在一个10岁男孩的支气管Sialadenoma Papilliferum
Shunhua Guo1, Alessandra Ceolin Schmitt2, James S Lewis2
1Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
International journal of surgical pathology
|October 3, 2024
概括
在一个男孩的支气管中发现了一种罕见的唾液瘤Sialadenoma papilliferum (SP). 这一案例凸显出需要将支气管SP与其他肺部瘤区分开来,以进行适当的患者管理.
科学领域:
- 肺部病理学 肺部病理学
- 在瘤学瘤学.
- 病理学 病理学 病理学
背景情况:
- 乳腺瘤 (SP) 是一种罕见的唾液腺瘤,通常在口腔中发现.
- 支气管中发生的SP异常罕见,这给诊断带来了挑战.
研究的目的:
- 报告一个小儿病人的支气管Sialadenoma papilliferum独特病例.
- 强调识别和区分支气管SP与其他肺部和唾液腺瘤的重要性.
主要方法:
- 支气管镜识别和切除多重型支气管病变.
- 组织病理学检查包括免疫组织化学 (SOX10,p63,BRAF V600E).
- 在MAML2和RET基因重排序中进行光在位杂交 (FISH).
主要成果:
- 一个支气管Sialadenoma papilliferum被诊断在一个10岁的男孩.
- 病变显示出具有异体和内体成分的乳头囊性特征,对SOX10和p63呈阳性,但对BRAF V600E.呈阴性.
- 通过FISH没有检测到MAML2或RET基因重组.
- 该患者经历了两次复发,需要切除袖子,但在治疗后24年内仍然没有疾病.
结论:
- 支气管Sialadenoma papilliferum是一种罕见的实体,需要在肺瘤的差分诊断中仔细考虑.
- 准确的病理和分子表征对于区分SP与其他瘤至关重要.
- 对于被诊断患有支气管SP的患者来说,长期随访是必不可少的,因为它有可能复发.
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