Vascular hamartomas represent a focal proliferation of disorganized vascular tissue, which is usually present at birth. An 8-month-old Scottish fold female cat presented with abdominal distention, mild dyspnea, pale mucous membranes, and lethargy. Ultrasound examination revealed a hepatic mass resembling multiple cysts affecting the right medial lobe. Surgical excision was performed, and tissue samples were sent for histopathological evaluation. The mass was composed of multiple, dilated, variably-sized well-differentiated arterioles and venules, consistent with vascular hamartoma. Immunohistochemical investigation of the cells lining the cystic structures showed positive immunolabeling for vimentin and negative immunolabeling for PanCK, supporting the histological diagnosis. Based on existing literature, this represents the first case of hepatic localization of vascular hamartoma in a cat. In addition, a comparative histological study between vascular hamartoma and biliary duct hamartoma and a review on hepatic vascular hamartomas in animals and hepatic cystic masses in cats was made.

Hepatic cystadenoma is a rare disease, accounting for about 5% of all cystic lesions, with a high tendency of malignant transformation. The preoperative diagnosis of cystadenoma is difficult, and some cystadenomas are easily misdiagnosed as hepatic cysts at first. Hepatic cyst is a relatively common liver disease, most of which are benign, but large hepatic cysts can lead to pressure on the bile duct, resulting in abnormal liver function. To better understand the difference between the microenvironment of cystadenomas and hepatic cysts, we performed single-nuclei RNA-sequencing on cystadenoma and hepatic cysts samples. In addition, we performed spatial transcriptome sequencing of hepatic cysts. Based on nucleus RNA-sequencing data, a total of seven major cell types were identified. Here we described the tumor microenvironment of cystadenomas and hepatic cysts, particularly the transcriptome signatures and regulators of immune cells and stromal cells. By inferring copy number variation, it was found that the malignant degree of hepatic stellate cells in cystadenoma was higher. Pseudotime trajectory analysis demonstrated dynamic transformation of hepatocytes in hepatic cysts and cystadenomas. Cystadenomas had higher immune infiltration than hepatic cysts, and T cells had a more complex regulatory mechanism in cystadenomas than hepatic cysts. Immunohistochemistry confirms a cystadenoma-specific T-cell immunoregulatory mechanism. These results provided a single-cell atlas of cystadenomas and hepatic cyst, revealed a more complex microenvironment in cystadenomas than in hepatic cysts, and provided new perspective for the molecular mechanisms of cystadenomas and hepatic cyst.

UNASSIGNED: Intrahepatic biliary cystadenoma (IBC) is a rare benign cystic tumor of the liver. So far, it has not been comprehensively understood, which causes incorrect diagnosis, treatment confusion, and even inappropriate treatment. Here, we reviewed clinical data of IBC patients in our center, shared our experiences and lessons learned, and improved the level of diagnosis and treatment.
UNASSIGNED: The clinical data of 10 patients with pathologically diagnosed IBC, admitted to the Department of Hepatobiliary Surgery of the Affiliated Hospital of Guangdong Medical University from January, 2007, to January, 2022 were retrospectively analyzed.
UNASSIGNED: 10 patients underwent surgery and were discharged successfully. Cyst morphology: multiple cysts: 6 cases (6/10), monocular cyst: four cases(4/10). Six patients (6/10) were diagnosed as IBC preoperatively and received hepatectomy. Four patients with monocular cyst IBC underwent intraoperative frozen section examination, except one case showed IBC; the rest were misdiagnosed as simple liver cyst. In three misdiagnosed patients, one underwent open left hepatectomy seven days after the initial operation. The other patient refused to undergo reoperation and required follow-up observation. The last patient could not tolerate hepatectomy due to insufficient residual liver volume and chose follow-up observation.
UNASSIGNED: For IBC, especially monocular IBC, it is easy to be misdiagnosed as simple hepatic cyst, which brings great confusion to clinical treatment. We propose strengthening communication with pathologists to deepen understanding of IBC. Attention should be paid to the cyst wall\'s shape and the cyst fluid\'s properties during the operation to avoid the missed diagnosis, misdiagnosis, or even improper operation. For suspicious cases, directly choose hepatectomy to avoid reoperation after thoroughly evaluating the patient\'s condition.

