胰腺导管内乳头状黏液肿瘤19例的病理学分析

目的 总结并分析胰腺导管内乳头状黏液肿瘤(IPMN)临床病理特征.方法 收集19例IPMN的临床及影像学资料,通过光镜观察及免疫组织化学EnVision法抗体包括p53、c-erbB-2、Ki-67、p16、Fascin]分析其临床、病理学特征及免疫表型.结果 19例IPMN患者平均年龄59岁,中位年龄61岁,男女比例12:7,6例有长期吸烟史,1例合并结肠腺癌,临床表现为上腹部不适、腹痛、食欲减退、体重下降、脂肪泻等.腹部CT及B超检查多数病例表现为囊性占位性病变,囊内有分隔,囊壁有乳头状回声,个别病例表现为单发或多发实性占位,其中3例行十二指肠镜逆行胰管造影(ERCP)检查见十二指肠乳头处有胶冻样黏液分泌物.18例行肿物及部分胰腺切除术,1例行胰管内肿瘤摘除术,11例主要位于胰头,病理诊断1例为胰腺导管内乳头状黏液腺瘤,3例为交界性IPMN,15例为胰腺导管内乳头状黏液腺癌,其中12例伴有浸润,浸润癌中10例为乳头型和(或)管型,2例为黏液型合并管型,2例为原位癌.大体检杳17例为单发,2例为多发病灶,6例为实性占位,13例表现为囊性或囊实性占位,囊性区部分内肇光滑剑有乳头附着,部分囊腔内充满乳头状组织;镜下检查8例为肠型,7例为胰胆管上皮型,3例为胃型,1例为嗜酸细胞型;导管周围纤维组织均明显增生,16例周围胰腺组织伴有慢性胰腺炎,2例肠壁浸润,淋巴结均未发现转移;术后13例分别随访4～48个月,平均随访20个月,1例术后24个月死于其他原因,1例可疑肝转移,1例姑息手术后胰头占位,10例无复发.免疫组织化学6例p53阳性,5例p16阳性,8例Fascin阳性,c-erbB-2均阴性,Ki-67指数1%～80%,平均38%.结论 IPMN为一组少见的胰腺肿瘤,肿瘤主要位于胰管内,伴有胰管扩张,肿瘤细胞为黏液性,有乳头形成,乳头状黏液腺癌多见,约2/3病例伴有浸润.Ki-67指数超过15%应考虑恶性.肿瘤预后较胰腺导管癌明显要好,治疗主要是局部切除及相应化疗,应长期随访.影像学、ERCP及胰液细胞学检查有助于疾病的早期发现和诊断.

Intraductal papillary mucinous neoplasm of pancreas: a clinicopathologic and immunohistochemical study of 19 cases

Objective To study the clinicopathologic features and diagnosis of intraductal papillary mucinous neoplasm (1PMN) of the pancreas. Methods Nineteen cases of IPMN encountered during the period from 1999 to 2007 were studied by light microscopy and immunohistochemiatry. Results IPMN occurred more often in males (68%). It affected patients in older age group (mean age=59) and was located mainly in the head of pancreas (60%). The mean tumor size was 4.2cm (range=1 to 8 cm). The clinical presentation was epigastric pain (53% ), weight loss (32% ), diabetes (21%), pancreatitis (21%) and jaundice (10%). Pancreatectomy was performed in 18 cases. The remaining patient received bypass surgery only. Features of in-situ or invasive malignancy were present in 15 of the 19 cases (78%). Histologically, the tumor consisted of papillary proliferations protruding into and expanding the pancreatic ducts. Invasion into the surrounding pancreatic parenchyma was noted in 12 cases and chronic pancreatitis was present in 16 eases. Follow-up data (4 to 48 months) were available in 13 patients. Apart from 1 patient who died of other disease, all were still alive. Immunohistochemical study showed that p53 was positive in 6 cases, p16 in 5 cases and fascin in 8 cases. The expression of c-erbB-2 was all negative. Ki-67 index ranged from 1% to 80% (mean=38% ). Conclusions Malignant changes are not uncommon in IPMN. The prognosis after surgical resection however is better than that of conventional pancreatic adenocarcinoma. The overexpression of p53, p16 and fascin may be related to tumor progression. The possibility of malignant transformation needs to be considered if the Ki-67 index is over 15%. Early recognition by radiologic examination (including ERCP) and pancreatic cytology would be helpful in early diagnosis. Surgical resection represents the mainstay of treatment and long-term post-operative follow up is needed.