Table 1 Clinicopathologic Features of the Pancreatobiliary Neoplasms with PRKACA Fusions

From: DNAJB1-PRKACA fusions occur in oncocytic pancreatic and biliary neoplasms and are not specific for fibrolamellar hepatocellular carcinoma

Case

Age /Sex

Initial presentation

Pathology diagnosis

Clinical follow-up

Intraductal component

Invasive component

Metastasis

1

63/M

7 cm mass in head of the pancreas

IOPN

Invasive adenocarcinoma with mucinous and oncocytic features

Not applicable

No follow-up data available

2

47/M

10 cm cystic mass in caudate lobe of liver

IOPN of the bile ducts

Invasive adenocarcinoma with oncocytic features

Not applicable

Recurrence after 4 years; now deceased

3

36/M

4.5 cm cystic mass in head of the pancreas

IOPN

Not applicable

Not applicable

No evidence of disease for 10 years

4

58/F

Complex cystic mass in head of the pancreas

IPMN, mixed oncocytic and pancreatobiliary features

Minute focus of invasion with mucinous and oncocytic features

Not applicable

No adjuvant therapy given. No evidence of disease for 9 months

5

52/M

2.8 cm cystic mass in head of the pancreas

IPMN, mixed oncocytic and gastric features

Not applicable

Not applicable

No evidence of disease for 6 years

6

76/M

Distal pancreatic mass

IPMN, mixed oncocytic and pancreatobiliary features

Invasive adenocarcinoma

Metastatic adenocarcinoma with oncocytic features, involving liver

Recurrence and liver metastases after 20 years, disease progression despite chemotherapy

  1. IOPN intraductal oncocytic papillary neoplasm, IPMN intraductal papillary mucinous neoplasm