Nasopharyngeal Microcystic Clear Cell Adenoma in a Child: A Rare Case Report.

Ruba Mshref, Ahmad Alkheder, Mariana Naief Sharaf Aldeen, Alma Mshref, Reham Eid, Raouf Salem Seif Eddin
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Abstract

Adenoidectomy, routinely performed for pediatric obstructive symptoms, typically reveals benign lymphoid hyperplasia. We report the first documented case of a primary clear cell microcystic adenoma within adenoid tissue in a 12-year-old male undergoing adenotonsillectomy for chronic snoring, recurrent tonsillitis, and mouth breathing. Intraoperative examination identified an unusual homogenous grayish-white adenoid mass. Histopathological analysis demonstrated benign epithelial hyperplasia with focal clusters of clear cells arranged in microcystic patterns, morphologically aligning with a low-grade microcystic adenoma-a lesion classically linked to von Hippel-Lindau (VHL) syndrome. This case emphasizes the critical role of histopathological scrutiny in routine surgical specimens, as incidental findings may reveal rare neoplasms with significant syndromic implications. While the lesion exhibited benign features, its histological resemblance to VHL-driven neoplasms necessitates vigilant long-term surveillance. This report advocates for multidisciplinary collaboration in managing such cases and highlights the importance of genetic assessment to guide familial screening. Further documentation of similar cases is required to establish this entity's clinical and molecular profile, refining diagnostic approaches for nasopharyngeal clear cell lesions.

儿童鼻咽微囊性透明细胞腺瘤:罕见病例报告。
腺样体切除术是儿科阻塞性症状的常规治疗方法,通常会发现良性淋巴样增生。我们报告第一例有文献记载的腺样组织内原发性透明细胞微囊性腺瘤病例,患者为12岁男性,因慢性打鼾、复发性扁桃体炎和口腔呼吸而行腺扁桃体切除术。术中检查发现一个不寻常的均匀灰白色腺样肿块。组织病理学分析显示,良性上皮增生伴透明细胞团状排列成微囊状,形态学上与低级别微囊性腺瘤一致,这是一种典型的与von Hippel-Lindau (VHL)综合征相关的病变。本病例强调了常规手术标本中组织病理学检查的关键作用,因为偶然发现可能会揭示具有显著综合征含义的罕见肿瘤。虽然病变表现出良性特征,但其组织学上与vhl驱动的肿瘤相似,需要长期警惕监测。本报告提倡多学科合作来管理此类病例,并强调遗传评估对指导家族筛查的重要性。需要进一步记录类似病例,以建立该实体的临床和分子特征,完善鼻咽透明细胞病变的诊断方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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