Peribiliary Cysts a Systematic Review and Proposal of a Classification Framework

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Published: 21 July 2014

Cystic tumor of the liver without ovarian-like stroma or bile duct advice: 2 case reports and a review of the literature

Masahiro Shinoda¹,
Yohei Masugi^two,
Osamu Itano¹,
Yoko Fujii-Nishimura^two,
Akihisa Ueno³,
Minoru Kitago¹,
Taizo Hibi¹,
Yuta Abe^one,
Hiroshi Yagi^ane,
Akihiro Tanimoto³,
Minoru Tanabe⁴,
Michiie Sakamaoto² &
Yuko Kitagawa^ane

World Journal of Surgical Oncology volume 12, Article number:229 (2014) Cite this article

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Abstruse

We report two cases of cystic neoplasm of the liver with mucinous epithelium in which both ovarian-like stroma and bile duct advice were absent. The get-go case was a 41-yr-old adult female. She underwent correct trisegmentectomy due to a multilocular cystic lesion, 15 cm in bore, with papillary nodular components in the medial segment and right lobe. Histologically, arborizing papillae were seen in the papillary lesion. The elective neoplastic cells had sufficient cytoarchitectural atypia to exist classified as high-grade dysplasia. The 2nd instance was a 60-yr-one-time woman. She underwent left lobectomy due to a unilocular cystic lesion, 17 cm in bore, in the left lobe. Histologically, the cyst wall was lined by low columnar epithelia with slight cellular atypia. In both cases, neither ovarian-like stroma nor bile duct communications were establish throughout the resected specimen. Co-ordinate to the well-nigh recent Globe Health Organization (WHO) classification in 2010, cystic tumors of the liver with mucinous epithelium are classified as mucinous cystic neoplasms when ovarian-like stromata are constitute, and as intraductal papillary neoplasm of bile duct when bile duct communication exists. Therefore, we diagnosed the cystic tumors as 'biliary cystadenoma' according to the past WHO classification scheme from 2000. We believe that the combined absence of both ovarian-similar stroma and bile duct communication is possible in mucinous cystic tumors of the liver. Herein, we have described the clinicopathologic features of the 2 cases and reviewed by cases in the literature.

Background

The disease entity 'biliary cystadenoma/adenocarcinoma' was described in the Earth Wellness Organisation (WHO) Nomenclature of Tumors 3^rd Edition, published in 2000 [1]. The disease is rare, commonly slow-growing, occurs predominantly in middle-anile women, and is characterized by multilocular cystic tumors filled with mucinous fluid. Microscopically, a single layer of mucin-secreting cells lines the cyst wall. However, the diagnostic criteria for biliary cystadenoma/cystadenocarcinoma have been vague. Cases of biliary cystadenoma/cystadenocarcinoma lacking ovarian-like stroma (Os) accept been reported, suggesting that the diagnosis of cystadenoma or cystadenocarcinoma was not necessarily fabricated based on the presence of Bone [2, three]. Given this confusion, the latest WHO Classification of Tumors for the digestive system, published in 2010, proposed that the disease entity previously designated as 'biliary cystadenoma/adenocarcinoma' should instead exist classified equally either mucinous cystic neoplasm (MCN) or intraductal papillary neoplasm of bile duct (IPN-B) depending on the presence of Bone and bile duct advice (BDC), respectively [4]. Herein, nosotros report two cases of cystic neoplasm of the liver with mucinous epithelium in which both OS and BDC were absent, and which could not exist clearly classified every bit either MCN or IPN-B. We encountered the commencement instance afterward publication of the WHO classification organization in 2010. The encounter with this case led u.s.a. to survey our past cases that had been diagnosed as biliary cystadenoma or biliary cystadenocarcinoma co-ordinate to the WHO classification system in 2000. We pathologically re-examined specimens of the past cases and identified another case without either Bone or BDC. We report this past instance equally the second of the ii case reports. We also reviewed past cases in the literature and talk over bug regarding the diagnostic criteria for cystic neoplasms of the liver in the absence of OS and BDC.

