Colloid carcinoma of the minor duodenal papilla

A 43-year-old woman presented with intermittent epigastric pain and nausea. Her hemoglobin concentration was 10.3 g/dL. Abdominal imaging revealed a 4-cm cystic mass in the pancreatic head ([Figure 1]). ERCP showed an ulcerated polypoid mass at the minor duodenal papilla ([Figure 2]). Cannulation of the normal-appearing major papilla showed a dilated, 4-mm main pancreatic duct and a stricture of the main duct in the head of the gland ([Figure 3]). Cannulation through the mass showed contrast in the ectatic dorsal pancreatic duct, which confirmed involvement of the minor papilla by tumor. Pancreas divisum was not present. A pancreaticoduodenectomy (Whipple procedure) revealed a polypoid gelatinous mass measuring 3 × 2 × 1.5 cm protruding from the minor papilla. Histologically the tumor showed mucin pools containing malignant epithelial cells ([Figure 4]). Resection margins and 15 peripancreatic lymph nodes were free of tumor. Despite an uncomplicated immediate postoperative course, CA 19 - 9 levels are rising 22 months later along with possible liver metastases.

Figure 1 Computed tomography showing cystic mass in the head of the pancreas.

Figure 2 Ulcerated, polypoid mass at the minor duodenal papilla.

Figure 3 Stricture of the proximal duct of Wirsung seen at ERCP.

Figure 4 Histological appearance of the tumor with pools of mucin containing scant malignant glandular epithelial cells. (H&E; original magnification × 20).

Tumors of the minor papilla are uncommon, but carcinoid tumors [1], somatostatinomas [2], and a case of a nonendocrine ductal adenocarcinoma [3] have been reported. Reasons for the rarity of recorded tumors in this location may be a low incidence but also the lack of symptoms caused by small indolent endocrine neoplasms and the absence of jaundice owing to patency of the major papilla [3]. Aggressive neoplasms may overgrow adjacent structures, thus obscuring their origin at the minor papilla [3]. Mucinous noncystic (colloid) carcinoma of the pancreas represents only 1 % - 2 % of all pancreatic nonendocrine neoplasms [4] [5] and has not been described in the minor papilla previously. It is characterized histologically by extracellular mucin lakes with “floating” malignant epithelial cells [4] [5]. It is important to distinguish colloid carcinoma from mucin-producing adenocarcinoma, signet-ring cell carcinoma and mucinous cystic neoplasms because the prognosis of colloid carcinoma is significantly better than that of ordinary pancreatic ductal adenocarcinoma, with a 5-year survival rate of 57 % [4] [5].

Endoscopy_UCTN_Code_CCL_1AB_2AZ_3AB

References

• 1 Noda Y, Watanabe H, Iwafuchi M, Furuta K. et al . Carcinoids and endocrine cell micronests of the minor and major duodenal papillae. Their incidence and characteristics.  Cancer. 1992;  70 1825-1833
• 2 Malone M J, Silverman M L, Braasch J W. et al . Early somatostatinoma of the duct of Santorini.  Arch Surg. 1985;  120 1381-1383
• 3 Yamao K, Ohhashi K, Furukawa T. et al . Primary carcinoma of the duodenal minor papilla.  Gastrointest Endosc. 1998;  48 634-636
• 4 Adsay N V, Pierson C, Sarkar F. et al . Colloid (mucinous noncystic) carcinoma of the pancreas.  Am J Surg Pathol. 2001;  25 26-42
• 5 Whang E E, Danial T, Dunn J C. et al . The spectrum of mucin-producing adenocarcinoma of the pancreas.  Pancreas. 2000;  21 147-151