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Background

This rare tumor describes an intraductal proliferation of neoplastic biliary epithelium, occuring within the liver. It has a morphologic resemblance to similar intraductal mucinous proliferations occurring within the pancreas.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Differential Diagnosis  
Prognosis and Treatment  
Commonly Used Terms  


EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS IPN
INCIDENCE Rare

 

DISEASE ASSOCIATIONS CHARACTERIZATION
HEPATOLITHIASIS  


Intraductal papillary neoplasia of the liver associated with hepatolithiasis.

Chen TC, Nakanuma Y, Zen Y, Chen MF, Jan YY, Yeh TS, Chiu CT, Kuo TT, Kamiya J, Oda K, Hamaguchi M, Ohno Y, Hsieh LL, Nimura Y.

Department of Pathology, Chang Gung Memorial Hospital, Chang Gung University School of Medicine, Tao Yuan, Taipei, Taiwan.

Hepatology 2001 Oct;34(4 Pt 1):651-8 Abstract quote

Intraductal papillary growth of neoplastic biliary epithelia with a fine fibrovascular stalk (intraductal papillary neoplasia of liver [IPN-L]) resembling intraductal papillary mucinous neoplasm of pancreas is occasionally associated with hepatolithiasis.

In this study, 136 cases of hepatolithiasis in Taiwan, between January 1998 and March 2000, and an additional 21 cases of IPN-L before December 1998, were examined histologically. IPN-L was found in 41 of 136 hepatolithiasis cases (30.1%). Sixty-two IPN-L cases (42 women and 20 men; age range, 59.8 +/- 10 years) were divided into 4 types (type 1, IPN-L with low-grade dysplasia, 23 cases; type 2, IPN-L with high grade dysplasia, 11 cases; type 3, IPN-L with in situ and microinvasive carcinoma, 13 cases; and type 4, IPN-L of types 2 and 3 with distinct invasive carcinoma, 15 cases). Intraductal spreading and glandular involvement were commonly observed in all types. About half of types 3 and 4 cases had mucobilia, and mucinous carcinoma was variably found in two thirds of group 4 patients. IPN-L frequently showed variable gastroenteric differentiation such as goblet cells and foveolar and colon-like metaplasia. IPN-L with goblet cells and colon-like metaplasia was frequently associated with overproduction of mucin and mucobilia (P <.01).

In Japan, IPN-L was not frequent in hepatolithiasis (12 of 135 cases). In conclusion, IPN-L forms a spectrum of biliary neoplasm in hepatolithiasis. It often displays variable gastroenteric metaplasia and significant intraductal spread. IPN-L tends to progress to mucinous carcinoma. Formerly reported "mucin-producing intrahepatic cholangiocarcinoma" with a favorable prognosis is included in IPN-L.

 

PATHOGENESIS CHARACTERIZATION
p53  


Increasing expression of gastrointestinal phenotypes and p53 along with histologic progression of intraductal papillary neoplasia of the liver.

Shimonishi T, Zen Y, Chen TC, Chen MF, Jan YY, Yeh TS, Nimura Y, Nakanuma Y.

Department of Pathology (II), Kanazawa University School of Medicine, Japan.

Hum Pathol 2002 May;33(5):503-11 Abstract quote

Intraductal papillary neoplasia of the liver (IPN-L) was recently proposed as the name for intraductal papillary proliferation of neoplastic biliary epithelium with a fine fibrovascular stalk resembling intraductal papillary mucinous neoplasm of the pancreas.

We histochemically and immunohistochemically examined IPN-L alone or associated with hepatolithiasis, with an emphasis on the gastrointestinal metaplasia, nuclear p53 expression, and histologic progression. A total of 66 cases of IPN-L were divided into 4 groups: group 1, IPN-L with low-grade dysplasia (13 cases); group 2, IPN-L with high-grade dysplasia (20 cases); group 3, IPN-L lined with carcinoma in situ and no or microinvasion (19 cases); and group 4, group 3 with distinct invasive carcinoma (14 cases). It is suggested that IPN-L progresses from group 1 to group 4. As controls, 20 cases of nonneoplastic intrahepatic large bile ducts and 17 cases of nonpapillary invasive intrahepatic cholangiocarcinoma (ICC) were used. Biliary epithelial hypersecretion of sialomucin rather than sulfomucin was prevalent in IPN-L, and this was associated with the progression of INP-L. Immunohistochemically, cytokeratin (CK) 20 and MUC2, a gastrointestinal marker, were expressed more frequently in IPN-L than in nonneoplastic bile ducts and nonpapillary ICC (P <0.01), and their incidence were significantly increased in parallel with the progression of IPN-L (P < 0.01).

