We describe the clinical and pathologic features of II intraductal onc
ocytic papillary neoplasms of the pancreas, a hitherto unrecognized tu
mor. 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 tumor
s exhibited mucin-filled cysts containing nodular papillary projection
s. Dilated ducts communicating with the main tumor were sometimes note
d. 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 th
in, delicate fibrovascular cores with focal myxoid changes and were li
ned by stratified oncocytic cells. Goblet cells and intraepithelial mu
cin-containing lumina were present, the latter resulting in a characte
ristic cribriform pattern. The exuberance of the epithelial proliferat
ion varied from case to case and between different regions within indi
vidual tumors; solid sheets of cells were often identified. Although t
he 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 anot
her showed widespread invasive carcinoma, the invasive elements appear
ing cytologically similar to the intraductal papillary components. The
oncocytic cells stained positively with phosphotungstic acid hematoxy
lin and Novelli stains. Immunohistochemically, all cases stained posit
ively for B72.3, and five cases showed focal, weak luminal membrane st
aining for carcinoembryonic antigen. Ultrastructurally many of the cel
ls were packed with mitochondria, and mucin was also identified. Seven
patients were alive and free of tumor from 1 month to 3 years (averag
e, 1 year) after resection. Two patients died postoperatively. The rem
aining two patients died with no evidence of disease at 2.5 and 5 year
s, the latter following a recurrence at 2.5 years. We conclude that in
traductal oncocytic papillary neoplasm is a distinctive pancreatic tum
or that is usually intraductal but may develop invasive carcinoma and
should be treated with complete resection.