Signet Ring Cell Carcinoma of the Ampulla of Vater: A Rare Histopathological Variant

Guus W de Klein; Joop van Baarlen; Leonie J Mekenkamp; Mike S L Liem; Joost M Klaase

doi:10.1159/000488903

Signet Ring Cell Carcinoma of the Ampulla of Vater: A Rare Histopathological Variant

Case Rep Gastroenterol. 2018 Apr 27;12(1):194-201. doi: 10.1159/000488903. eCollection 2018 Jan-Apr.

Authors

Guus W de Klein¹, Joop van Baarlen², Leonie J Mekenkamp³, Mike S L Liem¹, Joost M Klaase⁴

Affiliations

¹ Department of Surgery, Medisch Spectrum Twente, Enschede, The Netherlands.
² Laboratorium Pathologie Oost-Nederland (LabPON), Hengelo, The Netherlands.
³ Department of Internal Medicine, Medisch Spectrum Twente, Enschede, The Netherlands.
⁴ Department of Surgery, Universitair Medisch Centrum Groningen, Groningen, The Netherlands.

Abstract

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.

Keywords: Jaundice; Periampullary carcinoma; Signet ring cell carcinoma.

Publication types

Case Reports