variants in multiple genes at the genome. We found an overrepresentation of single nucleotide variants (SNVs) in extracellular matrix interactions and cell adhesion genes. Underrepresentation of SNVs in genes related to the regulation of autophagy and cell cycle control was also observed. We hypothesize that the package of alterations of our patient may help to explain why she presented colonic manifestations and thyroid cancer. Our findings suggest that multiple variants with minor impact, when considered together, may be helpful to characterize one particular clinical condition.
甲状腺癌是最常见的内分泌恶性肿瘤,而甲状腺乳头状癌(PTC)是主要亚型。筛状-微滤泡变异型是 PTC 的一种组织学表型,其特征是与家族性腺瘤性息肉病(FAP)有关。病例描述:我们报告了一名 20 岁女性患者的基因评估,该患者被诊断为 PTC 和 FAP 的筛状-微滤泡变异型。我们旨在评估报告患者的遗传背景,寻找有助于解释肿瘤发生易感性的变异。从外周血淋巴细胞中提取基因组 DNA,并进行全外显子组测序。我们应用过度表达和基因集富集分析来寻找基因组中多个基因中变异的累积效应。我们发现细胞外基质相互作用和细胞黏附基因中的单核苷酸变异(SNVs)过度表达。还观察到与自噬和细胞周期控制调节相关的基因中的 SNVs 表达不足。我们假设我们患者的改变组合可能有助于解释为什么她出现了结肠表现和甲状腺癌。我们的发现表明,当综合考虑时,多个具有较小影响的变体可能有助于描述特定的临床情况。