Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA, 90048, USA,
Endocr Pathol. 2015 Mar;26(1):54-62. doi: 10.1007/s12022-014-9346-3.
To compare the utility of PAX6 and PAX8 as immunohistochemical markers for neuroendocrine tumors (NETs) of pancreatic origin, we performed PAX6 and PAX8 immunostains on 178 NETs, including 110 primary NETs (26 pancreatic, 10 gastric, 12 duodenal, 22 jejuno-ileal, 10 rectal, 30 pulmonary) and 68 NETs metastatic to the liver (24 pancreatic, 1 duodenal, 37 jejuno-ileal, 1 rectal, 5 pulmonary). Among primary NETs, PAX6 and PAX8 were positive in 65 % (17/26) and 73 % (19/26) of pancreatic, 0 % (0/10) and 10 % (1/10) of gastric, 92 % (11/12) and 92 % (11/12) of duodenal, 0 % (0/22) and 0 % (0/22) of jejuno-ileal, 90 % (9/10) and 80 % (8/10) of rectal, and 0 % (0/30) and 23 % (7/30) of pulmonary NETs, respectively. PAX6 and PAX8 positivity was seen in 46 % (11/24) and 50 % (12/24) of metastatic pancreatic NETs to the liver, respectively. None of the nonpancreatic NETs metastatic to the liver were immunoreactive for either PAX6 or PAX8. PAX6 showed a slightly but statistically significant higher specificity for pancreatic NETs than did PAX8 (P = 0.039), while the sensitivities were similar (P = 0.51). PAX6 had the additional advantages over PAX8 of not exhibiting nonspecific cytoplasmic staining of tumor cells and only infrequently staining background lymphocytes. Since rectal NETs rarely present with metastatic disease, positive staining of a metastatic NET of unknown primary origin for PAX6 and/or PAX8 favors a pancreatic or duodenal origin. This information may be helpful in directing further diagnostic studies to identify the primary site of the metastatic tumor.
为了比较 PAX6 和 PAX8 作为胰腺来源神经内分泌肿瘤（NETs）免疫组化标志物的效用，我们对 178 例 NETs 进行了 PAX6 和 PAX8 免疫染色，包括 110 例原发 NETs（26 例胰腺 NETs、10 例胃 NETs、12 例十二指肠 NETs、22 例空肠-回肠 NETs、10 例直肠 NETs、30 例肺 NETs）和 68 例肝转移 NETs（24 例胰腺 NETs、1 例十二指肠 NETs、37 例空肠-回肠 NETs、1 例直肠 NETs、5 例肺 NETs）。在原发 NETs 中，PAX6 和 PAX8 在 65%（17/26）和 73%（19/26）的胰腺 NETs、0%（0/10）和 10%（1/10）的胃 NETs、92%（11/12）和 92%（11/12）的十二指肠 NETs、0%（0/22）和 0%（0/22）的空肠-回肠 NETs、90%（9/10）和 80%（8/10）的直肠 NETs以及 0%（0/30）和 23%（7/30）的肺 NETs中阳性，分别。在转移至肝脏的 46%（11/24）和 50%（12/24）的胰腺 NETs 中分别可见 PAX6 和 PAX8 阳性。没有一个转移到肝脏的非胰腺 NETs 对 PAX6 或 PAX8 免疫反应阳性。PAX6 对胰腺 NETs 的特异性略高于 PAX8（P=0.039），但敏感性相似（P=0.51）。与 PAX8 相比，PAX6 还有一个额外的优势，即肿瘤细胞的细胞质非特异性染色不明显，背景淋巴细胞染色也不常见。由于直肠 NETs 很少出现转移性疾病，对于未知原发部位的转移性 NETs，如果 PAX6 和/或 PAX8 染色阳性，则支持来源于胰腺或十二指肠。这些信息可能有助于指导进一步的诊断研究，以确定转移瘤的原发部位。