UNASSIGNED: With advances in technology and medical treatment, laparoscopy is increasingly used in hepatic cyst surgery. We hope that the analysis the safety and efficacy of laparoscopic hepatectomy versus open hepatectomy in giant hepatic cyst surgery will provide a theoretical basis for the choice of treatment means for clinicians.
UNASSIGNED: By searching CNKI (China National Knowledge Infrastructure), Wanfang, VIP (China Science and Technology Journal Database), CBM (China Biology Medicine disc), PubMed, Embase and Cochrane Library databases, all Chinese- and English-language articles on the safety and efficacy of laparoscopic hepatectomy and open hepatectomy for hepatic cysts were collected from database establishment to December 2021. Endnote X9 software was used for data checking and screening. Stata 15.1 software was used to analyze the relevant data. Sensitivity analysis was used to assess heterogeneity and funnel plots were used to detect bias in the results.
UNASSIGNED: A total of 43 relevant studies covering 3,375 patients with hepatic cysts were included: 1,733 patients in the laparoscopic hepatectomy group and 1,642 in the open hepatectomy group. Meta-analysis showed that the laparoscopic hepatectomy group had shorter operation time [standard mean difference (SMD) =-2.27, 95% confidence interval (CI): -2.63 to -1.92, P<0.001], less intraoperative blood loss (SMD =-3.62, 95% CI: -4.22 to -3.02, P<0.001), shorter hospital stay (SMD =-2.09, 95% CI: -2.41 to -1.78, P<0.001), faster postoperative gastrointestinal function recovery (SMD =-3.94, 95% CI: -4.68 to -3.20, P<0.001), and less postoperative complications [odds ratio (OR) =0.45, 95% CI: 0.35 to 0.58, P<0.001] than the open hepatectomy group, with significant statistical differences. Sensitivity analyses were largely centered, and it indicates that the results have a high stability. The funnel plot was left-right symmetrical. It indicates that the intervention group was better than the control group and the results were reliable.
UNASSIGNED: There are significant differences in the safety and efficacy of laparoscopic hepatectomy compared with open hepatectomy. Laparoscopic hepatectomy can be selected to reduce postoperative pain. There were few foreign studies included, so whether our results apply to European and American populations still needs further study.

Hepatic cysts are found in heterogeneous disorders with different pathogeneses, of which simple hepatic cysts and polycystic liver diseases are two major types. The process of hepatic cytogenesis for these two diseases is caused by defects in remodelling of the ductal plate during biliary tract development, which is called ductal plate malformation. SOX9 is a transcription factor participating in the process of bile duct development, and thus, its dysregulation may play important roles in hepatic cystogenesis. SEC63 encodes an endoplasmic reticulum membrane protein that is mutated in human autosomal dominant polycystic liver disease. However, the transcriptional regulation of SEC63 is largely unknown. In the present study, a liver-specific Sox9 knockout (Sox9LKO ) mouse was generated to investigate the roles and underlying mechanism of SOX9 in hepatic cystogenesis. We found that hepatic cysts began to be observed in Sox9LKO mice at 6 months of age. The number and size of cysts increased with age in Sox9LKO mice. In addition, the characteristics of hepatic cytogenesis, including the activation of proliferation, absence of primary cilium, and disorder of polarity in biliary epithelial cells, were detected in the livers of Sox9LKO mice. RNAi silencing of SOX9 in human intrahepatic biliary epithelial cells (HIBEpic) resulted in increased proliferation and reduced formation of the primary cilium. Moreover, Sec63 was downregulated in primary biliary epithelial cells from Sox9LKO mice and SEC63 in HIBEpic transfected with siSOX9. Chromatin immunoprecipitation assays and luciferase reporter assays further demonstrated that SOX9 transcriptionally regulated the expression of SEC63 in biliary epithelial cells. Importantly, the overexpression of SEC63 in HIBEpic partially reversed the effects of SOX9 depletion on the formation of primary cilia and cell proliferation. These findings highlight the biological significance of SOX9 in hepatic cytogenesis and elucidate a novel molecular mechanism underlying hepatic cytogenesis. © 2021 The Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

