Rivera J A, Fernández-del Castillo C, Pins M, Compton C C, Lewandrowski K B, Rattner D W, Warshaw A L
Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, USA.
Ann Surg. 1997 Jun;225(6):637-44; discussion 644-6. doi: 10.1097/00000658-199706000-00001.
The purpose of the study is to review a single institutional experience with mucinous ductal ectasia (MDE) and intraductal papillary neoplasms (IPNs) and to compare the clinicopathologic features of the two groups of tumors.
Mucinous ductal ectasia and IPNs represent newly recognized categories of pancreatic exocrine tumors, previously confused with pancreatic cystic neoplasms. The natural history of MDE and IPN is not well understood, and it is unclear whether MDE and IPN represent two distinct tumors or the same clinicopathologic entity.
The authors reviewed the clinical presentation, treatment, histopathology, and outcomes of 23 patients diagnosed with MDE or IPN at their institution over the past 6 years.
The mean age at presentation for the cohort of patients with MDE and IPN was 62.5 years. The prevalence of abdominal pain was 75%, jaundice 25%, weight loss 42%, steatorrhea 37.5%, diabetes 37.5%, and history of pencreatitis 29%. Serum CA 19-9 levels ranged from 0 to 5350 units/mL with high levels reflecting advanced disease. There were no significant differences between MDE and IPN with respect to these parameters. Both MDE and IPN comprised papillary villous epithelial neoplasms involving the main and large pancreatic ducts. The tumors ranged from a few millimeters in size to panductal and were distinguished easily from cystic neoplasms in all cases. Invasive carcinoma was present in 11 (46%) of 24 patients, carcinoma in situ in an additional 10 (42%) of 24 patients, and low grade dysplasia in the remaining 3 (12%) of 24 patients. Mucinous ductal ectasia and IPN differed histopathologically only in degree of mucin secretion and tumor location. Mucinous ductal ectasia, but not IPN, was characteristically mucin-hypersecreting and more frequently involved the head of the gland than did IPN (11/16 vs. 1/8 p < 0.04). All patients were explored surgically and 20 (83%) of 24 of the tumors were resectable with frozen section control of the duct margins (9 pancreatoduodenectomies, 4 distal pencreatectomies, 7 total pancreatectomies). Despite the 88% prevalence of cancer, the overall survival at a mean follow-up of 21 months was 13 (87%) of 15 for MDE and 5 (71%) of 7 for IPN.
Intraductal papillary neoplasms with or without MDE represent a spectrum of main duct papillary tumors ranging from adenoma to carcinoma with differing amounts of extracellular mucin production. Malignant IPNs with or without MDE typically exhibit extensive intraductal growth but are slow to invade the periductal tissues and slow to metastasize. The majority of patients with these tumors have resectable disease and a favorable prognosis; endoscopic therapy is inappropriate. The encompessing term intraductal papillary-mucinous tumors is appropriate.
本研究旨在回顾单一机构中黏液性导管扩张症（MDE）和导管内乳头状肿瘤（IPN）的经验，并比较两组肿瘤的临床病理特征。
黏液性导管扩张症和IPN是新认识的胰腺外分泌肿瘤类别，以前与胰腺囊性肿瘤混淆。MDE和IPN的自然病史尚不清楚，MDE和IPN是代表两种不同的肿瘤还是同一临床病理实体也不清楚。
作者回顾了过去6年在其机构诊断为MDE或IPN的23例患者的临床表现、治疗、组织病理学和预后。
MDE和IPN患者队列的平均就诊年龄为62.5岁。腹痛发生率为75%，黄疸25%，体重减轻42%，脂肪泻37.5%，糖尿病37.5%，胰腺炎病史29%。血清CA 19-9水平范围为0至5350单位/毫升，高水平反映疾病进展。在这些参数方面，MDE和IPN之间无显著差异。MDE和IPN均包括累及主胰管和大胰管的乳头状绒毛状上皮肿瘤。肿瘤大小从几毫米到累及全胰管不等，在所有病例中均易于与囊性肿瘤区分。24例患者中有11例（46%）存在浸润性癌，另外24例患者中有10例（42%）存在原位癌，其余24例患者中有3例（12%）存在低级别发育异常。黏液性导管扩张症和IPN在组织病理学上仅在黏液分泌程度和肿瘤位置方面有所不同。黏液性导管扩张症的特征是黏液分泌过多，比IPN更常累及胰头（11/16比1/8，p<0.04）。所有患者均接受手术探查，24例肿瘤中有20例（83%）可切除，术中通过冰冻切片控制切缘（9例行胰十二指肠切除术，4例行远端胰腺切除术，7例行全胰切除术）。尽管癌症发生率为88%，但平均随访21个月时，MDE组15例患者中有13例（87%）存活，IPN组7例患者中有5例（71%）存活。
伴有或不伴有MDE的导管内乳头状肿瘤代表了一系列主胰管乳头状肿瘤，范围从腺瘤到癌，细胞外黏液产生量不同。伴有或不伴有MDE的恶性IPN通常表现为广泛的导管内生长，但侵犯导管周围组织缓慢且转移缓慢。这些肿瘤的大多数患者疾病可切除且预后良好；内镜治疗不合适。涵盖性术语导管内乳头状黏液性肿瘤是合适的。