Case presentation

Example ane

A 41-year-sometime Japanese adult female had undergone laparoscopic deroofing due to a liver cyst in the right lobe in 2009 at some other infirmary. After the functioning, the cystic lesion could still exist confirmed by ultrasonography, and the patient subsequently underwent follow-upwardly for blood analysis and ultrasonography every six months for three years. Recent ultrasonography revealed an increase in the size of the cystic lesion. The patient was referred to our hospital for further examination in 2012. Since the patient had a medical history of iodine hypersensitivity, we could not use contrast-enhanced computed tomography and endoscopic retrograde cholangiography (ERC) for the examinations. Computed tomography without contrast enhancement revealed a multilocular cystic lesion, 15 cm in diameter, in the medial segment and correct lobe (Figure 1A). T2-weighted magnetic resonance imaging as well clearly revealed the cystic lesion (Figure 1B). Dissimilarity-enhanced magnetic resonance imaging showed that the lesion independent multiple papillary nodular components with enhancement (Figure 1C). Diffusion-weighted magnetic resonance imaging showed diffusion restriction in the nodular components (Figure 1D). Ultrasonography with Sonazoid™ (Daiichi-Sankyo, Tokyo, Japan) revealed an enhancement corresponding to the solid component that was evident by diffusion-weighted magnetic resonance imaging. No evidence of metastases to the lymph nodes or other organs was constitute in the preoperative images. The extrahepatic bile duct was not dilated in the images. Aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, gamma-glutamyltransferase, albumin, and full bilirubin levels were all inside normal limits. Serology tests were negative for hepatitis B and C viruses. The serum levels of carcinoembryonic antigen and CA19-9 were inside normal limits. According to the pathology report from the patient's previous hospital, the resected cyst wall was lined by elementary columnar epithelium with papillary alter, no cancerous changes were axiomatic, and Os was absent in the specimen. The diagnosis in the report was cystadenoma. From the information contained in the previous report and the findings from imaging at our infirmary, we could non diagnose the cystic lesion as either MCN or IPN-B, just we suspected that the solid component in the cystic lesion had a malignancy and performed correct trisegmentectomy. Ten days after surgery, she adult bile leakage and underwent endoscopic retrograde nasobiliary drainage. The postoperative course was otherwise uneventful and the patient was discharged on postoperative twenty-four hours 64. The patient is notwithstanding alive at 19 months postoperatively.

Macroscopically, the cystic lesion, 130 by 90 mm in size, was multilocular and had a solid lesion within (Figure 2A) with a big amount of mucin. Whole liver specimens were cut into five to 10 mm thick pieces, which were and then sliced into v-μm sections. Histologically, the tumor showed papillary nodular growth in several cysts (Figure 2B). The tumor had circuitous and arborizing papillae with thin-walled vessels. The papillae or cysts were lined by multilayers of cuboidal to columnar cells with abundant eosinophilic cytoplasm forming intraepithelial lumina. The neoplastic cells had round, large, and adequately compatible nuclei with unmarried prominent nucleoli. There was no evidence of stromal invasion; even so, the tumor had sufficient cytoarchitectural atypia to exist classified every bit having high-form dysplasia (Figure 2C). All sections were examined carefully, only no Os were seen throughout the cyst walls (Effigy 2d, Eastward). BDC was too not evident throughout the specimens. Immunohistochemical analyses revealed that neoplastic cells were positive for MUC-5 AC and MUC-half-dozen simply not for MUC-1, MUC-2, or CDX-two. Histological and immunohistochemical findings both suggested that the tumor could exist classified histologically as the oncocytic type. Since both Os and BDC were not detected in the specimen and ERC was not employed in this example, nosotros diagnosed the cystic tumor as biliary cystadenoma, based on the WHO nomenclature system from 2000 [1].

