Intraductal Oncocytic Papillary Neoplasms of the Pancreas

Abstract

We describe the clinical and pathologic features of 11 intraductal oncocytic papillary neoplasms of the pancreas, a hitherto unrecognized tumor. The patients were six men and five women, and most of the tumors were in the head (head: body/tail = 8:3). The mean patient age was 62 (range, 39-78), and the average tumor size was 6 cm. Grossly the tumors exhibited mucin-filled cysts containing nodular papillary projections. Dilated ducts communicating with the main tumor were sometimes noted. Microscopically the cystic structures appeared to represent dilated ducts containing intraductal tumor. The tumors were characterized by variably complex, arborizing papillary structures. The papillae had thin, delicate fibrovascular cores with focal myxoid changes and were lined by stratified oncocytic cells. Goblet cells and intra-epithelial mucin-containing lumina were present, the latter resulting in a characteristic cribriform pattern. The exuberance of the epithelial proliferation varied from case to case and between different regions within individual tumors; solid sheets of cells were often identified. Although the degree of cytologic atypia was not generally severe, the complexity of the architecture justified a designation of intraductal oncocytic papillary carcinoma in 10 of the 11 cases. In nine cases the tumor was entirely intraductal; one case exhibited focal microinvasion and another showed widespread invasive carcinoma, the invasive elements appearing cytologically similar to the intraductal papillary components. The oncocytic cells stained positively with phosphotungstic acid hematoxylin and Novelli stains. Immunohistochemically, all cases stained positively for B72.3, and five cases showed focal, weak luminal membrane staining for carcinoembryonic antigen. Ultrastructurally many of the cells were packed with mitochondria, and mucin was also identified. Seven patients were alive and free of tumor from 1 month to 3 years (average, 1 year) after resection. Two patients died postoperatively. The remaining two patients died with no evidence of disease at 2.5 and 5 years, the latter following a recurrence at 2.5 years. We conclude that intraductal oncocytic papillary neoplasm is a distinctive pancreatic tumor that is usually intraductal but may develop invasive carcinoma and should be treated with complete resection.

Fascin overexpression in intraductal papillary mucinous neoplasms (adenomas, borderline neoplasms, and carcinomas) of the pancreas, correlated with increased histological grade

“…Gastric types usually showed mild dysplasia and intestinal types showed moderate to severe dysplasia, whereas pancreatobiliary and oncocytic types showed severe dysplasia corresponding to carcinoma in situ; 8,10,14 intestinal-type neoplasms were more frequently associated with a mucinous noncystic (colloid) invasive pattern compared with other types; 4,7-9,12 and the oncocytic type was a rare variant. 46 With regard to the relationship between fascin expression and the phenotypic subclassification, fascin overexpression occurred more frequently in intestinal-type neoplasms (90%) than in the gastric type (61%) by multiple comparisons. In contrast, intestinal-type neoplasms exhibited a higher histological grade than the gastric type.…”

Oncocytic Biliary Cystadenocarcinoma Is a Form of Intraductal Oncocytic Papillary Neoplasm of the Liver

“…Recently, Adsay et al introduced a new disease entity, "intraductal oncocytic papillary neoplasm of the pancreas" (13). This type of tumor develops in middle-to old-aged men as well as women.…”

Molecular, Histologic, and Radiologic Findings of High-Grade Invasive Adenocarcinoma Arising in Oncocytic Subtype of Intraductal Papillary Mucinous Neoplasm: A Case Report and Review of Literature

“…3 Recent molecular studies suggest that, when strict morphological criteria are applied, the oncocytic subtypes are genetically distinct lesions. These lesions lack the most common molecular abnormalities found in IPMNs.…”

“…This provides additional evidence that O-IPMNs have a distinct genetic tumorigenesis. This may explain why, empirically, these lesions have an improved 5-year survival compared with gastric, pancreatobiliary, or intestinal IPMNs 1 with or without an invasive component.…”

“…The epithelial subtypes (gastric, intestinal, pancreatobiliary, and oncocytic) have been shown to be independent predictors of biological behaviors, recurrence, and long-term survival rate. The rarest subtype, oncocytic, was first described by Adsay et al 1 These lesions tended to be larger than the typical IPMN, measuring up to 15 cm in size, with no predilection for location in the pancreas. Infrequently, these tumors develop an associated low-grade invasive carcinoma that retains the oncocytic features.…”