[Two Operations for Intrahepatic Recurrence of Biliary Cystadenocarcinoma-A Long-Term Survivor].

We report a case of biliary cystadenocarcinoma in which long-term survival was achieved after 2 operations for intrahepatic recurrence. A 72-year-old man with biliary cystadenocarcinoma located mainly in segment 3 of the liver underwent left hepatectomy, extrahepatic bile duct resection, and lymph node dissection. Seven years and 9 months after the initial resection, he underwent partial liver resection(segment 5)for intrahepatic recurrence detected by computed tomography. Fifteen years and 7 months after the initial resection, he underwent repeat partial resection of the liver(segment 5)for intrahepatic recurrence. Histologically, these tumors were confirmed to be recurrence of biliary cystadenocarcinoma. He remains alive and well with no further recurrence 21 years and 6 months after the initial resection. This case and a literature review suggest that hepatic resection is a useful treatment option for intrahepatic recurrence of biliary cystadenocarcinoma.

Laparoscopic Left Lateral Sectionectomy Using the Extrahepatic Glissonean Approach: A Secure Option for Achieving a Negative Margin for Lesions with Ductal Extension.

INTRODUCTION: In patients with liver lesions with ductal extension, the corresponding Glissonean pedicle should be divided at its origin to achieve a negative ductal margin; however, during laparoscopic hepatectomy, it is difficult to precisely transect the liver and divide the Glissonean pedicle as planned. METHODS: We present a video of a laparoscopic left lateral sectionectomy using the extrahepatic Glissonean approach for a lesion with ductal extension. RESULTS: A 76-year-old woman presented with a cystic neoplasm in the liver segment 3 bile duct (B3). The preoperative workup suggested biliary extension of the lesion towards the origin of B3. A decision was made to perform laparoscopic left lateral sectionectomy with division of the segment 3 Glissonean pedicle (G3) at its origin, and, additionally, left hepatectomy if the B3 ductal margin turned out to be positive. During the procedure, prior to parenchymal transection, the Arantius' ligament was dissected, and G2 and G3 were extrahepatically taped. The ischemic border was visualized by clamping the isolated pedicle, and was also clearly demonstrated by indocyanine green fluorescence. After transecting the liver towards the tape, G3 was divided at its origin, and the frozen section of the ductal margin was negative for tumors. CONCLUSION: The extrahepatic Glissonean approach can help to obtain a maximal ductal margin for liver lesions with possible biliary extension, although the technique potentially poses the risk of bleeding and/or biliary injury, and requires expertise in hepatobiliary surgery. Further studies with larger sample sizes are warranted to validate the feasibility and efficacy of this strategy.

Clinicopathological analysis of borderline ovarian tumours and risk factors related to recurrence: experience of single institution.

This study was conducted to determine the demographic and clinicopathologic characteristics and evaluate the prognostic value of various factors, such as the extensiveness of surgery, related to the tumour itself and the clinical features in the recurrence of borderline ovarian tumours (BOT). We retrospectively evaluated the data of 103 patients with a borderline ovarian tumours treated at our institution between the years 2000 and 2012. The median age was 37 (16-79) years and the majority of the patients were premenopausal (76.7%). During the follow-up, 16 recurrences were observed (15.5%). The multivariate analysis showed that the micropapillary architecture and fertility sparing surgery were the only significant independent predictors for the development of a recurrence amongst all of the demographic and clinicopathological features. In our study group, we identified that the micropapillary architecture itself and the fertility sparing surgery had a significant impact on the development of a BOT recurrence. The patients who possess these features should be followed up more closely for a long time period. Impact statement What is already known on this subject? A borderline ovarian tumour is known as a recurrent disease. The recurrence rate varies between 5 and 20%. It is well known in the literature that patients treated by an oophorectomy have a relatively lower risk of development of a recurrence compared to the patients treated by cystectomy. What do the results of this study add? Although some of the clinicopathological features are shown to be risk factors for the development of a recurrence in many studies, some of the pathological-clinical and the demographic features have not been described as yet, or have been considered to be equivocal regarding the development of a recurrence. In this study, we investigate all possible demographic, pathological, and clinical factors associated with a recurrence. Not only the well-known pathological characteristics but also the new pathological parameters and clinical approaches have been investigated. For instance, microinvasion architecture and lymphadenectomy speculated in the literature as the risk factors for the development of a recurrence, have not been identified as risk factors in our study. On the other hand, our statistical analyses have revealed that micropapillary architecture should be described as a risk factor for the development of a recurrence. What are the implications of these findings for clinical practice and/or further research? We hope our study becomes influential in the literature on the field of a micropapillary architecture and the development of a recurrence. The patients carrying this feature have to be followed up very closely and carefully. Furthermore, our findings have indicated no significant relation between the performing of a lymphadenectomy and the rate of a recurrence. This result might be encouraging for the gynaecological surgeons to refrain from a lymphadenectomy for the borderline ovarian tumours.

