胰腺导管内乳头状黏液性肿瘤诊疗进展

Progress in Diagnosis and Treatment of Intraductal Papillary Mucinous Neoplasm of Pancreas

胰腺导管内乳头状黏液瘤(IPMN)是由胰腺导管内产生黏液的上皮细胞呈乳头状增殖形成的肿瘤。与经典胰腺癌相比,IPMN具有低度恶性、生长缓慢的特点。IPMN根据肿瘤累及的部位可分为主胰管型、分支胰管型和混合型。分支胰管型IPMN多为良性,主胰管型和混合胰管型IPMN的恶性可能性较大。IPMN临床表现多样且特异性差,多种影像学检查可显示弥漫性或节段性扩张的主胰管和囊状扩张的分支胰管,ERCP经扩大的乳头获取黏液和胰液,取胰腺导管内皮组织和壁结节活检有助于诊断。治疗根据肿瘤的性质采取不同方案。良性和交界性IPMN完整切除肿瘤即可,恶性IPMN一般需要根治性切除加淋巴结清扫术,对于无症状的分支胰管型,如无明显壁结节、细胞学检查阴性、囊肿小于30mm的可行临床观察。主胰管型和混合胰管型的IPMN则应全部手术切除。术中送冰冻病理确定手术范围,大多数手术患者采用胰十二指肠切除或胰体尾切除术,全胰切除仅占少数。IPMN手术切除率高,术后5年生存率高于一般的胰腺癌。本文就其临床表现、分类、病理特征、影像学诊断和治疗等方面进行综述。

Intraductal papillary mucinous neoplasm （IPMN） is a spectrum of neoplasia in the pancreatic duct epithelium, which originates from papillary proliferations of mucin-producing epithelial cells with excessive mucus production. Compared with conventional pancreatic carcinoma, IPMNs are characterized by low malignancy, slow progression, rarely invasion of surrounding structures, low rate of lymph nodes metastasis as well as recurrence. According to the site of involvement IPMNs are classified into three categories, i.e., main duct type, branch duct type, and mixed type. Most branch duct IPMNs are benign, whereas the other two types are often malignant. The clinic presentation of IPMN varies and non-specific. Often the diagnosis is incidental, multiple radiological approaches can display diffusive or segmental dilatation of main pancreatic duct and cystic dilatation of branch ducts. Through enlarged papilla, ERCP sampling of mucin, pancreatic juice, pancreatic duct wall and mural nodules for biopsy can help diagnosis. Treatment options for patients with IPMN range from observation to pancreatic resection depending on the natural history of the lesion. Complete resection is sufficient for benign and noninvasive malignant IPMNs. Malignant IPMNs acquiring aggressive treatment after parenchymal invasion necessitate adequate lymph node dissection. On the other hand, asymptomatic branch duct IPMNs, in the absence of symptoms, mural nodules, positive cytology, or cyst size less than 30 mm, can be observed without resection for a considerably long time. Main duct and mixed type IPMN carry a significant risk of malignancy, and surgery is recommended regardless of the presence of symptoms. When operation is indicated, targeted pancreatic resection with frozen-section analysis of margins is recommended. Pancreatoduodenectomy or distal pacreatectomy is appropriate for the majority. Only in a small number of patients is the disease so diffuse at presentation that total pancreatectomy is necessary. IPMNs have a high surgical resection rate and the prognosis is favorable. In this article, we reviewed the clinic presentation, classification, pathological features, radiological diagnosis, and treatment of IPMNs.