Cytologic Assessment of Intraductal Papillary Neoplasm of the Bile Duct (IPNB): Case Report
sherehan Zada*, Di Lu, Beverly Wang, Behdokht Nowroozizadeh
Affiliation
*Department of Pathology and Laboratory, University of California, Irvine
Corresponding Author
Sherehan Zada, M.D, Pathology resident, Department of Pathology and Laboratory, University of California, Irvine, 10162 Barbara Ann St, Cypress, CA, 90630, Tel: 5623095544; E-mail: sherehaz@uci.edu
Citation
Zada, S., et al. Cytologic Assessment of Intraductal Papillary Neoplasm of the Bile Duct (IPNB): Case Report. (2022) Intl J Cancer Oncol 8(1): 1-3.
Copy rights
© 2022 Zada, S. This is an Open access article distributed under the terms of Creative Commons Attribution 4.0
Keywords
IPNB; Papillary Biliary Neoplasms; FNA of Biliary neoplasms; Fine Needle Aspiration; Intraductal biliary papillary neoplasms
Abstract
The intraductal papillary neoplasm of the bile duct (IPNB) is an uncommon entity that has been encountered in the extrahepatic and/or intrahepatic bile ducts. This lesion is a precursor lesion that can affect the biliary epithelium with a high rate of associated dysplasia/ invasive carcinoma.
Herein, we report a case of intraductal papillary neoplasm of the bile duct that has been diagnosed based upon the cytology specimen. A 78-year-old male presented with an incidental liver hilum mass during an imaging study. The cytological evaluation revealed clusters of biliary epithelial cells with papillary architecture and fibrovascular cores. The biliary epithelial cells demonstrate low and focal high-grade dysplasia, which in conjunction with imaging studies, are indicative of an intraductal papillary neoplasm of the bile duct. The histologic examination of the excised specimen later confirmed the primary cytology findings. To our knowledge, documentation of the diagnosis of intraductal papillary neoplasm of the bile duct, especially from a cytologic evaluation standpoint, remains limited and will herein is elucidated.
Introduction
Intraductal papillary neoplasms of the bile duct (IPNBs) are uncommon, premalignant, papillary epithelial structures with delicate fibrovascular cores arising within the intrahepatic and/or extrahepatic bile ducts. Histologically these lesions may be classified as gastric, intestinal, pancreaticobiliary, and oncocytic[1]. Imaging studies with biopsy are the most reliable method of diagnosing these entities. Cytology is a safe and effective method in the general diagnosis of biliary neoplasms; however, it is not a commonly utilized method in diagnosing intraductal lesions[2,3]. To our knowledge, limited cases in the literature have diagnosed IPNBs based upon cytological evaluation with imaging correlation. Herein, we report a case of 78 y/o male with a diagnosis of IPNB that has been called and managed in concordance with cytological evaluation. The subsequent surgical excision of the specimen has confirmed the cytology findings.
Case Presentation
A78-year-old male with a significant past medical history of hypertension, coronary artery disease, and congestive heart failure presented with gradually declining renal function of unclear etiology. The patient was diagnosed with stage III chronic kidney disease and subsequently underwent a Computed Tomography (CT) scan of the abdomen and pelvis which showed an incidental left hepatic biliary dilation with a possible mass measuring approximately 5.4 x 2.8 cm. The patient was scheduled for abdominal Magnetic Resonance Imaging (MRI) to further assess his incidental asymptomatic mass which showed a left hepatic biliary dilation and central left hepatic lobe tubular structure, hepatomegaly, and splenomegaly (Figure 1).
Figure 1: Abdominal Magnetic Resonance Imaging (MRI) With/without Contrast showed a left hepatic biliary dilation and central left hepatic lobe tubular structure.
Given the concern of cholangiocarcinoma, a diagnostic Endoscopic Retrograde Cholangiopancreatography (EUS/ERCP) with Fine Needle Aspiration (FNA) was performed and revealed an ill-defined (23mm) mass located near the liver hilum and extending into the dilated left intrahepatic ductal system and the non dilated right hepatic system. The FNA of the mass showed a cellular specimen with clusters of cuboidal to columnar epithelial cells in apapillary architecture. The biliary epithelial cells showed low and focal high-grade dysplasia (Figure 2). Based on these findings, cytologic diagnosis of papillary neoplasm of the bile duct with low-grade dysplasia and focal high-grade dysplasia was rendered. The patient subsequently underwent a laparoscopic left hepatic lobectomy with external biliary stent placement. Gross pathology revealed an ill-defined, tan-gray and homogeneous mass measuring 5.0 x 2.8 x 2.0 cm. The histologic examination confirmed the cytology diagnosis of intraductal papillary neoplasm of the bile duct with low-grade and high-grade dysplasia. No evidence of invasive carcinoma was identified in the specimen (Figure 3).
