考登综合征与口腔病变:MLPA 1例报告。

IF 1 Q3 MEDICINE, GENERAL & INTERNAL American Journal of Case Reports Pub Date : 2025-01-12 DOI:10.12659/AJCR.945876
Mariana Cristina Barrón-Márquez, Rogelio González-González, Lucina Bobadilla-Morales, Victor Ulises Rodriguez-Machuca, Ronell Bologna-Molina, Nelly Molina-Frechero, Omar Alejandro Tremillo-Maldonado, Sandra López-Verdín
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引用次数: 0

摘要

考登综合征是一种遗传疾病,使个体易患癌症,其特征是错构瘤起源于3胚层。虽然临床症状可能是病理特征,但诊断通常是通过活检、口腔和皮肤病变的组织病理学检查和遗传研究(包括多重结扎依赖性探针扩增(MLPA))来辅助的。病例报告:我们报告一例35岁的女性患者,其口腔黏膜、舌背和牙龈出现多发病变,面部皮肤和手背表面出现乳头状瘤状丘疹。这些病变被鉴定为错构瘤。实验室测试,包括血液生物测定、血液化学和凝血分析,结果在正常范围内。病史显示子宫肌瘤,怀疑为考登综合征。遗传咨询证实了诊断,显示PTEN杂合缺失。结论本病例说明了多学科方法在诊断考登综合征中的重要性,特别是牙科专业人员在识别早期临床症状方面的作用。由于患者患恶性肿瘤的风险较高,通过基因检测进行早期诊断至关重要。医疗保健提供者必须对诸如考登综合征之类的综合征保持警惕,特别是在有相关家族史的患者中,以确保及时干预和全面管理。
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Cowden Syndrome and Oral Lesions: A Case Report Using MLPA.

BACKGROUND Cowden syndrome is a genetic disorder that predisposes individuals to cancer and is characterized by hamartomas derived from 3 germ layers. Although the clinical signs can be pathognomonic, diagnosis is often aided by biopsies, histopathological examination of oral and cutaneous lesions, and genetic studies, including multiple ligation-dependent probe amplification (MLPA). CASE REPORT We report a case of a 35-year-old woman who manifested with multiple lesions in the buccal mucosa, dorsum of the tongue, and gums, along with papillomatous papules on her facial skin and the dorsal surfaces of her hands. These lesions were identified as hamartomas. Laboratory tests, including blood biometry, blood chemistry, and coagulation profiles, returned results within normal ranges. Her medical history revealed uterine fibroids, raising suspicion of Cowden syndrome. A genetic consultation confirmed the diagnosis, revealing a heterozygous PTEN deletion. CONCLUSIONS This case illustrates the importance of a multidisciplinary approach in diagnosing Cowden syndrome, especially the role of dental professionals in recognizing early clinical signs. Early diagnosis through genetic testing is crucial due to the patient's elevated risk of malignancies. Healthcare providers must remain vigilant to syndromes such as Cowden syndrome, particularly in patients with relevant family histories, to ensure timely intervention and comprehensive management.

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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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