Mao C, Guvendi M, Domenico D R, Kim K, Thomford N R, Howard J M
Department of Surgery, Mercy Hospital, Toledo, Ohio, USA.
Surgery. 1995 Nov;118(5):821-8. doi: 10.1016/s0039-6060(05)80271-5.
The papillary cystic and solid tumor of the pancreas is rare. It occurs predominantly in young women, and most present a benign behavior. The pathogenesis of this tumor has attracted a number of investigations but remains unclear.
We present three patients with this tumor and a review of 289 others from the world's literature, a total of 292 cases. On the basis of the analyses of the clinical and pathologic features from the reported cases, the pathogenesis of this unusual tumor has been further explored.
Ninety percent of the patients were female, with a mean age of 23.9 years. The tumors were usually quite large with a mean diameter of 10.3 cm. Ninety-two percent of these tumors were totally or partially cystic. Rupture of the capsule resulted in hemoperitoneum in eight cases, five of which were without any identifiable cause. Forty-three tumors (14.7%) have been recognized as malignant. The overall prognosis has been excellent and an aggressive approach to resection is indicated.
The results of immunohistochemical staining and electromicroscopy were rather diverse, but most, including the current cases, support the hypothesis that the tumor originates from pleuripotential embryonic stem cells. Thus the term pancreatic embryonic tumors seems preferable to papillary cystic and solid tumor of the pancreas to delineate the origin of the tumor and to reflect some of its biologic characteristics.
胰腺乳头状囊性实性肿瘤较为罕见。它主要发生于年轻女性，多数表现为良性行为。该肿瘤的发病机制已吸引了诸多研究，但仍不明确。
我们报告了3例该肿瘤患者，并对世界文献中的其他289例进行了回顾，共292例。基于对报告病例的临床和病理特征分析，进一步探讨了这种不寻常肿瘤的发病机制。
90%的患者为女性，平均年龄23.9岁。肿瘤通常较大，平均直径为10.3 cm。其中92%的肿瘤为完全或部分囊性。8例患者出现包膜破裂导致腹腔积血，其中5例原因不明。43例肿瘤（14.7%）被认定为恶性。总体预后良好，建议积极采取手术切除。
免疫组化染色和电镜检查结果差异较大，但多数结果，包括当前病例，支持该肿瘤起源于多能胚胎干细胞的假说。因此，胰腺胚胎性肿瘤这一术语似乎比胰腺乳头状囊性实性肿瘤更适合用来描述肿瘤的起源并反映其一些生物学特征。