Case ii

A 60-yr-old Japanese woman came to our hospital in 2004 complaining of abdominal discomfort. Computed tomography with contrast enhancement revealed a unilocular cystic lesion, 17 cm in bore, in the left lobe of the liver preoperatively. No nodular components were identified in the cystic lesion and no dissimilarity enhancement was observed in the cyst wall. T2-weighted magnetic resonance imaging revealed septum-like structures in the cystic lesion (Figure 3). Ultrasonography too showed floating septum-like structures in the lesion. ERC was non scheduled. She had been afebrile before the operation. The white claret cell count and levels of C-reactive poly peptide, carcinoembryonic antigen, and CA19-nine were inside normal limits. Based on this preoperative data, the cystic lesion was diagnosed equally a simple cyst or biliary cystadenoma and the attending surgeon scheduled an operation for cyst fenestration or left lobectomy. At the start of the operation, the surgeon punctured the cyst and constitute that the fluid was turbid chocolate-brown. Although the full bilirubin concentration in the fluid was 2.one mg/dl and cytological examination showed no atypical or malignant cells, the surgeon worried well-nigh the possibility of infection in the lesion and its spread to the abdominal crenel, so a left lobectomy was performed. The postoperative course was uneventful and the patient was discharged on postoperative day 16. The patient remains alive at 9 years postoperatively.

Macroscopically, the cystic lesion, 200 by 150 mm in size, was unilocular, had no solid lesion inside, and did not contain mucin. Whole liver specimens were cut into 5 to 10 mm thick pieces, which were then sliced into 5-μm sections. Histologically, the cyst wall was lined past low columnar epithelia with slight cellular atypia (Effigy 4). All sections were examined at the fourth dimension of surgery, but no OS or BDC were seen throughout the specimens. We diagnosed the cystic tumor as biliary cystadenoma based on the WHO classification system from 2000. We carefully re-examined the whole specimens once again and confirmed that at that place were no Bone or BDC throughout the specimens.

Discussion

We encountered a case of cystic neoplasm of the liver in which both OS and BDC could not be pathologically confirmed (case 1). According to the nigh contempo WHO classification, cystic neoplasms of the liver are classified as MCN with OS and without BDC, and equally IPN-B with BDC and without OS [4]; therefore, information technology is difficult to conspicuously classify the case as either of these two disease entities. We assumed that the combined absence of both Os and BDC would be possible in mucinous cystic tumors of the liver, and we surveyed other cases of cystic neoplasm of the liver with mucinous epithelium that we had seen over the past x years at our hospital. There were iii cases of cystic neoplasm of the liver with mucinous epithelium excluding the beginning case. They were diagnosed every bit biliary cystadenoma in two cases and biliary cystadenocarcinoma in one example, according to the WHO classification organisation in 2000 [1]. Nosotros pathologically re-examined specimens from these three cases; we confirmed the presence of Bone and absenteeism of BDC in two cases (one case of biliary cystadenoma and one example of biliary cystadenocarcinoma), and nosotros found a case without either Os or BDC (case 2). We believe that there may be potential cases of cystic neoplasm of the liver with mucinous epithelium in which both Os and BDC are absent.

In a search of the PubMed database, we institute 18 manufactures that addressed cystic IPN-B [v–22]. The 18 articles reported a total of 193 cases of cystic IPN-B in which BDC was confirmed in 120 cases, unclear in xi cases, and non described in 62 cases. The 11 cases in which BDC was unclear were reported in three manufactures [5–vii]. Kubota and associates reported 119 cases of IPN-B in their multi-institutional retrospective report and found seven cases of possible IPN-B without either OS or BDC fifty-fifty after their re-examination of the original sections in 2013 [5]. In 2011, Lim and assembly surveyed 87 cases with surgically resected IPN-B and identified 2 cases in which both OS and BDC were not detected [6]. In the same yr, Mano and associates reported two cases of cystic tumors of the liver without OS and BDC as possible MCN [seven]. Combining the past 11 cases and our two cases, at that place have been 13 published cases of cystic tumour of the liver without Os and BDC.