The impact of imaging on the surgical management of biliary cystadenomas and cystadenocarcinomas; a systematic review.

BACKGROUND: 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. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

[Laparoscopic appendectomy for mucinous cystadenoma of the appendix]. / Laparoskopicheskaia appendéktomiia pri mutsinoznoi tsistadenome cherveobraznogo otrostka.

Treatment of acute and chronic appendicitis is still an actual problem. There are some rare courses of the disease besides well-known complications of appendicitis. Mucocele is one of the rarest forms of chronic appendicitis. Mucocele is not accompanied by clinical symptoms and diagnosed accidentally in more than 25% of cases. The most serious complication of mucocele of the appendix is malignization observed in up to 36% of cases. Ultrasound, CT and colonoscopy are the most effective methods of perioperative diagnosis. Unclear symptoms, delayed diagnosis and surgical treatment increase the risk of complications (i.e. cystadenocarcinoma).

Total prostatectomy as a treatment for prostatic carcinoma in 25 dogs.

OBJECTIVE: To describe the complications and outcome after total prostatectomy in dogs with histologically confirmed prostatic carcinoma. STUDY DESIGN: Multi-institutional retrospective case series. ANIMALS: 25 client-owned dogs. METHODS: Medical records of dogs undergoing total prostatectomy were reviewed from 2004 to 2016. Data retrieved included signalment, presenting signs, preoperative clinical findings, laboratory data, diagnostic imaging, surgical technique, histologic diagnosis, postoperative complications, occurrence of postoperative metastasis, and survival. RESULTS: Twenty-five dogs underwent total prostatectomy for prostatic carcinoma. Urinary anastomotic techniques included urethrourethral anastomosis in 14 dogs, cystourethral anastomosis in 9 dogs, ureterocolonic anastomosis in 1 dog, and anastomosis between the bladder neck and penile urethra in 1 dog. All dogs survived to discharge. Fifteen dogs were diagnosed with transitional cell carcinoma, 8 dogs with prostatic adenocarcinoma, 1 with prostatic cystadenocarcinoma, and 1 with an undifferentiated carcinoma. Permanent postoperative urinary incontinence was present in 8 of 23 dogs. The median survival time was shorter in dogs with extracapsular tumor extension compared with those with intracapsular tumors. The overall median survival time was 231 days (range, 24-1255), with 1- and 2-year survival rates equal to 32% and 12%, respectively. CONCLUSION AND CLINICAL SIGNIFICANCE: Total prostatectomy, combined with adjunct therapies, prolongs survival and lowers complication rates compared to previous reports of dogs with prostatic carcinoma. It should be noted, however, that case selection likely played a significant role in postoperative outcome.

Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma.

Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.

Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor.

