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.

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 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.

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.

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.

Peliosis hepatis and systemic lupus erythematosus: A rare condition identified by magnetic resonance imaging.

Peliosis hepatis is a rare benign disorder characterized by the presence of multiple cavities filled with blood with no preferential localization in the liver parenchyma. It may be related to several etiologic conditions, especially infections and toxicity of immunosuppressive drugs. To our knowledge, there are only three articles reporting the association between peliosis hepatis and systemic lupus erythematosus. In this report, we describe a case of this rare condition, highlighting the importance of magnetic resonance imaging. A short review of this subject is also presented.

Liver Peliosis: A Life-Threatening Condition With No Clear Indication for Liver Transplant.

We present a 21-year-old patient, remarkable for huge hepatomegaly with the liver, occupying almost the entire abdominal cavity, and mild portal hypertension due to splenic vein compression. After ultrasonography-guided liver biopsy, performed to establish the diagnosis, the patient had bleeding from the liver. Fortunately, emergency laparotomy was started immediately, and the patient was saved. Macroscopically, the liver appeared to be of purple-red color, flabby to the touch, and able to be easily wrinkled with fingers. When all available clinical data were considered, a diagnosis of liver peliosis was made. The patient was recommended close follow-up at the specialized liver surgery clinic with access to emergency surgical procedures, including liver transplant.

Peliosis hepatis and systemic lupus erythematosus: A rare condition identified by magnetic resonance imaging / Peliose hepática e lúpus eritematoso sistêmico: uma rara condição identificada pela ressonância nuclear magnética

Summary Peliosis hepatis is a rare benign disorder characterized by the presence of multiple cavities filled with blood with no preferential localization in the liver parenchyma. It may be related to several etiologic conditions, especially infections and toxicity of immunosuppressive drugs. To our knowledge, there are only three articles reporting the association between peliosis hepatis and systemic lupus erythematosus. In this report, we describe a case of this rare condition, highlighting the importance of magnetic resonance imaging. A short review of this subject is also presented.

Resumo Peliose hepática é uma patologia benigna rara caracterizada pela presença de múltiplas cavidades preenchidas por sangue sem localização preferencial no parênquima do fígado. Pode estar relacionada a uma série de condições etiológicas, dentre elas doenças infecciosas e toxicidade por drogas imunossupressoras. Para nosso conhecimento, existem apenas três artigos que abordam a associação entre peliose hepática e lúpus eritematoso sistêmico. Neste relato, descrevemos um caso desta rara condição, destacando a importância da ressonância magnética. Uma breve revisão sobre o tema é apresentada.

Peliosis hepatis: 2 case reports of a rare liver disorder and its differential diagnosis.

RATIONALE: Peliosis hepatis (PH) is a rare tumor-like liver lesion composed of multiple blood-filled cavities within the liver parenchyma. It is hard to differentiate PH from other liver lesions by imaging, such as carcinoma, metastases, or abscess. PATIENT CONCERNS: Here, we reported 2 cases that presented with liver lesions under ultrasound and computed tomography (CT) scanning, without any history of liver diseases or drug usage traced back. DIAGNOSES: Liver biopsy and laparoscopy were processed, and the lesions were eventually diagnosed as PH by histopathology, which microscopically presented with multiple sinusoidal dilatations with blood-filled cystic spaces. INTERVENTIONS: After the liver biopsy or laparoscopy, the patients were discharged and followed up in the clinic. OUTCOMES: Both patients were followed up for at least 1 year with good recovery. LESSONS: PH should always be recognized in the differentiation of liver lesions, particularly indistinctive lesion(s) without any history of liver-related diseases.

Contrast enhanced ultrasound of a rare case of Peliosis hepatis.

Peliosis hepatis is a rare benign disorder of the liver, histologically characterized by blood-filled cystic cavities of various sizes and irregular shapes, communicating with the hepatic sinusoids. Only a few cases of peliosis hepatis have been described using contrast enhanced ultrasound showing admittedly various dynamic enhancement patterns. We present a case of peliosis hepatis with a typical target-sign enhancement depicted by means of contrast enhanced ultrasound.

Peliosis Hepatis Mimicking Malignant Hypervascular Tumors.

BACKGROUND: Peliosis hepatis is an uncommon vascular condition characterized by multiple, randomly distributed, blood-filled, and cyst-like cavities throughout the liver. The unique clinical nature consisting of an asymptomatic, benign, and incidental finding sometimes conceals itself even until autopsy. However, the exact pathomechanism remains as yet unproven. METHOD: We encountered the patient with multiple hypervascular masses in the whole liver and investigated the clinicopathological features underlying this condition. RESULTS: Detailed herein is a case of peliosis hepatis on the basis of our findings. Our patient illustrated a lethal instance which mimicked a malignancy although it is usually an indolent finding. CONCLUSION: Peliosis hepatis should always be borne in mind as a differential diagnosis of atypical hypervascular hepatic lesion, especially in patients with clinical conditions described in the text.

[Focal hepatic peliosis without evident aetiology].

Hepatic peliosis is a rare vascular disorder of the liver characterized by small cavities containing blood. It is most often asymptomatic but may give rise to portal hypertension and even fatal liver rupture. In this case a 47-year-old woman presented with abdominal pain and elevated alkaline phosphatase. An ultrasonography and additional MRI of the liver detected three lesions suspected of focal nodular hyperplasia. A core needle biopsy revealed that hepatic peliosis was the correct diagnosis. No causative factors were found and the patient was followed by consecutive MRIs.