UNASSIGNED: Isolated autosomal-dominant polycystic liver disease (ADPLD) is generally considered a rare disease. However, the frequency of truncating mutations to ADPLD genes in large, population sequencing databases is 1:496. With the increasing use of abdominal imaging, incidental detection of hepatic cysts and ADPLD has become more frequent. The present study was performed to ascertain the incidence and point prevalence of ADPLD in Olmsted County, MN, USA, and how these are impacted by the increasing utilisation of abdominal imaging.
UNASSIGNED: The Rochester Epidemiology Project and radiology databases of Mayo Clinic and Olmsted Medical Center were searched to identify all subjects meeting diagnostic criteria for definite, likely, or possible ADPLD. Annual incidence rates were calculated using incident cases during 1980-2016 as numerator, and age- and sex-specific estimates of the population of Olmsted County as denominator. Point prevalence was calculated using prevalence cases as numerator, and age- and sex-specific estimates of the population of Olmsted County on 1 January 2010 as denominator.
UNASSIGNED: The incidence rate and point prevalence of combined definite and likely ADPLD were 1.01 per 100,000 person-years and 9.5 per 100,000 population, respectively. Only 15 of 35 definite and likely incident ADPLD cases had received a diagnostic code, and only 8 had clinically significant hepatomegaly. The incidence rates were much higher when adding possible cases, mainly identified through radiology databases, particularly in recent years and in older patients because of the increased utilisation of imaging studies.
UNASSIGNED: Clinically significant isolated ADPLD is a rare disease with a prevalence <1:10,000 population. The overall prevalence of ADPLD, however, to a large extent not clinically significant, is likely much higher and closer to the reported genetic prevalence.
UNASSIGNED: Isolated autosomal-dominant polycystic liver disease (ADPLD) is generally considered a rare disease. However, we demonstrate that it is a relatively common disease, which is rarely (<1:10,000 population) clinically significant.

Feline polycystic kidney disease (PKD), an inherited autosomal dominant disease, has been reported to occur mostly in Persian or Persian related cats, and to be associated with a mutation from C to A at position 10063 in exon 29 of the feline PKD1 gene (PKD1 mutation). Many clinical cases have been recognized in Japan, but the mutation rate in cats has not been reported. The objective of this study was to determine epidemiological characteristics and clinical features in cats with the PKD1 mutation. Referring veterinarians sent blood samples of 377 cats for the PKD1 gene evaluation. The blood samples were from 159 cats with renal cysts confirmed by ultrasonography, 60 cats without renal cysts, and 158 cats that did not undergo ultrasonography. In total, 150 cats carried the PKD1 mutation and the signalment, site and number of renal cysts, and results of blood test were evaluated in cats with the PKD1 mutation. The breeds with the highest rate of the PKD1 mutation were Persian (46%), Scottish Fold (54%) and American Shorthair cats (47%). However, mixed breed cats also showed high rates of the PKD1 mutation. Of cats with the mutation, the incidence of high plasma creatinine (≥1.6 mg/dl) was greater in cats ≥3 years old, although a few cats ≥9 years of age had low plasma creatinine (<1.6 mg/dl). The coincidence of renal and hepatic cysts was 12.6%, with the high prevalence in Persian cats (31%).

BACKGROUND: We present the short-term outcomes of robotic fenestration of symptomatic liver cysts using the EndoWrist One Vessel Sealer.
METHODS: Data from patients who underwent robotic deroofing were collected and analysed retrospectively.
RESULTS: A total of 17 patients were treated. Mean cyst size was 14 cm (median 15 cm, range 6.3-24). Seven cysts were in posterosuperior or central segments. There were no mortalities or conversions. Blood loss was minimal in all but one case of 200 ml. Mean operating time was 174 minutes (median 170 min, range 97-335). Mean hospital stay was 2.5 days (median 2 days, range 1-10). One patient developed a bile leak requiring ERCP. There are no recurrences with a median follow-up of 19 months.
CONCLUSIONS: Robotic fenestration can be safely performed and offers distinct advantages over the laparoscopic approach in the treatment of posterosuperior and perihilar cysts at the expense of longer operating times and increased cost.