OBJECTIVE: To characterize clinical and radiological features associated with biliary cystic tumors (BCTs) of the liver, and to define recurrence-free and overall survival. BACKGROUND: Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare tumors that arise in the liver. METHODS: Between 1984 and 2013, 248 patients who underwent surgical resection of BCA or BCAC were identified. Clinical and outcome data were analyzed. RESULTS: Median total bilirubin, CA19-9, and carcinoembryonic antigen (CEA) levels were 0.6 mg/dL, 15.0 U/mL, and 2.7 ng/mL, respectively. Preoperative imaging included computed tomography only (62.5%), magnetic resonance imaging only (6.9%), or CT + MRI (18.5%). Features on cross-sectional imaging included multiloculation (56.9%), mural nodularity (16.5%), and biliary ductal dilatation (17.7%). The presence of these factors did not reliably predict BCAC versus BCA (sensitivity, 81%; specificity, 21%). Median biliary cyst size was 10.0 cm (interquartile range, 7-13 cm). Operative interventions included unroofing/partial excision of the lesion (14.1%), less than hemihepatectomy (48.8%), or hemi-/extended hepatectomy (36.3%). On pathology most lesions were BCA (89.1%), whereas 27 (10.9%) were BCAC. At last follow-up, there were 46 (18.3%) recurrences; 2 patients who initially had BCA recurred with BCAC. Median overall survival was 18.1 years; 1-year, 3-year, and 5-year survival was 95.0%, 86.8%, and 84.2%, respectively. Long-term outcomes were associated with BCAC versus BCA, as well as the presence of spindle cell/ovarian stroma (both P < 0.05). CONCLUSIONS: Among patients undergoing surgery for BCT, associated malignancy was uncommon (10%) and no preoperative findings reliably predicted underlying BCAC. After excision of BCA, long-term outcomes were good; however, patients with BCAC had a worse long-term prognosis.

Intrahepatic biliary mucinous cystic neoplasms: clinicoradiological characteristics and surgical results.

BACKGROUND: Intrahepatic biliary mucinous cystic neoplasms are rare hepatic tumors and account for less than 5% of intrahepatic cystic lesions. Accurate preoperative diagnosis is difficult and the outcome differs among various treatment modalities.The aim of this study is to investigate the clinico-radiological characteristics of intrahepatic biliary mucinous cystic neoplasms and to establish eligible diagnostic and treatment suggestions. METHODS: Nineteen patients with intrahepatic biliary cystadenomas and two patients with biliary cystadenocarcinomas were retrospectively reviewed. Their clinico-radiological variables and survival outcome were analyzed. RESULTS: Of the 19 patients with biliary cystadenoma, 16 (84.2 %) were female. 11 (57.9 %) patients had symptoms before operation with the most common presenting symptom being abdominal pain. Among the patients with available data, serum and cystic fluid CA 19-9 levels were invariably elevated and the CA 19-9 level in the cystic fluid was significantly higher than that in the serum. Loculations (84.2 %) and septations (63.2 %) were the most common radiologic findings. For treatment, 11 (57.9 %) patients received radical resection by either enucleation or hepatic resection, while the remaining 8 (42.1 %) patients underwent only fenestration of liver cysts. Radical resection provided a significantly better clinical outcome than fenestration in terms of tumor recurrence (p = 0.018). The only two male patients with biliary cystadenocarcinoma received radical hepatic resection and achieved a disease-free survival of 16.5 months and 33 months, respectively. CONCLUSION: Intrahepatic biliary mucinous cystic neoplasms are rare and preoperative diagnosis is difficult. Internal septations and loculations on radiologic examinations should raise some suspicion of this diagnosis. Complete tumor excision is the standard treatment that may provide patients with better long term results after the operation.

[SURGICAL TREATMENT OF TUMORS OF THE LEFT PANCREATIC ANATOMICAL SEGMENT].

