A Comparison of Hepatic Mucinous Cystic Neoplasms With Biliary Intraductal Papillary Neoplasms

doi:10.1016/j.cgh.2009.02.019

Clinical Gastroenterology and Hepatology

Volume 7, Issue 5, May 2009, Pages 586-593

https://doi.org/10.1016/j.cgh.2009.02.019 Get rights and content

Background & Aims

There is controversy regarding the term biliary intraductal papillary neoplasms (IPN-B) and their pathology, which frequently are confused with hepatic mucinous cystic neoplasms (MCN). We aimed to summarize the clinicopathologic features of IPN-B and differentiate them from MCN.

Methods

From January 1998 to December 2007, there were 19 patients with intrahepatic IPN-B and 13 patients with MCN who underwent surgical treatment at Zhongshan Hospital. Multiple demographic and clinicopathologic parameters were reviewed retrospectively and compared between the groups.

Results

The mean ages of patients with IPN-B and MCN were 59.5 ± 11.1 and 44.4 ± 9.7 years, respectively (P = .0004); the male:female ratios also differed (11:8 vs 2:11; P = .028). Tumors were significantly smaller (6.0 vs 11.2 cm; P = .006) in patients with IPN-B than in those with MCN. More patients with IPN-B also had hepatolithiasis (47.4% vs 0%, P = .004); cholangiectasis and communication between the cyst and main bile duct were more frequent in patients with IPN-B than in those with MCN (P < .001). The IPN-B consisted of 4 subtypes—the gastric subtype was the least invasive. Malignant lesions were more common in patients with IPN-B than in those with MCN (78.9% vs 38.5%; P = .03). The overall 5-year survival rates of patients with IPN-B and MCN were 82% and 100%, respectively.

Conclusions

Intrahepatic IPN-B represents a distinct clinicopathologic entity that differs clinically, histologically, and radiologically from MCN. Curative resection has a favorable prognosis for patients with IPN-B, but further studies of its subtype are required.

Section snippets

Patients and Methods

A retrospective review was made of the medical records of all patients seen from January 1998 to December 2007 with histologically proven IPN-B in Zhongshan Hospital, Fudan University. IPN-B was defined microscopically as an intraductal papillary growth of neoplastic biliary epithelia with fine fibrovascular cores in the lumen of the biliary tree,⁸ and the absence of an ovarian-type stroma.¹⁰ Noninvasive tumors were classified as adenoma, borderline, or carcinoma in situ depending on the degree

Clinical Characteristics

There were 19 cases of intrahepatic IPN-B and 13 cases of MCN. Clinical characteristics are described in Table 1. The mean age of patients with IPN-B was 59.5 ± 11.1 years (median, 60 y; range, 30–76 y) and the male:female ratio was 11:8, which was quite different from those patients with MCN, for whom the mean age was 44.4 ± 9.7 years (median, 43 y; range, 28–60 y; P = .0004), and female predominance was marked (M:F ratio, 2:11; P = .028).

Overall, more than half of the patients of both groups

Discussion

In our study, IPN-B and MCN share some common characteristics, such as rare incidence, mucin production, and cystic mass. However, there are unique clinical findings for each type. IPN-B develops most commonly in patients between 50 and 70 years of age, which is much later than MCN. Although female predominance was noted in the MCN group, there was no marked sex preponderance in the IPN-B group. These data were similar to that of IPMN-P,15, 16 but different from the data of INP-B in Japan and

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Cited by (34)