Laparoscopic fenestration is one of the treatment options for symptomatic hepatic cysts, either solitary or in context of polycystic liver disease (PLD), but indications, efficacy and surgical techniques are under debate.
A systematic literature search (1950-2017) of PubMed, Embase, Web of Science and the Cochrane Library was performed (CRD42017071305). Studies assessing symptomatic relief or symptomatic recurrence after laparoscopic fenestration in patients with symptomatic, non-parasitic, hepatic cysts were included. Complications were scored according to Clavien-Dindo. Methodological quality was assessed by Newcastle-Ottawa scale (NOS) for cohort studies. Pooled estimates were calculated using a random effects model for meta-analysis.
Out of 5277 citations, 62 studies with a total of 1314 patients were included. Median NOS-score was 6 out of 9. Median follow-up duration was 30 months. Symptomatic relief after laparoscopic fenestration was 90.2% (95% CI 84.3-94.9). Symptomatic recurrence was 9.6% (95% CI 6.9-12.8) and reintervention rate was 7.1% (95% CI 5.0-9.4). Post-operative complications occurred in 10.8% (95% CI 8.1-13.9) and major complications in 3.3% (95% CI 2.1-4.7) of patients. Procedure-related mortality was 1.0% (95% CI 0.5-1.6). In a subgroup analysis of PLD patients (n = 146), symptomatic recurrence and reintervention rates were significantly higher with respective rates of 33.7% (95% CI 18.7-50.4) and 26.4% (95% CI 12.6-43.0). Complications were more frequent in PLD patients, with a rate of 29.3% (95% CI 16.0-44.5).
Laparoscopic fenestration is an effective procedure for treatment of symptomatic hepatic cysts with a low symptomatic recurrence rate. The symptomatic recurrence rate and risk of complications are significantly higher in PLD patients.

OBJECTIVE: To explore the outcomes associated with fetal hepatobiliary cysts.
METHODS: MEDLINE and EMBASE were searched for studies reporting on outcomes of fetal hepatobiliary cysts. Outcomes observed were resolution/reduction and increase in cyst size, associated congenital anomalies of the biliary tract and liver, abnormal postnatal liver function tests, clinical symptoms, need for surgery, postsurgical complications and predictive accuracy of prenatal ultrasound in identifying correctly hepatobiliary cysts. Meta-analysis of proportions was used to analyze the data.
RESULTS: The search identified 1498 articles, and 22 studies (252 fetuses) were included in the systematic review. For fetal hepatic cysts, resolution or reduction in cyst size either pre- or postnatally occurred in 59.3% (95% CI, 30.9-84.7%) of cases, while an increase in cyst size occurred in 8.7% (95% CI, 1.1-22.4%). No case of hepatic cyst had associated malformations of the biliary tract at birth. Clinical symptoms occurred in 14.8% (95% CI, 6.3-26.1%) of cases and, in 5.4% (95% CI, 0.9-13.6%), they were related to the presence of bile obstruction due to compression of the cyst on the biliary tract. No case of hepatic cyst had abnormal liver function at birth. For fetal biliary cysts, resolution or reduction in cyst size occurred in 8.7% (95% CI, 2.7-17.5%) of cases and an increase in size occurred in 34.4% (95% CI, 20.5-49.8%). Congenital anomalies of the biliary tract and liver, such as fibrosis, occurred in 21.5% (95% CI, 10.2-35.6%) and 17.4% (95% CI, 5.4-34.4%) of cases, respectively. 57.3% (95% CI, 33.9-79.0%) of cases showed impairment in liver function after birth, while 55.0% (95% CI, 37.5-71.9%) showed clinical symptoms, mainly due to bile obstruction (47.9% (95% CI, 29.4-66.7%)). Postsurgical complications occurred in 10.9% (95% CI, 3.7-21.3%) of operated cases. Risk assessment according to different cut-offs of cyst size could not be performed in view of the very small number of included studies.
CONCLUSIONS: Fetal hepatic cysts are benign, with a low likelihood of associated anomalies of the hepatobiliary tract, abnormal liver function or clinical symptoms. Congenital biliary cysts are associated with a high rate of progression, abnormal liver function after birth and clinical symptoms. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.