Intraductal papillary mucinous tumor of bile ducts radiologic and pathologic features: a case report

Abstract

We report a case of a 67-year-old Caucasian man with right upper quadrant abdominal pain. He underwent radiologic investigations that revealed a solid, focal mass, at the V hepatic segment. Because a definitive diagnosis, based on imaging appearance of the lesion, was impossible in our case, we performed a hystopathological investigation but the biopsies were inconclusive. So, the definitive diagnosis of intraductal papillary mucinous tumor of bile ducts was made on surgical resected material.Intraductal papillary neoplasm of the liver (IPNL) is a recently recognized entity which closely resembles an intraductal papillary mucinous tumor (IPMT) of the pancreas.

Figure 1

Transverse CT scan. It reveals a solid focal mass, about 3 cm in diameter, hypodense respect to the surrounding tissue in the basal phase (1) with a light peripherical enhanced rim in the arterial phase (2) that increases in the portal venous phase (3). In late phase (4), the lesion shows a puntacte aspect because of the presence of intralesional hypervascular spots (arrow). The bile ducts are not dilatated.

Figure 2

Transverse CT scan. It reveals a solid focal mass, about 3 cm in diameter, hypodense respect to the surrounding tissue in the basal phase (1) with a light peripherical enhanced rim in the arterial phase (2) that increases in the portal venous phase (3). In late phase (4), the lesion shows a puntacte aspect because of the presence of intralesional hypervascular spots (arrow). The bile ducts are not dilatated.

Figure 3

Transverse CT scan. It reveals a solid focal mass, about 3 cm in diameter, hypodense respect to the surrounding tissue in the basal phase (1) with a light peripherical enhanced rim in the arterial phase (2) that increases in the portal venous phase (3). In late phase (4), the lesion shows a puntacte aspect because of the presence of intralesional hypervascular spots (arrow). The bile ducts are not dilatated.

Figure 4

Transverse CT scan. It reveals a solid focal mass, about 3 cm in diameter, hypodense respect to the surrounding tissue in the basal phase (1) with a light peripherical enhanced rim in the arterial phase (2) that increases in the portal venous phase (3). In late phase (4), the lesion shows a puntacte aspect because of the presence of intralesional hypervascular spots (arrow). The bile ducts are not dilatated.

Figure 5

Transverse RM scan. It reveals a slightly dishomogeneous hyperintense mass in sequences with long TR (5) and the appearance of intralesional hypervascular spots (arrow) in late phase after administration of paramagnetic contrast agent (6).

Figure 6

Transverse RM scan. It reveals a slightly dishomogeneous hyperintense mass in sequences with long TR (5) and the appearance of intralesional hypervascular spots (arrow) in late phase after administration of paramagnetic contrast agent (6).

Figure 7

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 8

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 9

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 10

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 11

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 12

Pathological sections specimens. (7) Low magnification showing encapsulated papillary tumor with thin fibrovascular stalk, covered by tall mucinous moderately dysplastic epithelium (8). Higher magnification demonstrates area with oncocitic pattern (9). The immunohistochemical test showed a diffuse CK7+ (10) and apomucines MUC5AC (11) and MUC6 (12) positivity in more than 50% of neoplastic cells.

Figure 13

CT follow up examination. It shows the patient free of the disease.