MRI of Peliosis Hepatis: A Case Series Presentation With a 2022 Systematic Literature Update.

BACKGROUND: Peliosis hepatis (PH) is a rare benign condition, characterized by hepatic sinusoidal dilatation and blood-filled cystic cavities, often found incidentally, with still challenging diagnosis by imaging due to polymorphic appearance. PURPOSE: Based on a retrospective analysis of our series (12 patients) and systematic literature review (1990-2022), to organize data about PH and identify features to improve characterization. STUDY TYPE: Retrospective case series and systematic review. POPULATION: Twelve patients (mean age 48 years, 55% female) with pathology-proven PH and 49 patients (mean age 52 years, 67% female) identified in 33 studies from the literature (1990-2022). FIELD STRENGTH/SEQUENCE: 1,5-T; T1-weighted (T1W), T2-weighted (T2W), diffusion-weighted (DW), contrast-enhanced (CE) T1W imaging. ASSESSMENT: We compared our series and literature data in terms of demographic (gender/age/ethnicity), clinical characteristics (symptoms/physical examination/liver test), associated conditions (malignancies/infectious/hematologic/genetic or chronic disorders/drugs or toxic exposure) percentage. On magnetic resonance imaging lesion numbers/shape/mean maximum diameter/location/mass effect/signal intensity were compared. PH pathological type/proposed imaging diagnosis/patient follow-up were also considered. STATISTICAL TESTS: Joanna Briggs Institute (JBI) Critical Appraisal Checklist for Case Reports/Series quality assessment. Intraclass correlation and Cohen's kappa coefficients for levels of inter/intrareader agreement in our experience. RESULTS: Patients were mainly asymptomatic (92% vs. 70% in our study and literature) with associated conditions (83% vs. 80%). Lesions showed homogeneous T1W-hypointensity (58% vs. 65%) and T2W-hyperintensity (58% vs. 66%). Heterogeneous nonspecific (25% vs. 51%), centrifugal (34% vs. 8%), or rim-like centripetal (25% vs. 23%) patterns of enhancement were most frequent, with hypointensity on the hepatobiliary phase (HBP), without restricted diffusivity. Good inter- and intrareader agreement was observed in our experience. Concerning JBI Checklist, 19 out of 31 case reports met at least 7 out of 8 criteria, whereas 2 case series fulfilled 5 and 6 out of 10 items respectively. DATA CONCLUSION: A homogeneous, not well-demarcated T1W-hypointense and T2W-hyperintense mass, with heterogeneous nonspecific or rim-like centripetal or centrifugal pattern of enhancement, and hypointensity on HBP, may be helpful for PH diagnosis. Among associated conditions, malignancies and drug exposures were the most frequent. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY: Stage 2.

Hepatic Angiosarcoma with Peliosis Hepatis.

A 59-year-old woman presented to our hospital with liver dysfunction. Imaging revealed multiple lesions in the liver. The patient was diagnosed with peliosis hepatis using percutaneous and laparoscopic biopsies. However, her condition worsened with the appearance of new, obvious mass-forming lesions. Therefore, she underwent a second percutaneous biopsy of these lesions and was diagnosed with hepatic angiosarcoma. Her condition progressed rapidly, and she died two weeks after the diagnosis. Diagnosis of hepatic angiosarcoma in the early stages is difficult. It should be noted that hepatic angiosarcoma may be associated with the development of peliosis hepatis.

Haemophagocytic lymphohistiocytosis associated with bartonella peliosis hepatis following kidney transplantation in a patient with HIV.

Bacillary peliosis hepatis is a well recognised manifestation of disseminated Bartonella henselae infection that can occur in immunocompromised individuals. Haemophagocytic lymphohistiocytosis is an immune-mediated condition with features that can overlap with a severe primary infection such as disseminated Bartonella spp infection. We report a case of bacillary peliosis hepatis and secondary haemophagocytic lymphohistiocytosis due to disseminated Bartonella spp infection in a kidney-transplant recipient with well controlled HIV. The patient reported 2 weeks of fever and abdominal pain and was found to have hepatomegaly. He recalled exposure to a sick dog but reported no cat exposures. Laboratory evaluation was notable for pancytopenia and cholestatic injury. The patient met more than five of eight clinical criteria for haemophagocytic lymphohistiocytosis. Pathology review of a bone marrow core biopsy identified haemophagocytosis. A transjugular liver biopsy was done, and histopathology review identified peliosis hepatis. Warthin-Starry staining of the bone marrow showed pleiomorphic coccobacillary organisms. The B henselae IgG titre was 1:512, and Bartonella-specific DNA targets were detected by peripheral blood PCR. Treatment with doxycycline, increased prednisone, and pausing the mycophenolate component of his transplant immunosuppression regimen resulted in an excellent clinical response. Secondary haemophagocytic lymphohistiocytosis can be difficult to distinguish from severe systemic infection. A high index of suspicion can support the diagnosis of systemic Bartonella spp infection in those who present with haemophagocytic lymphohistiocytosis, especially in patients with hepatomegaly, immunosuppression, and germane animal exposures.