The results of treatment of 231 patients, suffering tumoral affection of pancreatic left anatomical segment in period of 2009-2013 yrs were analyzed. Individualized approach, using modern technologies, was applied. Radical operations were performed in 129 patients, ageing 14-81 yrs old, including pancreatic distal resections in various modifications, central resection and tumoral enucleation. Possibilities of the extended pancreatic resection performance were studied in conditions of tumoral invasion of adjacent organs, regional vessels, as well as impact of such interventions on postoperative complications and lethality rate. While performing pancreatic subtotal distal resection with simultant resection of affected main venous vessels and adjacent organs the operative intervention risk is enhanced, but possibilities of a radical operations performance in previously considered inoperable patients are expanding.

Hepatobiliary cystadenocarcinoma without mesenchymal stroma in a female patient: a case report.

BACKGROUND: Hepatobiliary cystadenocarcinoma is a rare epithelial malignant neoplasm of the liver or extrahepatic bile ducts. Early diagnosis of hepatobiliary cystadenocarcinoma is difficult because of its asymptomatic features and rarity. Moreover, the molecular pathogenesis of hepatobiliary cystadenocarcinoma remains unclear. Herein, we described a case of hepatobiliary cystadenocarcinoma in female with chronic hepatitis B and repeated hepatolithiasis. CASE PRESENTATION: A 65-year-old woman with medical history of latent hepatitis B virus infection, repeated choledocholisthiasis, and cholecystitis was admitted due to a heterogeneous cystic mass (5.6 cm × 4 cm) shown on abdominal ultrasonography during regular physical checkup. The patient complained about irregular bowel movements with intermittent diarrhea for two months before presentation. Computed tomography (CT) disclosed a multiloculated cystic lesion in the left hepatic lobe with the presence of intraductal stones and dilatation of intrahepatic ducts. Histological results obtained from left lobectomy specimens showed hepatobiliary cystadenocarcinoma without accompanied mesenchymal stroma. CONCLUSION: Notably, hepatobiliary cystadenocarcinoma without mesenchymal stroma seldom occurs in women and is usually associated with poor prognosis. We present the rare findings in this patient and suggest that chronic inflammatory insults in the intrahepatic bile ducts might shed light on the cystadenocarcinogenesis.

Validity of intraoperative diagnosis at laparoscopic surgery for ovarian tumors.

STUDY OBJECTIVE: To evaluate the accuracy and usefulness of intraoperative diagnosis of ovarian tumor during laparoscopic surgery. DESIGN: Retrospective cohort study (Canadian Task Force classification II-3). SETTING: Tertiary care university hospital. PATIENTS: We reviewed the cases of 262 patients who underwent laparoscopic surgery at our institution between January 2005 and December 2011 in whom a benign ovarian tumor was diagnosed intraoperatively. INTERVENTIONS: Intraoperative pathologic assessment of frozen sections. MEASUREMENTS AND MAIN RESULTS: Intraoperative diagnosis of ovarian tumors demonstrated sensitivity of 80%, specificity of 99.6%, positive predictive value of 80%, and diagnostic accuracy of 99.2%. Mucinous tumors diagnosed intraoperatively showed differing intraoperative and final pathologic diagnoses significantly more frequently than did other types of tumors. CONCLUSION: Intraoperative pathologic assessment of benign ovarian tumors during laparoscopic surgery is reliable. However, clinicians should recognize that it is possible to make an incorrect diagnosis in some situations and should exercise caution accordingly.

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

We report two cases of cystic neoplasm of the liver with mucinous epithelium in which both ovarian-like stroma and bile duct communication were absent. The first case was a 41-year-old woman. She underwent right trisegmentectomy due to a multilocular cystic lesion, 15 cm in diameter, with papillary nodular components in the medial segment and right lobe. Histologically, arborizing papillae were seen in the papillary lesion. The constituent neoplastic cells had sufficient cytoarchitectural atypia to be classified as high-grade dysplasia. The second case was a 60-year-old woman. She underwent left lobectomy due to a unilocular cystic lesion, 17 cm in diameter, 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 found throughout the resected specimen. According to the most recent World 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 found, 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-like stroma and bile duct communication is possible in mucinous cystic tumors of the liver. Herein, we have described the clinicopathologic features of the two cases and reviewed past cases in the literature.

Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver.

Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare biliary duct neoplasms. This study investigated reasonable management strategies of cystic neoplasms in the liver. Charts of 39 BCA/BCAC patients (9 males, 30 female; median age 53.74 ± 14.50 years) who underwent surgery from January 1999 to December 2009 were reviewed retrospectively. Cyst fluid samples of 32 BCA/BCAC patients and 40 simple hepatic cyst patients were examined for the tumor markers carbohydrate associated antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA). The most frequent symptoms were abdominal pain (N = 10), abdominal mass (N = 7), abdominal distension (N = 4), jaundice (N = 2), and fever (N = 3); the remaining patients showed no clinical symptoms. Liver resection (N = 17) or enucleation (N = 22) was performed in the 39 patients. Ultimately, 35 patients were diagnosed with intrahepatic BCA and four patients were diagnosed with BCAC. The median CA19-9 level was significantly higher in BCA/BCAC patients than in simple hepatic cyst patients. The median CEA levels in BCA/BCAC patients and controls were 6.83 ± 2.43 and 4.21 ± 2.91 mg/L, respectively. All symptoms were resolved after surgery, and only one BCAC patient showed recurrence. The incidence of intrahepatic cystic lesions was 1.7%. Increased CA19-9 levels in the cyst fluid is a helpful marker for distinguishing BCA/BCAC from common simple cysts. The presence of coarse calcifications is suggestive of BCAC. Complete surgical removal of these lesions yielded satisfying long-term outcomes with a very low recurrence rate.

Cytologic features of low-grade cribriform cystadenocarcinoma of the submandibular gland: a case report.

BACKGROUND: Low-grade cribriform cystadenocarcinomas (LGCCC) are rare salivary gland tumors, classified into a variant of cystadenocarcinoma by the 2005 WHO classification. All previously reported cases arose from parotid glands, except for a case from a minor salivary gland. We report here for the first time a case of LGCCC arising from the submandibular gland. CASE: A 65-year-old man presented with a 4-cm multicystic mass in the left submandibular gland. Smears from fine-needle aspiration cytology showed tumor cells, appearing solitarily or partly in clusters, with thick cytoplasm and central nuclei. Some clustering tumor cells showed large cytoplasmic vacuoles and peripherally dislocated nuclei. Although these findings indicated a possible mucoepidermoid carcinoma in the submandibular gland, the final diagnosis of the resected specimen was LGCCC. CONCLUSION: LGCCC can arise not only from the parotid glands, but also in the submandibular glands. LGCCC is thought to be of low-grade malignancy; no reported cases have shown tumor metastasis and there are no patients who are known to have died of this disease. Thus, differential diagnosis of this tumor from other malignant salivary gland tumors is quite important; however, this might be difficult when based solely on cytological findings.

Inferior vena cava obstruction and collateral circulation as unusual manifestations of hepatobiliary cystadenocarcinoma.

BACKGROUND: Hepatobiliary cystadenocarcinoma represents a rare epithelial malignant tumor derived from the intrahepatic bile duct. METHODS: A 71-year-old woman, who had undergone laparoscopic drainage of a cystic lesion of the right hepatic lobe, was misdiagnosed as having hepatic echinococcal disease, and received intracystic infusion of 95% ethanol four years ago. She was admitted to our hospital for further treatment. RESULTS: Physical examination revealed dilated superficial veins across the right abdominal wall. After mapping the direction of blood flow in these vessels, we assumed that this was a sign of inferior vena cava obstruction. Abdominal ultrasound, computed tomography, magnetic resonance imaging combined with magnetic resonance angiography showed a large cystic mass in the right upper quadrant and epigastrium, displacing the adjacent structures, adherent to the inferior vena cava, which was not patent, resulting in dilation of superficial epigastric veins. The patient underwent an exploratory laparotomy. Total excision of the huge mass measuring 16X15 cm was possible under selective vascular exclusion of the liver. Removal of the tumor resulted in immediate restoration of flow in the inferior vena cava. On the basis of the pathology and findings of immunohistochemical analysis, a hepatobiliary cystadenocarcinoma was diagnosed. CONCLUSIONS: In the present case, hepatobiliary cystadenocarcinoma was accompanied by dilated superficial venous collaterals due to inferior vena cava obstruction. Selective vascular exclusion of the liver allowed a safe oncological resection of the tumor.