Mucin-producing cystic hepatobiliary neoplasms: Key diagnostic information for radiologists
2024, Radiologia
Las neoplasias quísticas hepatobiliares productoras de mucina son neoplasias poco frecuentes que incluyen el tumor mucinoso hepático y el tumor papilar mucinoso intraductal del conducto biliar.
Los tumores mucinosos hepáticos se caracterizan por la presencia de epitelio estromal ovarian-like, y son neoplasias comúnmente benignas. Se presentan como masas grandes quísticas, multiloculadas, sin comunicación con la vía biliar, y son más frecuentes en mujeres de mediana edad.
El tumor papilar mucinoso intraductal del conducto biliar es una lesión premaligna, con origen en la vía biliar y que presenta comunicación con la misma, con 3 patrones posibles de crecimiento: quístico, ductoectásico o formador de masa. Se presenta en torno a los 50-70 años, sin predilección por género. Presentan una alta probabilidad de malignización, por lo que la resección segmentaria hepática es el tratamiento de elección.
Ambas entidades presentan características superpuestas y otras distintivas, siendo importante que el radiólogo conozca y distinga cada una de ellas, ya que presentan pronóstico y manejo diferente.
Mucin-producing cystic hepatobiliary neoplasms are rare neoplasms which include mucinous cystic neoplasms of the liver and intraductal papillary mucinous neoplasms of the bile duct.
Mucinous cystic neoplasms of the liver are characterised by the presence of ovarian-like stroma, and are usually benign. They present as large, multiloculated, cystic masses without biliary communication and are more common among middle-aged women.
Intraductal papillary mucinous neoplasms of the bile duct are premalignant lesions originating in the bile duct and presenting with biliary communication. There are 3 growth patterns: cystic, duct-ectatic and mass-forming. They tend to appear between the ages of 50 and 70, with no gender predilection. There is a high probability of malignancy, so segment-oriented hepatic resection is the treatment of choice.
Both entities have overlapping and distinctive features, and it is important for the radiologist to be aware of these and be able to distinguish between them, as they have different prognoses and require different management approaches.
MRI features of intraductal papillary mucinous neoplasm of the bile ducts, “The myth about the cyst”: A systematic review
2023, European Journal of Radiology Open
Intraductal papillary mucinous neoplasm of the bile ducts (IPMN-B) is a true pre-cancerous lesion, which shares common features with pancreatic IPMN (IPMN-P). While IPMN-P is a well described entity for which guidelines were formulated and revised, IPMN-B is a poorly described entity.
We carried out a systematic review to evaluate the existing literature, emphasizing the role of MRI in IPMN-B depiction.
PubMed database was used to identify original studies and case series that reported MR Imaging features of IPMN-B. The search keywords were "IPMN OR intraductal papillary mucinous neoplasm OR IPNB OR intraductal papillary neoplasm of the bile duct AND Biliary OR biliary cancer OR hepatic cystic lesions”. Risk of bias and applicability were evaluated using the QUADAS-2 tool.
884 Records were Identified through database searching. 12 studies satisfied the inclusion criteria, resulting in MR features of 288 patients. All the studies were retrospective. Classic features of IPMN-B are under-described. Few studies note worrisome features, concerning for an underlying malignancy. 50 % of the studies had a high risk of bias and concerns regarding applicability.
The MRI features of IPMN-B are not well elaborated and need to be further studied. Worrisome features and guidelines regarding reporting the imaging findings should be established and published. Radiologists should be aware of IPMN-B, since malignancy diagnosis in an early stage will yield improved prognosis
Cystic biliary tumors of the liver: diagnostic criteria and common pitfalls
2021, Human Pathology
Citation Excerpt :
Interestingly, the absence of ovarian-type stroma was thought to be associated with a worse prognosis [75,78–82]. It is likely that many of the neoplasms reported in earlier literature as MCN variants without ovarian-type stroma, particularly those in men or with a connection to the biliary duct system, would now be classified as cystic IPNBs [43,83,84]. The presence of ovarian stroma in MCN and its morphologic appearance similar to ovarian MCNs has led to the hypothesis that the neoplasm arises from ectopic ovarian mesenchymal stroma that migrates to the liver or pancreas during embryonic development [85].
With major advancements and frequent use of abdominal imaging techniques, hepatic cysts are increasingly encountered in clinical practice. Although the majority of cysts are benign, a small subset represents neoplastic precursors to cholangiocarcinoma. These cystic precursors include intraductal papillary neoplasms of the bile duct (IPNB) and mucinous cystic neoplasms of the liver (MCN-L), and bear striking pathologic resemblance to corresponding cystic neoplastic precursors within the pancreas. This review examines the salient clinical, gross, microscopic and molecular features of IPNBs and MCN-Ls, and, in particular, provides histopathologic comparison to their pancreatic counterparts. Considering these neoplasms may be diagnostically challenging, we also discuss other hepatic lesions within the differential diagnosis, and the potential for molecular methods to improve their preoperative evaluation and the early detection of cholangiocarcinoma.
Intraductal papillary neoplasm of the bile duct – A comprehensive review
2021, Advances in Medical Sciences
Intraductal papillary neoplasm of the bile ducts is a rare tumor type. Management decisions are currently based upon a small case series. The authors have large own experience with IPNB.
The review aims at reporting on clinicopathological features of IPNB in order to provide guidance for management.
We searched PubMed, Medline, Microsoft Academic and Embase databases to identify studies of relevance. The analysis of own experience was also included.
We analyzed 59 retrospective series and 25 cases from authors’ clinical experience. The main sign was jaundice and cholangitis, 33% and 48%, respectively. CT’s were performed in 63–76% and MR in 40–56%. Intraductal mass was found in 31–32% and duct dilatation in 27–30%. Endoscopic Retrograde Cholangio-Pancreatography (ERCP) was performed in 48–62%. IPNB with invasive carcinoma was found in 35.7–60% and IPNB with intraepithelial neoplasia in 36–60%. Histopathological confirmation before surgery was rare. The main treatment of IPNB is resection, in our material, both, hepatectomy and hepatectomy plus bile duct resections were performed in 40% of patients. The percentage of postoperative complications was 20%. The 5-year survival rate of all IPNB’s patients was 53.6%; in patients with associated invasive carcinoma - 22.2% and without invasive carcinoma - 100% (p = 0.001).
Early surgery is advisable for radiologically suspected IPNB. The results of treatment depend on histopathology. They are worse at intraductal invasive carcinoma than at neoplasm with neoplasia.
A Rapidly Progressing Polycystic Liver Disease?
2020, Gastroenterology
The impact of imaging on the surgical management of biliary cystadenomas and cystadenocarcinomas; a systematic review
2019, HPB
Biliary Cystadenomas (BCA) are considered to be benign but may transform to Biliary Cystadenocarcinomas (BCAC). The aim of this systematic review was to assess the diagnostic work-up and necessity of complete surgical resection.
A systematic literature search was performed in Embase.com, Medline (Ovid), Cochrane Central, Web-of-Science and Google Scholar. Articles reporting on diagnostic work-up or outcome of various treatment strategies were included.
Fifty-one articles with 1218 patients were included: 971 with BCA and 247 with BCAC. Patients with BCA were more often female (91% vs 63.8%, p < 0.001). On radiologic imaging BCAC more often had calcifications (p = 0.008), mural nodules (p < 0.001) and wall enhancement (p < 0.001). Reported treatment strategies were resection, enucleation, or fenestration/marsupialization. Recurrence was reported in 5.4% after resection for BCA and 4.8% after resection for BCAC. Recurrence after fenestration/marsupialization varied from 81.6% to 100% for both BCA as BCAC. Mortality rate was 0 in patients with BCA and 24% in BCAC.
Due to the difficulty in accurately diagnosing these biliary cystic lesions and the availability of different surgical approaches, patients with suspected BCA or BCAC should be treated in a center specialized in liver surgery with state-of-the-art imaging and all surgical techniques to prevent mismanagement of this rare disease.