The HBx protein from hepatitis B virus coordinates a redox-active Fe-S cluster.

The viral protein HBx is the key regulatory factor of the hepatitis B virus (HBV) and the main etiology for HBV-associated liver diseases, such as cirrhosis and hepatocellular carcinoma. Historically, HBx has defied biochemical and structural characterization, deterring efforts to understand its molecular mechanisms. Here we show that soluble HBx fused to solubility tags copurifies with either a [2Fe-2S] or a [4Fe-4S] cluster, a feature that is shared among five HBV genotypes. We show that the O2-stable [2Fe-2S] cluster form converts to an O2-sensitive [4Fe-4S] state when reacted with chemical reductants, a transformation that is best described by a reductive coupling mechanism reminiscent of Fe-S cluster scaffold proteins. In addition, the Fe-S cluster conversions are partially reversible in successive reduction-oxidation cycles, with cluster loss mainly occurring during (re)oxidation. The considerably negative reduction potential of the [4Fe-4S]2+/1+ couple (-520 mV) suggests that electron transfer may not be likely in the cell. Collectively, our findings identify HBx as an Fe-S protein with striking similarities to Fe-S scaffold proteins both in cluster type and reductive transformation. An Fe-S cluster in HBx offers new insights into its previously unknown molecular properties and sets the stage for deciphering the roles of HBx-associated iron (mis)regulation and reactive oxygen species in the context of liver tumorigenesis.

Peliosis Hepatis with Chylous Ascites in a Dog.

A 6-year-old spayed female Toy Poodle dog was referred to the Hokkaido University Veterinary Teaching Hospital for abdominal distension. Abdominocentesis yielded ascitic fluid that had a mildly increased total protein concentration and a 2.7-fold higher triglyceride concentration than plasma, and was interpreted as chylous ascites. The patient had an enlarged liver, which contained multiple, small, nodular masses and cyst-like structures. Microscopically, these lesions were multifocal dilated spaces containing lymphocytes, endothelial cells, fibrin and islands of hepatocytes. Increased α-smooth muscle actin-positive cells were observed in hepatic sinusoids. Based on these findings, we diagnosed peliosis hepatis with chylous ascites, which is likely to have been due to lymphangiectasia and disrupted hepatic sinusoids. Neither Bartonella spp DNA nor mutations in ACVRL1 and MTM1 genes were detected, although there was a 47-fold increase in hepatic ACVRL1 expression compared with age-matched control liver. To the authors' knowledge, this is the first report of chylous ascites resulting from peliosis hepatis in any species.

Psoriatic arthritis associated with peliosis hepatis: characteristics and therapeutic management.

Peliosis hepatis is characterized by hepatic sinusoidal dilatation and multiple blood-filled cystic cavities within the liver parenchyma. It can be due to infectious diseases, immunological disorders, neoplasia, and the use of various kinds of drugs. We presented the case of a nonsmoker 55-year-old man who complained about a 5-month history of arthritis. Medical history was consistent with psoriasis and hypertension. He denied any drug use or alcohol consumption. Physical examination showed extended psoriatic lesions. He had arthritis of the knees, ankles, wrists, and elbows. His body mass index was 22 kg/m2. Laboratory findings revealed an increased serum gamma-glutamyl transferase level (1014 UI/L, normal value (N) 11-55) and total alkaline phosphatase (278 U/L, N 30-171). Hepatitis A, B, and C serologic test results were negative. Anti-nuclear antibodies, anti-Ro/SSA, anti-GP210, anti-SP100, anti-SLA, anti-LKM1, anti-M2, anti-LC1, and anti-PML were also negative. Histopathological examination of a liver biopsy specimen revealed peliosis hepatis.The pelvic radiograph showed bilateral ankylosis of sacroiliac joints. Hand and foot radiographs showed periosteal bone apposition. The diagnosis of psoriatic arthritis associated with peliosis hepatis was made. The patient received infliximab (5 mg/kg) with a significant improvement after 3 months of follow-up. Peliosis hepatis should be considered as a possible etiology of liver enzyme abnormalities in patients with psoriatic arthritis. We highlighted the effectiveness and safety of the TNF inhibitors in the treatment of peliosis hepatis associated with psoriatic arthritis. Key Points • Peliosis hepatis should be considered as a possible etiology of liver enzyme disturbance in patients with psoriatic arthritis. • Special caution should be advised in the management of psoriatic arthritis associated with peliosis hepatis to avoid the worsening of liver function. • Infliximab is suggested as a possible treatment of peliosis hepatis associated with psoriatic arthritis.

