Suzuki Rei, Okada Ryo, Muto Makoto, Takagi Tadayuki, Sugimoto Mitsuru, Irie Hiroki, Nakamura Jun, Takasumi Mika, Kato Tsunetaka, Hashimoto Minami, Notohara Kenji, Suzuki Osamu, Hashimoto Yuko, Hikichi Takuto, Marubashi Shigeru, Ohira Hiromasa
Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan.
Department of Hepato-Biliary-Pancreatic and Transplant Surgery, Fukushima Medical University School of Medicine, Fukushima, 960-1295, Japan.
Clin J Gastroenterol. 2020 Dec;13(6):1315-1321. doi: 10.1007/s12328-020-01162-z. Epub 2020 Jun 27.
The present case involved a 60-year-old man with autoimmune pancreatitis (AIP). While his AIP was in remission for 6 years, a follow-up CE-CT revealed a dilated main pancreatic duct (MPD) and an enhanced mural nodule. Fluorodeoxyglucose uptake was positive on positron emission tomography. Endoscopic retrograde pancreatography showed a filling defect of the MPD, and pancreatoscopy revealed a nodule partially covered with papillary lesions. Although a repeat biopsy revealed no evidence of malignancy, we speculated that there was a high likelihood of main-duct-type intraductal papillary mucinous neoplasm (IPMN)-derived carcinoma concomitant with AIP. Subsequently, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy. A surgical specimen showed a 35 mm protuberant papillary lesion with abundant stroma, located in the main duct of the pancreas. Further histological evaluation revealed that the nodule was predominantly composed of IPMN with low-grade dysplasia, which was accompanied by abundant IgG4-positive lymphoplasmacytic infiltration, and fibrosis existed predominantly around the IPMN. The epithelium of the cyst showed mucinous hyperplasia with focal papillary structures of gastric phenotype (MUC5A+, MUC6+, MUC1-, MUC2-, CDX-). After surgical resection, we did not find any imaging evidence suggesting a recurrent tumor and AIP relapse in the remnant pancreas. In conclusion, we report a case of IPMN coincidentally found in a patient with type 1 AIP. Active AIP may exaggerate the morphology of IPMN, and careful evaluation should be performed to select appropriate management.
本病例为一名60岁的自身免疫性胰腺炎（AIP）男性患者。其AIP缓解6年后，随访的增强CT显示主胰管（MPD）扩张及壁结节强化。正电子发射断层扫描显示氟脱氧葡萄糖摄取呈阳性。内镜逆行胰胆管造影显示MPD有充盈缺损，胰管镜检查发现一个结节部分被乳头状病变覆盖。尽管再次活检未发现恶性证据，但我们推测存在主胰管型导管内乳头状黏液性肿瘤（IPMN）衍生的癌合并AIP的高度可能性。随后，患者接受了保留胃的胰十二指肠次全切除术。手术标本显示胰腺主胰管内有一个35毫米突出的乳头状病变，间质丰富。进一步的组织学评估显示，该结节主要由低级别异型增生的IPMN组成，伴有大量IgG4阳性淋巴浆细胞浸润，且纤维化主要存在于IPMN周围。囊肿上皮显示黏液性增生，有局灶性胃表型乳头状结构（MUC5A+、MUC6+、MUC1-、MUC2-、CDX-）。手术切除后，我们未发现任何影像学证据提示残留胰腺有肿瘤复发和AIP复发。总之，我们报告了一例在1型AIP患者中偶然发现的IPMN病例。活动性AIP可能会夸大IPMN的形态，应进行仔细评估以选择合适的治疗方法。
