胰腺黏液性非肿瘤性囊肿：黏蛋白表型将其与导管内乳头状黏液性肿瘤区分开来。
Mucinous nonneoplastic cyst of the pancreas: apomucin phenotype distinguishes this entity from intraductal papillary mucinous neoplasm.
机构信息
Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA.
出版信息
Hum Pathol. 2010 Apr;41(4):513-21. doi: 10.1016/j.humpath.2009.05.017. Epub 2009 Dec 1.
Mucinous nonneoplastic cyst of the pancreas is a newly described and rare cystic lesion with unknown histogenesis. It is defined as a cystic lesion lined with mucinous epithelium, supported by hypocellular stroma and not communicating with the pancreatic ducts. It is very challenging to differentiate this lesion from other cystic mucinous neoplasms of the pancreas such as branch-duct intraductal papillary mucinous neoplasm by morphology. In this study, a total of 436 pancreatic specimens resected between 2002 and 2007 in our institution were reviewed. Fifteen (3.4%, 15/436) mucinous nonneoplastic cysts were identified. They included 3 males and 12 females, with a median age of 60 years. Forty-six percent of cases (7/15) occurred in pancreatic head, 27% (4/15) in neck, 7% (1/15) in body, and 20% (3/15) in tail. The size of lesions ranged from 0.5 to 3.5 cm in greatest dimension. In most cases (12/15, 80%), mucinous nonneoplastic cyst was associated or adjacent to acinar-ductal mucinous metaplasia. These morphologic data indicate that mucinous nonneoplastic cyst is not really a rare disease and may originate from acinar-duct mucinous metaplasia histogenestically. Furthermore, apomucin immunostains of mucinous nonneoplastic cyst showed MUC1 expressed in 27% (4/15) cases, MUC5AC in 67% (10/15 cases), and MUC2 was were negative in all cases, whereas intraductal papillary mucinous neoplasm (n = 17; 5 main duct type, 12 branch-duct type) showed focal and weak MUC1 positivity in 18% (3/17) cases, MUC2 positivity in 71% (12/17) cases, and all intraductal papillary mucinous neoplasm (17/17) were MUC5AC positive. The clonality assay with the HUMARA gene revealed that the mucinous nonneoplastic cysts were of polyclonal origin. For the first time, using HUMARA assay, we demonstrate the nonneoplastic nature of these cysts and further characterize morphologic and immunophenotypic properties that allow differentiation from intraductal papillary mucinous neooplasm.
胰腺黏液性非肿瘤性囊肿是一种新描述的罕见囊性病变，其组织发生尚不清楚。它被定义为一种囊性病变，内衬黏液性上皮，由细胞稀疏的基质支撑，与胰腺导管不通。通过形态学很难将这种病变与其他胰腺囊性黏液性肿瘤（如分支胰管内乳头状黏液性肿瘤）区分开来。在这项研究中，我们回顾了 2002 年至 2007 年间在我们机构切除的 436 份胰腺标本，发现了 15 例（3.4%，15/436）黏液性非肿瘤性囊肿。其中包括 3 名男性和 12 名女性，中位年龄为 60 岁。46%的病例（7/15）发生在胰头部，27%（4/15）发生在胰颈部，7%（1/15）发生在胰体部，20%（3/15）发生在胰尾部。病变的最大直径范围为 0.5 至 3.5 厘米。在大多数情况下（12/15，80%），黏液性非肿瘤性囊肿与腺管黏液性化生相关或相邻。这些形态学数据表明，黏液性非肿瘤性囊肿并不是一种罕见疾病，可能起源于腺管黏液性化生。此外，黏液性非肿瘤性囊肿的 apomucin 免疫染色显示 MUC1 在 27%（4/15）的病例中表达，MUC5AC 在 67%（10/15）的病例中表达，而 MUC2 在所有病例中均为阴性，而胰腺导管内乳头状黏液性肿瘤（n=17；5 例主胰管型，12 例分支胰管型）在 18%（3/17）的病例中显示局灶性和弱阳性 MUC1 阳性，71%（12/17）的病例中 MUC2 阳性，所有胰腺导管内乳头状黏液性肿瘤（17/17）均 MUC5AC 阳性。HUMARA 基因的克隆性检测显示，黏液性非肿瘤性囊肿为多克隆起源。首次使用 HUMARA 检测，我们证明了这些囊肿的非肿瘤性质，并进一步描述了其形态学和免疫表型特征，使其能够与胰腺导管内乳头状黏液性肿瘤相区分。
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