In contrast, expression of CK 7, a biliary marker, was decreased in IPN-L compared with nonpapillary ICC. Nuclear p53 immunostaining was detected in 30% of IPN-L as a whole and increased in tandem with the progression of IPN-L (P < 0.01).

It is suggested that IPN-L forms a spectrum of biliary epithelial neoplasia with frequent gastrointestinal metaplasia, different from the usual nonpapillary ICC, and shows stepwise progression from the perspective of mucin profile, gastrointestinal metaplasia, and p53 nuclear expression. Copyright 2002, Elsevier Science (USA). All rights reserved.

 

LABORATORY/RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  


Radiological spectrum of intraductal papillary tumors of the bile ducts.

Lim JH, Yi CA, Lim HK, Lee WJ, Lee SJ, Kim SH.

Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Kangnam-gu, Seoul 135-710, Korea.

Korean J Radiol 2002 Jan-Mar;3(1):57-63 Abstract quote

Papillary tumor of the bile duct is characterized by the presence of an intraductal tumor with a papillary surface comprising innumerable frondlike infoldings of proliferated columnar epithelial cells surrounding slender fibrovascular stalks.

There may be multiple tumors along the bile ducts (papillomatosis or papillary carcinomatosis), which are dilated due to obstruction by a tumor per se, by sloughed tumor debris, or by excessive mucin. Radiologically, the biliary tree is diffusely dilated, either in a lobar or segmental fashion, or aneurysmally, depending on the location of the tumor, the debris, and the amount of mucin production. A tumor can be depicted by imaging as an intraductal mass with a thickened and irregular bile duct wall. Sloughed tumor debris and mucin plugs should be differentiated from bile duct stones.

Cystically or aneurysmally, dilated bile ducts in mucin-hypersecreting variants (intraductal papillary mucinous tumors) should be differentiated from cystadenoma, cystadenocarcinoma and liver abscess.

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL Intraductal papillary growth with marked dilatation of intrahepatic ducts
VARIANTS  

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL Intraductal papillary growth of neoplastic biliary epithelia with fine vascular cores
VARIANTS  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF PANCREAS  


Intraductal papillary mucinous neoplasms of the pancreas: an increasingly recognized clinicopathologic entity.

Sohn TA, Yeo CJ, Cameron JL, Iacobuzio-Donahue CA, Hruban RH, Lillemoe KD.

Department of Surgery, the Johns Hopkins Medical Institutions, Baltimore, Maryland 21287-4606, USA.er studies of the molecular genetics and natural history of these unusual neoplasms.

Ann Surg 2001 Sep;234(3):313-21 Abstract quote

OBJECTIVE: To assess the authors' experience with intraductal papillary mucinous neoplasms of the pancreas (IPMNs).

SUMMARY BACKGROUND DATA: Intraductal papillary mucinous neoplasms of the pancreas are being recognized with increasing frequency.

METHODS: All patients who underwent pancreatic resection for an IPMN at the Johns Hopkins Hospital between January 1987 and December 2000 were studied. The data were compared with those of 702 concurrent patients with infiltrating ductal adenocarcinoma of the pancreas not associated with an IPMN resected by pancreaticoduodenectomy.

RESULTS: In the 13-year time period, 60 patients underwent pancreatic resection for IPMNs, with 40 patients undergoing resection in the past 3 years. Mean age at presentation was 67.4 +/- 1.4 years. The most common presenting symptom in patients with IPMNs was abdominal pain (59%). Most IPMNs were in the head of the pancreas or diffusely involved the gland, with 70% being resected via pancreaticoduodenectomy, 22% via total pancreatectomy, and 8% via distal pancreatectomy. Twenty-two patients (37%) had IPMNs with an associated infiltrating adenocarcinoma. In a subset of IPMNs immunohistochemically stained for the Dpc4 protein (n = 50), all of the intraductal or noninvasive components strongly expressed Dpc4, whereas 84% of associated infiltrating cancers expressed Dpc4. The 5-year survival rate for all patients with IPMNs (n = 60) was 57%.

CONCLUSION: Intraductal papillary mucinous neoplasms represent a distinct clinicopathologic entity being recognized with increasing frequency. IPMNs are clinically, histologically, and genetically disparate from pancreatic ductal adenocarcinomas. The distinct clinical features, the presumably long in situ or noninvasive phase, and the good long-term survival of patients with IPMNs offer a unique opportunity for early diagnosis, curative resection, and further studies of the molecular genetics and natural history of these unusual neoplasms.

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
PROGNOSTIC FACTORS  
TREATMENT Surgical resection

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.


Commonly Used Terms

Liver


Last Updated 8/1/2002


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The Doctor's Doctor