Figure 2: Cytologic features of an intraductal papillary neoplasm of the bile duct.
A (20X) &B (4X): Three-dimensional branching papillary configurations with focal high-grade dysplasia (Diff-Quick stain).
C (20X) &D (4X): Atypical cells with a high nuclear‐cytoplasmic ratio, hyperchromasia, and irregular nuclear membranes characteristic of high‐grade dysplasia (Papanicolaou stain).
Figure 3:
A: H&E 2X demonstrating intraductal papillary neoplasm of the bile duct
B: H&E 10X demonstrating areas with high grade dysplasia
Discussion
Intraductal papillary neoplasms of the bile duct are uncommon, premalignant neoplasms composed of papillary structures with delicate fibrovascular cores within the bile ducts[1]. The true incidence of these lesionsis not well established due to challenges in rendering the diagnosis via various modalities[1,4-6]. IPNBs usually affect the elderly (60 -66)years and are more commonly seen among males[5]. Their prevalence is higher in Eastern countries where theyaccount for almost 9.9-30 % of bile duct tumors, while accounting for 7-11 % of bile duct tumors in Western countries[5,7].
Although IPNBs may occur in both intrahepatic and extrahepatic bile ducts, they are more common in the intrahepatic ducts and on the left side[1,5,6]. Few studies reported the hilum as the most common side of these lesions[5]. The underlying etiology is still not completely established with several existing theories. The main risk factors involved in the pathogenesis of IPNBs include hepatolithiasis and Clonorchis’s infection[5,8,9]. Other possible risk factors include environmental, racial factors, and biliary malformations[8].
IPNBs can manifest clinically as abdominal pain, acute cholangitis, jaundice, and elevated liver function tests. However, some patients are asymptomatic, and their tumors are incidentally discovered during radiologic studies for different reasons[5,6]. These lesions are classified histologically into four subtypes: gastric, intestinal, pancreaticobiliary, and oncocytic[1,6]. They are also further classified according to their cytologic features and architecture into low-intermediate grade, high grade, and invasive carcinoma[6]. Laboratory studies can be helpful in the diagnostic process and may give clues to bile duct obstruction. The most commonly measured values areAspartate Amino Transferase (AST), Alanine Amino Transferase (ALT), total bilirubin, direct bilirubin, gamma-glutamyl transpeptidase, and alkaline phosphatase[10,11].
Imaging studies play an important role in the diagnostic process as IPNBs can have different manifestationsaccording to their location, size, presence or absence of mucin, and the morphologic features of the lesion[5]. Different imaging techniques can be utilized including Ultrasonography, CT scan, MRI, Cholangiography, Peroral Cholangioscopy-guided forceps biopsy, and Endoscopic Retrograde Cholangiography (ERCP)[1,10,12].
The classical features of cytology specimens include hypercellular specimens, broad and double-based epithelium, papillary architecture, and absence of invasive features[11,12]. The top differential diagnoses on cytology specimens include biliary intraepithelial neoplasia and cholangiocarcinoma.
Utilizing imaging studies with biopsy is currently the most reliable method of diagnosing IPNBs. Fine needle aspirationof the bile duct is a common, safe and efficient way ofdiagnosing biliary lesions. However, in the absence of classical features of IPNBs in the hypocellular specimens, especially papillary patterns, making a diagnosis through solely cytological evaluation is challenging.
IPNB is a premalignant lesion, with its prognosis dependent on multiple factors, namely the multiplicity of the tumor, the presence or absence of invasion, and the status of the resection margins[5,13]. The management of IPNBs can be challenging since they may be numerous,andthey tend to spread along the biliary tree[5]. Treatment options include hepatectomy with or without extra hepatic duct resection, pancreatoduodenectomy (in severe and disseminated cases), chemotherapy, and radiotherapy[1,5,9].
Conclusions
In conclusion, cytologic evaluation can be an effective modality for diagnosing IPNBs on the contingency that we obtain a FNA specimen that is adequately representative of the entire lesion. Conducting such evaluation can be immensely beneficial for the determination of the patient’s treatment course and outcome.
Statement of Ethics
An informed and written consent was obtained from the patient. The case was submitted without identifiers.
Disclosure Statement
The authors disclose that no sponsorship or funding arrangements relating to their research pose a conflict of interest.
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