Peliosis Hepatis in a Child with X-Linked Myotubular Myopathy Treated with Living-Donor Liver Transplant: A Case Report.

BACKGROUND: Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. CASE REPORT: We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis. CONCLUSIONS: Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

Peliosis hepatis complicated by portal hypertension following renal transplantation.

Peliosis hepatis is a rare benign disease, but in last years the number of identified cases has increased. This disease is known to be sometimes accompanied by hepatocellular carcinoma. In the recent article, Yu et al describe a case of liver peliosis, characterized by an increased proliferative index. Therefore, additional diagnosis of patients should include analyzing other tumor markers expression in order to assess the risk of malignant cell transformation in peliosis hepatis.

Living-donor liver transplantation for liver hemorrhaging due to peliosis hepatis in X-linked myotubular myopathy: Two cases and a literature review.

X-linked myotubular myopathy (MTM) (OMIM 310400) is a severe neuromuscular disorder caused by mutations in the myotubularin (MTM1) gene. Liver hemorrhaging due to peliosis hepatis (PH) is a fatal complication. We herein report 2 successful cases of living-donor liver transplantation (LDLT) for MTM patients due to liver hemorrhaging caused by PH and review previous reports. A boy who was 9 years and 4 months old initially underwent left lateral segmentectomy due to massive hepatic and intraperitoneal hemorrhaging. As bleeding from the remnant liver continued after hepatectomy, this patient emergently underwent LDLT using a left lateral segment graft from his father. Another boy who was 1 year and 7 months old underwent transcatheter arterial embolization due to hepatic hemorrhaging and was referred to our hospital for LDLT using a left lateral segment graft from his father. The pathological findings in both cases showed sinusoidal dilatation with degenerative changes in reticular fiber and hematoma in the explanted liver, which were consistent with PH associated with MTM. LT should be considered as a treatment option for patients with episodes of hepatic hemorrhaging due to MTM in order to protect against fatal bleeding.

A rare case of peliosis hepatis in a patient with chronic renal failure and renal cell carcinoma.

Peliosis hepatis (PH) is a rare disease characterized by the presence of sinusoidal dilation and blood-filled cysts throughout the hepatic parenchyma. We report a case of PH in a 49-year-old woman with chronic renal failure (CRF) on hemodialysis and with renal cell carcinoma (RCC). Dynamic contrast-enhanced computed tomography (CT) showed a 35-mm-diameter, hypervascular tumor in the liver and RCC in the right renal cyst. Ultrasound and superparamagnetic iron oxide-enhanced magnetic resonance imaging were also performed; however, the liver tumor could not be distinguished from the metastasis of RCC. Therefore, echo-guided biopsy of the liver tumor using an 18-G Majima needle was performed. Histological evaluation of the specimen showed irregular sinusoidal dilatation and blood-filled cavities without malignant cells. She was ultimately diagnosed with PH. Subsequently, she underwent total right nephrectomy for RCC and was diagnosed with RCC stage 1 (pT1N0M0). A follow-up CT performed 4 months after nephrectomy showed no growth of PH. Although the development of PH in patients with CRF or RCC who do not undergo renal transplantation is extremely rare, it should be considered in the differential diagnosis to distinguish PH from the metastasis of RCC.

Huge peliosis hepatis mimicking cystic echinococcosis: A case report.

RATIONALE: Peliosis hepatis (PH), which is characterized by blood-filled cavities in the liver, is a rare disease. Its diagnosis depends on postoperative pathological examinations and immunohistochemistry. PATIENT CONCERNS: A 44-year-old female complained of right-middle upper abdominal pain and distension for 1 month, with occasional vomiting and fever. DIAGNOSIS: Because of the similar imaging features, the patient was initially misdiagnosed as cystic echinococcosis (CE). The immunoassay of echinococcosis was negative. Irregular hepatectomy was performed. Eventually, the patient was diagnosed with PH based on postoperative histopathology and immunohistochemistry. INTERVENTIONS: The patient underwent hepatectomy. Then, the cystic lesion was collected for intraoperative pathological examination. Thus, the blood liquid was extracted from the cystic lesion. Pringle maneuver was administered to prevent bleeding, and then the whole cystic lesion was removed. OUTCOMES: She recovered smoothly and there was no relapse occurred during 6 months' follow-up. LESSONS: It is difficult to differentiate PH from CE and other hepatic diseases due to the lack of special imaging features. Pathological examinations and immunohistochemistry can provide a confirmed diagnosis of PH.