We assume that in that location are two possibilities for cases with absence of both Bone and BDC. 1 possibility is a failure to prove the presence of BDC during the diagnostic process. Presence of BDC is usually confirmed past preoperative ERC. However, BDC is ofttimes unclear in preoperative imaging examinations. If the BDCs are very thin and are filled with mucus, dissimilarity medium may not menstruation into the communicating bile duct. At that place may exist cases of iodine hypersensitivity every bit in the present case. Fifty-fifty in pathological examinations, information technology is possible that BDC cannot exist detected throughout the specimen though information technology may in fact be. It has been noted that intraoperative injection of contrast medium into the specimen may be technically difficult because resected liver specimens have many transected bile duct branches at the margins [7]. Another possibility is that all or some of the 13 cases of cystic neoplasm of the liver without both Os and BDC consist of a disease entity that is neither IPN-B nor MCN. Since Kubota and colleagues [5] provided little information regarding the clinicopathological features of subjects in their report, we summarized the features of four cases previously reported past Lim [6] and Mano [7] and 2 previous cases from our hospital (Table 1). The mean age was 57.8 years and the cohort consisted of three males and iii females. The mean diameter was 13.v cm and the diameter was greater than 10 cm in five of 6 cases. The tumor location was the left lobe in four cases, cardinal bi-segment in one instance, and right lobe in one instance. At that place were two cases of malignancy and 4 cases of depression- and/or loftier-form dysplasia. It remains to be adamant whether the features of middle- to old-age, no gender difference, relatively large size, left lobe authorisation, and malignant potential represent a disease entity that is neither IPN-B nor MCN. Farther assessment of boosted cases of cystic neoplasm of the liver without OS and BDC is needed to clarify the features of this potential disease entity.

Table 1 Literature review for the cystic tumor of the liver without ovarian-similar stroma or bile duct communication

Total size table

Conclusion

We presented two cases of cystic tumour of the liver without OS and BDC and identified four similar controversial cases from the PubMed database. Considering these six cases, nosotros conclude that it is difficult to clearly classify cystic neoplasms of the liver with mucinous epithelium into MCN or IPN-B based just on clinical or histological findings. It is important to recognize that the most recent WHO classification is based on histological nomenclature alone, which is probably unsatisfactory. We agree with the prevailing consensus that the presence of OS or BDC has become an of import differential diagnostic criterion for IPN-B and MCN, but look forwards to a amend means for diagnosing cystic neoplasm of the liver in cases where both OS and BDC are non detected during preoperative examination and analysis of resected specimens. We hope that this example presentation and literature review serve as a stimulus for further investigation to establish a method to accurately diagnose cystic neoplasms of the liver with absenteeism of Os and BDC.

Consent

Written informed consent was obtained from the patients for publication of the case written report and any accompanying images.

Abbreviations

BDC:: Bile duct advice
ER:: Estrogen receptor
ERC:: Endoscopic retrograde cholangiography
IPN-B:: Intraductal papillary neoplasm of the bile duct
MCN:: Mucinous cystic neoplasm
Os:: Ovarian-like stroma
PgR:: Progesterone receptor
WHO:: World Wellness Organization.

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Affiliations

Department of Surgery, Keio Academy, School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan

Norihiro Kishida, Masahiro Shinoda, Osamu Itano, Minoru Kitago, Taizo Hibi, Yuta Abe, Hiroshi Yagi & Yuko Kitagawa
Section of Pathology, Keio University, School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan

Yohei Masugi, Yoko Fujii-Nishimura & Michiie Sakamaoto
Department of Diagnostic Radiology, Keio University, School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan

Akihisa Ueno & Akihiro Tanimoto
Department of Surgery, Tokyo Medical and Dental Academy, 1-v-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Nihon

Minoru Tanabe

Corresponding author

Correspondence to Masahiro Shinoda.

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Competing interests

The authors declare that they take no competing interests.

Authors' contributions

NK wrote the manuscript. MS supervised the writing of the manuscript. YM, YF-Northward, and MS analyzed the pathological specimens and wrote the pathological description. OI supervised the writing of the manuscript, especially the Discussion department. AU and AT prepared the radiological images and wrote the radiological description. MK, TH, YA, and HY prepared the data from the reviewed articles. MT participated in the performance as a chief surgeon and supervised the writing of the manuscript. YK represents our surgical department and supervised the writing of the manuscript. All authors significantly contributed to this study and approved the final manuscript.

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Kishida, N., Shinoda, M., Masugi, Y. et al. Cystic tumor of the liver without ovarian-like stroma or bile duct communication: two case reports and a review of the literature. World J Surg Onc 12, 229 (2014). https://doi.org/x.1186/1477-7819-12-229

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Received: 17 November 2013
Accepted: 04 July 2014
Published: 21 July 2014
DOI : https://doi.org/10.1186/1477-7819-12-229

Keywords

Cystic tumour
Liver
Ovarian-like stroma
Bile duct communication
Mucinous cystic tumour
Intraductal papillary neoplasm of bile duct

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