View all citing articles on Scopus

: Conflicts of interest The authors disclose no conflicts.

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Original article—liver, pancreas, and biliary tractA Comparison of Hepatic Mucinous Cystic Neoplasms With Biliary Intraductal Papillary Neoplasms

Background & Aims

Methods

Results

Conclusions

Section snippets

Patients and Methods

Clinical Characteristics

Discussion

J Hepatol

Hepatology

Mod Pathol

J Hepatol

Gastrointest Endosc

Am J Surg

Gastrointest Endosc

Gastrointest Endosc

Gastrointest Endosc

Clinicopathologic review of 58 patients with biliary papillomatosis

Cancer

Papillomatosis of the intra- and extrahepatic bile ducts with involvement of the pancreatic duct

Hepatogastroenterology

Clinical studies of mucin-producing cholangiocellular carcinoma: a study of 22 histopathology-proven cases

Ann Surg

Mucin-hypersecreting bile duct tumor characterized by a striking homology with an intraductal papillary mucinous tumor (IPMT) of the pancreas

Endoscopy

Intraductal papillary mucinous neoplasms of the pancreas: an updated experience

Ann Surg

Branch-duct intraductal papillary mucinous neoplasms of the pancreas: to operate or not to operate?

Gut

Biliary papillary tumors share pathological features with intraductal papillary mucinous neoplasm of the pancreas

Hepatology

Cystadenocarcinoma of the liver without mesenchymal stroma: possible progression from a benign cystic lesion suspected by follow-up imagings

J Gastroenterol

Hepatobiliary cystadenoma with mesenchymal stroma

Ann Surg

Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study

Virchows Arch

Original article—liver, pancreas, and biliary tract
A Comparison of Hepatic Mucinous Cystic Neoplasms With Biliary Intraductal Papillary Neoplasms