肺大细胞基底样腺癌:12例常被误认为大细胞神经内分泌癌的独特形式肺癌的临床病理学研究》(A Clinicopathologic Study of 12 Cases of a Distinctive Form of Lung Cancer Often Makes for Large-cell Neuroendocrine Carcinoma)。

IF 4.5 1区 医学 Q1 PATHOLOGY
David Suster, Haider A Mejbel, Alexander Craig Mackinnon, Saul Suster
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引用次数: 0

摘要

本文描述了一种近似大细胞神经内分泌癌的独特肺腺癌。肿瘤发生在 6 名女性和 6 名男性身上,年龄在 46-86 岁之间(平均=58.4 岁)。它们表现为周围胸膜下肿块,大小为 2-12 厘米(平均=6.5 厘米)。从组织学角度看,它们的特征是由大的非典型细胞组成的岛状、吻合状和绢丝状细胞串,显示出明显的核周围钙化、高有丝分裂活性和突出的粉瘤样坏死区。由于与神经内分泌肿瘤极为相似,其中一些病例最初被诊断为大细胞神经内分泌癌,尽管没有神经内分泌标记物。免疫组化结果显示,肿瘤细胞的TTF1和napsin-A呈阳性,p40呈阴性。肿瘤的多种神经内分泌标记物也一致呈阴性,包括嗜铬粒蛋白、突触素、CD56和INSM1。在 2 个病例中进行的电子显微镜检查显示,膜结合致密核心神经分泌颗粒呈阴性。在通过新一代测序检测的 8 例肿瘤中,有 5 例检测到致病性改变。在 5 例患者中发现了 KRAS 和 TP53 的点突变。2名患者存在GNAS、KIT和FGFR1的低水平扩增。未发现 RB1 基因突变。对10例患者的临床随访显示,2例患者死于肿瘤,2例患者出现远处转移,6例患者在确诊后1至13年(中位数=7.1年)生存良好。大细胞基底样腺癌是一种不常见的肺癌变异,很容易与大细胞神经内分泌癌混淆。认识这种不常见的肺腺癌变体对于治疗和预后以及避免误诊非常重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Large-cell Basaloid Adenocarcinoma of the Lung: A Clinicopathologic Study of 12 Cases of a Distinctive Form of Lung Cancer Often Mistaken for Large-cell Neuroendocrine Carcinoma.

A distinctive form of lung adenocarcinoma that closely mimics large-cell neuroendocrine carcinoma is described. The tumors arose in 6 women and 6 men aged 46-86 years (mean=58.4). They presented as peripheral subpleural masses measuring 2-12 cm (mean=6.5 cm). Histologically they were characterized by islands or anastomosing and serpiginous strands of large, atypical cells showing striking peripheral palisading of nuclei, with high mitotic activity and prominent comedo-like areas of necrosis. Because of the striking resemblance to neuroendocrine tumors, some of the cases were initially diagnosed as large-cell neuroendocrine carcinoma despite the absence of neuroendocrine markers. Immunohistochemistry showed positivity of the tumor cells for TTF1 and napsin-A, and negative staining for p40. The tumors were also uniformly negative for multiple neuroendocrine markers, including chromogranin, synaptophysin, CD56, and INSM1. Electron microscopy performed in 2 cases was negative for membrane-bound dense core neurosecretory granules. Pathogenic alterations were detected in 5 of 8 tumors tested by next-generation sequencing. Point mutations in KRAS and TP53 were identified in 5 patients. Low-level amplification of GNAS , KIT , and FGFR1 was present in 2 patients. No RB1 mutations were identified. Clinical follow-up in 10 cases showed that 2 patients died of their tumors, 2 experienced distant metastases, and 6 were alive and well from 1 to 13 years after diagnosis (median=7.1 y). Large-cell basaloid adenocarcinoma is an unusual variant of lung cancer that is easily confused with large-cell neuroendocrine carcinoma. Awareness of this unusual variant of lung adenocarcinoma is important for treatment and prognosis and for avoiding misdiagnosis.

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来源期刊
CiteScore
10.30
自引率
5.40%
发文量
295
审稿时长
1 months
期刊介绍: The American Journal of Surgical Pathology has achieved worldwide recognition for its outstanding coverage of the state of the art in human surgical pathology. In each monthly issue, experts present original articles, review articles, detailed case reports, and special features, enhanced by superb illustrations. Coverage encompasses technical methods, diagnostic aids, and frozen-section diagnosis, in addition to detailed pathologic studies of a wide range of disease entities. Official Journal of The Arthur Purdy Stout Society of Surgical Pathologists and The Gastrointestinal Pathology Society.
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