Syringocystadenocarcinoma papilliferum: a rare tumor with a favorable prognosis.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare adenexal carcinoma with only 21 cases reported in the literature. Most patients describe a long-standing mass with recent change, supporting the idea that SCACP arises from malignant transformation of pre-existing syringocystadenoma papilliferum (SCAP). Syringocystadenocarcinoma papilliferum is generally treated with wide surgical excision of the lesion and patients do exceeding well and require no systemic therapy.

[Laparoscopic management of ovarian masses during pregnancy]. / Prise en charge coelioscopique des masses ovariennes au cours de la grossesse.

BACKGROUND: The diagnosis of an adnexal mass associated with pregnancy is increasingly common with the routine use of ultrasound during prenatal care. AIM: To assess the feasibility, advantages and limiting factors of laparoscopy in the management of ovarian masses during pregnancy. METHODS: Rretrospective study of a series of 34 pregnant women operated by laparoscopy for adnexal masses during a period of 14 years. RESULTS: The mean age of patients was 29 years. Fifty percent of patients were nulliparous. In 62% of cases, patients were asymptomatic. Laparoscopy was performed at a mean gestational age of 15 weeks. Open laparoscopy was performed in 58.8% of cases. Intraperitoneal cystectomy was performed in 28 cases (72%). In two cases (5 %), it was a borderline ovarian tumor requiring further surgery outside of pregnancy. Fetal loss was noted at a term of 16 weeks. CONCLUSION: Laparoscopic management of adnexal masses during pregnancy appears to be safe and reproducible procedure with few maternal and fetal complications.

Management of Mucinous Cystic Neoplasms of the Liver.

BACKGROUND: Mucinous cystic neoplasms of the liver (MCN-L) including biliary cystadenomas (BCA) and biliary cystadenocarcinomas (BCAC) are rare cystic lesions that comprise less than 5% of all liver cysts and affect only a small subset of individuals. We herein review the current evidence regarding the clinical presentation, imaging characteristics, tumor markers, pathological findings, clinical management, and prognosis of MCN-L. METHODS: A comprehensive review of the literature was performed using MEDLINE/Pubmed and Web of Science databases. In PubMed, the terms "biliary cystadenoma," "biliary cystadenocarcinoma," and "non parasitic hepatic cysts" were queried to identify the most recent data on MCN-L. RESULTS: US imaging, CT, and MRI, as well as consideration of clinicopathological features, are required to appropriately characterize and diagnose hepatic cystic tumors. BCA are premalignant lesions and cannot be reliably differentiated from BCAC based on imaging alone. As such, both types of lesions should be treated with margin-negative surgical resection. Following surgical resection, recurrence is fairly low among patients with BCA and BCAC. Despite having worse long-term outcomes than BCA, the prognosis following surgical resection of BCAC still remains more favorable than other primary malignant liver tumors. CONCLUSION: MCN-L are rare cystic liver tumors that include BCA and BCAC, which can be difficult to differentiate based on imaging alone. Surgical resection remains the mainstay of management for MCN-L with recurrence being generally uncommon. Future multi-institutional studies are still required to better understand the biology behind BCA and BCAC to improve the care of patients with MCN-L.