Signet ring cell carcinoma of the ampulla of Vater: Immunophenotype and differentiation

Abstract

Signet ring cell carcinoma (SRC) of the ampulla of Vater is extremely rare and the histogenesis remains unknown. In the present study, to investigate the immunohistochemical phenotypes, discuss the histological origin and evaluate the correlation between the immunohistochemical phenotypes and survival of ampullary SRC patients, a retrospective review was conducted. This included all ampullary carcinoma patients treated at The First Affiliated Hospital, College of Medicine, Zhejiang University, and was performed over a five-year period between 2008 and 2012. Eight resected ampullary SRC specimens were examined histopathologically and immunohistochemically, using cytokeratin (CK) and mucin (MUC) immunohistochemical phenotypes. Of all 162 patients with ampullary lesions, eight cases (4.9%) of ampullary SRC were identified. Immunohistochemical analyses of the eight cases revealed the positive expression of CK7 in five, CK19 in seven, CK20 in one, MUC1 in five, MUC2 in three, caudal-related homeobox transcription factor 2 in one, MUC5AC in seven and MUC6 in four of the eight cases, while loss of E-cadherin and β-catenin was observed in four of the eight cases. According to immunohistochemical classification, ampullary SRC can be classified into four subtypes: Intestinal (I), pancreatobiliary (PB), gastric and mixed types (composed of I mucosa lining and PB epithelium). Patients with the I-type ampullary SRC demonstrated a more favorable prognosis than that of patients with the PB-type ampullary SRC. Additionally, patients with ampullary SRC of I or PB type with gastric differentiation may have a worse prognosis than others. The coexpression of the E-cadherin/β-catenin complex may also indicate poor prognosis in PB-type ampullary SRC. In conclusion, the clinical five-year follow-up of the patients with pure SRC was more positive than that of those with I-, PB-, gastric- or mixed-type ampullary SRC. The coexpression of the E-cadherin/β-catenin complex may present a poor prognosis in the PB type of ampullary SRC.

Poorly differentiated adenocarcinoma with signet-ring cells in duodenal papilla: a case report

“…She was diagnosed with DPC incidentally in the health checkup before having jaundice; therefore, the ratio of signet-ring cells was relatively low. Similar reports are very few for DPC [25]. …”

Signet Ring Cell Carcinoma of the Ampulla of Vater with Early Development of Bone Metastasis: Case Report and Review of the Rare Malignancy

“…Blundell et al have further characterized these tumors to be neuroendocrine in origin [28]. Variable immunostaining for ampullary signet cancers (positivity for CK7, CK19, CK20, CDX2, MUC1/2, MUC5AC, MUC6) indicates heterogeneous origin from cells with pancreatobiliary, intestinal, and gastric differentiation [27]. There appears to be higher incidence in males than that in females.…”

“…Distant metastases appear to be associated with a short life expectancy after surgery. Acharya [3] 78/F T3N0M0 Pancreatoduodenectomy -Alive at 6 months Akatsu [4] 43/F T2N0M0 Pancreatoduodenectomy -Alive at 7.5 years Bloomston [5] 58/F T2N0M0 Pancreatoduodenectomy -Alive at 11 years Dauodi [6] 55/M T3N0M0 Pancreatoduodenectomy Chemotherapy Alive at 8 months Erquchi [7] 83/M T3N0M0 Pancreatoduodenectomy -Alive at 18 months Fang [8] 53/M T2N0M0 Pancreatoduodenectomy -Alive at 2 years Gao [9] 38/F T3N0M0 Pancreatoduodenectomy -Alive at 6 months Garcia [10] 73/M T2N1M0 Pancreatoduodenectomy -Alive at 14 months Garcia [10] 74/M T3N0MX Total pancreatectomy -Alive at 3 months Gardner [2] 69/F T3N0M0 Pancreatoduodenectomy -Unknown Gheza [11] 66/M Unknown Pancreatoduodenectomy -Alive at 8 months Hara [12] 68/M T2N0M0 Pancreatoduodenectomy -Alive at 10 months Ishibashi [13] 59/M T3N0M0 Pancreatoduodenectomy -Alive at 1.5 years Kim [14] 4 7 / M T X N 1 M X -Chemotherapy Died at 15 months Lesquereux-Martinez [15] 78/F TXN1M0 Pancreatoduodenectomy Chemotherapy Alive at 14 months Li [16] 56/F T2N1M0 Pancreatoduodenectomy -Alive at 12 months Maekawa [17] 75/M T3N0M0 Pancreatoduodenectomy -Died at 6 months Nabeshima [18] 49/M T3NXM1 -Chemotherapy Died at 12 months Paplomata [19] 45/F T4N1MX Pancreatoduodenectomy Chemotherapy Died at 12 months Purohit [20] 32/F TXNXM1 Declined Declined Unknown Ramia [21] 67/F T2N0M0 Pancreatoduodenectomy -Alive at 12 months Sekoguchi [22] 47/M T3N0M0 --Unknown Tas [23] 4 0 / M T X N 1 M 1 --Unknown Terada [24] 74/F TXNXM0 Biliary stent Chemotherapy Died at 14 months Tseng [25] 47/M T3N0M0 Pancreatoduodenectomy -Alive at 6 months Valeri [26] 66/M Unknown Pancreatoduodenectomy -Alive at 6 months Wen [27] 40/F T3N0M0 Pancreatoduodenectomy Chemotherapy Alive at 8 months Wen [27] 64/F T4NXM0 Pancreatoduodenectomy -Alive at 6 years Wen [27] 75/F T4NXM0 Pancreatoduodenectomy Chemoradiation Died at 16 months Wen [...…”

“…Pattern of recurrence appears to be variable, with distant metastasis scarcely reported in the literature as a first presentation. Signet ring cell carcinoma has been shown to metastasize to distant regions such as the bone marrow [18], the liver [27], and also the leptomeninges [19].…”

Immunohistochemical expression of cytokeratins in human salivary gland acinic cell carcinomas

“…53,54 In normal salivary glands, strong expression of CK19 has been reported in luminal ductal cells. Positive staining was also observed in the myoepthelium, acinar cells, and ductal basal cells.…”