de Klein Guus W, van Baarlen Joop, Mekenkamp Leonie J, Liem Mike S L, Klaase Joost M
Department of Surgery, Medisch Spectrum Twente, Enschede, The Netherlands.
Laboratorium Pathologie Oost-Nederland (LabPON), Hengelo, The Netherlands.
Case Rep Gastroenterol. 2018 Apr 27;12(1):194-201. doi: 10.1159/000488903. eCollection 2018 Jan-Apr.
Signet ring cell carcinoma (SRCC) of the ampulla of Vater is an extremely rare tumor. Our case describes a 45-year-old female presenting with jaundice and pruritus. Computed tomography, endoscopy, and endoscopic retrograde cholangiopancreatography showed a tumor of the ampulla of Vater without distant metastasis. Histological biopsy confirmed a malignant tumor with SRCC characteristics and immunohistochemical staining revealed a mixed type profile (both intestinal and pancreatobiliary characteristics). A pylorus-preserving pancreatoduodenectomy was performed and the patient recovered without complications. Pathology results concluded a pT2N0 ampullary SRCC. SRCC of the ampulla of Vater is known to be highly malignant. After 13 months of follow-up, our patient showed no signs of recurrence.
壶腹的印戒细胞癌（SRCC）是一种极其罕见的肿瘤。我们的病例描述了一名45岁出现黄疸和瘙痒的女性。计算机断层扫描、内窥镜检查和内镜逆行胰胆管造影显示壶腹有肿瘤且无远处转移。组织学活检证实为具有SRCC特征的恶性肿瘤，免疫组化染色显示为混合型（兼具肠型和胰胆管型特征）。实施了保留幽门的胰十二指肠切除术，患者恢复良好，无并发症。病理结果确诊为pT2N0壶腹SRCC。已知壶腹SRCC具有高度恶性。经过13个月的随访，我们的患者未出现复发迹象。
