Yang Chen, Gonzalez Ivan, Zhang Lingxin, Cao Dengfeng
Department of Pathology, Yale School of Medicine, New Haven, CT 06510, USA.
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Gastroenterology Res. 2019 Jun;12(3):120-127. doi: 10.14740/gr1138. Epub 2019 Jun 7.
Adenocarcinoma ex-goblet cell carcinoid (AdexGCC) was considered a neuroendocrine adenocarcinoma, despite majority of tumor cells being negative for conventional neuroendocrine markers such as chromogranin and synaptophysin. Recently, insulinoma-associated protein 1 (INSM1) has been identified as a novel neuroendocrine marker that is more sensitive than chromogranin, synaptophysin, and CD56 in pulmonary neuroendocrine tumors.
We studied this marker in conjunction with chromogranin, synaptophysin, and CD56 in 36 appendiceal AdexGCCs (21 primaries, 15 metastatic).
Primary AdexGCCs showed staining for INSM1, chromogranin, synaptophysin, and CD56 in 13/21 (62%), 18/21 (86%), 18/21 (86%), and 9/19 (47%) cases, respectively. However, the mean proportion of tumor cells stained for INSM1, chromogranin, synaptophysin, and CD56 was only 8.0% (median 1%, range 0-70%), 15.7% (median 2%, range 0-70%), 19.9% (median 5%, range 0-90%), and 5.6% (median 0%, range 0-50%), respectively. Metastatic AdexGCCs showed staining for INSM1, chromogranin, synaptophysin, and CD56 in 8/15 (53%), 11/15 (73%), 12/15 (80%), and 3/14 (21%) cases. The mean proportion of tumor cells stained for INSM1, chromogranin, synaptophysin, and CD56 in metastatic tumors was 1% (median 1%, range 0-3%), 12% (median 1%, range 0-85%), 17% (median 5%, range 0-85%), and 2% (median 0%, range 0-20%), respectively.
Primary and metastatic AdexGCCs showed no difference in INSM1, chromogranin, synaptophysin, or CD56 staining. INSM1 exhibits low expression in AdexGCCs and is expressed by a lower proportion of tumor cells compared to chromogranin and synaptophysin.
杯状细胞类癌腺癌（AdexGCC）尽管大多数肿瘤细胞对嗜铬粒蛋白和突触素等传统神经内分泌标志物呈阴性，但仍被视为神经内分泌腺癌。最近，胰岛素瘤相关蛋白1（INSM1）已被确定为一种新型神经内分泌标志物，在肺神经内分泌肿瘤中比嗜铬粒蛋白、突触素和CD56更敏感。
我们在36例阑尾AdexGCC（21例原发性，15例转移性）中研究了该标志物与嗜铬粒蛋白、突触素和CD56。
原发性AdexGCC中，INSM1、嗜铬粒蛋白、突触素和CD56染色阳性的病例分别为13/21（62%）、18/21（86%）、18/21（86%）和9/19（47%）。然而，INSM1、嗜铬粒蛋白、突触素和CD56染色的肿瘤细胞平均比例分别仅为8.0%（中位数1%，范围0 - 70%）、15.7%（中位数2%，范围0 - 70%）、19.9%（中位数5%，范围0 - 90%）和5.6%（中位数0%，范围0 - 50%）。转移性AdexGCC中，INSM1、嗜铬粒蛋白、突触素和CD56染色阳性的病例分别为8/15（53%）、11/15（73%）、12/15（80%）和3/14（21%）。转移性肿瘤中INSM1、嗜铬粒蛋白、突触素和CD56染色的肿瘤细胞平均比例分别为1%（中位数1%，范围0 - 3%）、12%（中位数1%，范围0 - 85%）、17%（中位数5%，范围0 - 85%）和2%（中位数0%，范围0 - 20%）。
原发性和转移性AdexGCC在INSM1、嗜铬粒蛋白、突触素或CD56染色方面无差异。INSM1在AdexGCC中表达较低，与嗜铬粒蛋白和突触素相比，其表达的肿瘤细胞比例更低。