An autopsy case of peliosis hepatis with X-linked myotubular myopathy.

This report describes the autopsy case of a 4-year-old boy who died from hepatic hemorrhage and rupture caused by peliosis hepatis with X-linked myotubular myopathy. Peliosis hepatis is characterized by multiple blood-filled cavities of various sizes in the liver, which occurs in chronic wasting disease or with the use of specific drugs. X-linked myotubular myopathy is one of the most serious types of congenital myopathies, in which an affected male infant typically presents with severe hypotonia and respiratory distress immediately after birth. Although each disorder is rare, 12 cases of pediatric peliosis hepatis associated with X-linked myotubular myopathy have been reported, including our case. Peliosis hepatis should be considered as a cause of hepatic hemorrhage despite its low incidence, and it requires adequate gross and histological investigation for correct diagnosis.

Liver haematoma as a presentation of peliosis hepatis.

Peliosis hepatis (PH) is an uncommon, benign hepatic vascular condition involving liver lesions consisting of dilated sinusoidal spaces that ultimately lead to numerous blood-filled cavities within the liver parenchyma. We detail the case of a 62-year-old woman who presented with a large hepatic haematoma resulting from intracystic haemorrhage. She was subsequently diagnosed with PH via arteriogram. Her symptoms were successfully treated surgically with open fenestration. Patient's postoperative course was unremarkable, with no recurrence of symptoms to date.

[Magnetic resonance imaging and diffusion weighted imaging features of focal peliosis hepatis].

Objective: To investigate the MRI and diffusion weighted imaging (DWI) features of focal peliosis hepatis. Methods: The clinical data and MRI of 19 cases with focal peliosis hepatis confirmed by pathology from January 2012 to March 2018 in Zhongshan Hospital of Fudan University were retrospectively analyzed. The number, location, size, shape, signal intensity of plain scan of lesions, enhancement pattern of lesions, vessels within lesions, and perfusion disorders of hepatic parenchyma were analyzed. The apparent diffusion coefficient (ADC) values of the lesions and adjacent hepatic parenchyma were measured, then the differences between them were explored statistically. All 24 lesions were categorized into group A with tumor-related chemotherapy and group B without tumor-related chemotherapy. The differences of MR features between the two groups were explored statistically. Results: In all 24 lesions, 22 lesions were located in the right lobe, 2 lesions in the left lobe. The median size was 7.5-72.0 (24.4±17.2) mm.On T(1)WI,21 lesions showed slightly hypointensity, 1 lesion showed slightly hyperintensity and 2 lesions were isointensity; all 24 lesions showed slightly hyperintensity on T(2)WI, and isointensity or slightly hyperintensity on DWI. The mean ADC value was (1.511±0.415)×10(-3) mm(2)/s in the lesions and (1.769±0.690)×10(-3) mm(2)/s in the adjacent hepatic parenchyma, which showed no difference between the two groups (P>0.05). On dynamic MR images, 20 lesions showed gradually filling enhancement, 4 lesions showed markedly and persistent enhancement. Punctiform or filiform vessels were found in 9 lesions. Adjacent hepatic perfusion disorders showed in 8 lesions. The median lesion size was 7.5-38.5(17.6±9.8) mm in the tumor-related-chemotherapy group and 9.0-72.0(33.8±21.2) mm in the no chemotherapy group.There was significant difference between the two groups (P<0.05). Conclusions: The MRI performance of focal peliosis hepatis had a certain characteristic. MRI combined with diffusion weighted imaging could help to make diagnoses.

Peliosis Hepatis Simulating Metastatic Liver Disease on FDG PET/CT.

A 20-year-old man with gastrointestinal symptoms and weight loss underwent FDG PET/CT, which revealed multiple hypermetabolic hepatic lesions concerning for metastatic liver disease. The outcome of liver biopsy was consistent with the diagnosis of peliosis hepatis which is a rare benign disease characterized by multiple blood-filled cystic spaces in the hepatic parenchyma. The findings of peliosis on FDG PET/CT are not well reported in the literature. These interesting images emphasize the importance of including peliosis hepatis in the differential diagnosis of multiple hypermetabolic hepatic lesions on FDG PET/CT, which could simulate malignancy.