Department of Pathology, The First College of Clinical Medical Science, China Three Gorges University, Yichang, 443000, China.
Department of Pathology, Yichang Central People's Hospital, Yichang, 443100, China.
J Cardiothorac Surg. 2024 Jun 20;19(1):336. doi: 10.1186/s13019-024-02852-2.
Pulmonary papillary adenoma is an extremely rare benign tumor. It is derived from type II lung cells and club cells, suggesting that it may originate from stem cells with two-way differentiation. Only one case has been reported with FGFR2-IIIb overexpression.
Two cases of pulmonary papillary adenoma with available data on clinical features, histological morphology, immunophenotype and molecular characteristics were analyzed.
Both tumors were well-circumscribed unencapsulated nodules composed of papillary structures with fibrovascular cores lined by a single layer of cuboidal or columnar epithelium without necrosis, nuclear atypia and mitoses, or invasion. But malignant transformation features include complex branching structures and significantly enlarged, irregular, and crowded malignant cells in one case. Immunohistochemistry showed that the tumor cells were strongly positive for TTF1, NapsinA, EMA and CK7 and negative for CEA and P63, with a low Ki-67 proliferation index. The EGFR somatic mutation exon19:c.2236_2256delinsATC (p.E746_S752delinsI) was found in one case by next-generation sequencing (NGS) technology.
Pulmonary papillary adenoma is very rare. Virtually all papillary adenomas are clinically silent and discovered incidentally. They are benign tumors, and resection is curative. An EGFR 19 exon deletion mutation in a patient with this tumor type was detected for the first time by NGS, and our results suggest that the malignant transformation of pulmonary papillary adenoma may be mediated by EGFR mutation.
肺乳头状腺瘤是一种极为罕见的良性肿瘤。它来源于Ⅱ型肺泡细胞和 club 细胞,提示其可能起源于具有双向分化潜能的干细胞。仅有一例报道存在 FGFR2-IIIb 过表达。
分析两例具有临床特征、组织形态学、免疫表型和分子特征等相关数据的肺乳头状腺瘤病例。
两例肿瘤均为边界清楚的无包膜结节,由纤维血管核心构成的乳头状结构组成,被覆单层立方或柱状上皮,无坏死、核异型性和有丝分裂,或浸润。但恶性转化特征包括在一例中出现复杂的分支结构和显著增大、形态不规则且密集的恶性细胞。免疫组化显示肿瘤细胞强烈表达 TTF1、NapsinA、EMA 和 CK7,而 CEA 和 P63 阴性,Ki-67 增殖指数低。一例通过下一代测序(NGS)技术发现 EGFR 外显子 19:c.2236_2256delinsATC(p.E746_S752delinsI)的体细胞突变。
肺乳头状腺瘤非常罕见。几乎所有的乳头状腺瘤均为临床无症状偶然发现。它们是良性肿瘤,切除可治愈。首例患者检测到该肿瘤类型存在 EGFR 19 外显子缺失突变,我们的结果提示肺乳头状腺瘤的恶性转化可能与 EGFR 突变有关。