Idiopathic hypereosinophilic syndrome with hepatic sinusoidal obstruction syndrome:A case report and literature review

BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.

Simultaneous transcatheter arterial chemoembolization and portal vein embolization for patients with large hepatocellular carcinoma before major hepatectomy

BACKGROUND Sequential transarterial chemoembolization(TACE)and portal vein embolization(PVE)are associated with long time interval that can allow tumor growth and nullify treatments'benefits.AIM To evaluate the effect of simultaneous TACE and PVE for patients with large hepatocellular carcinoma(HCC)prior to elective major hepatectomy.METHODS Fifty-one patients with large HCC who underwent PVE combined with or without TACE prior to hepatectomy were included in this study,with 13 patients in the simultaneous TACE+PVE group,17 patients in the sequential TACE+PVE group,and 21 patients in the PVE-only group.The outcomes of the procedures were compared and analyzed.RESULTS All patients underwent embolization.The mean interval from embolization to surgery,the kinetic growth rate of the future liver remnant(FLR),the degree of tumor size reduction,and complete tumor necrosis were significantly better in the simultaneous TACE+PVE group than in the other groups.Although the patients in the simultaneous TACE+PVE group had a higher transaminase levels after PVE and TACE,they recovered to comparable levels with the other two groups before surgery.The intraoperative course and the complication and mortality rates were similar among the three groups.The overall survival and disease-free survival were higher in the simultaneous TACE+PVE group than in the other two groups.CONCLUSION Simultaneous TACE and PVE is a safe and effective approach to increase FLR volume for patients with large HCC before major hepatectomy.

Rare cause of upper gastrointestinal bleeding owing to hepatic cancer invasion:A case report

Upper gastrointestinal bleeding refers to bleeding that arises from the gastrointestinal tract proximal to the ligament of Treitz.The primary reason for gastrointestinal bleeding associated with hepatocellular carcinoma is rupture of a varicose vein owing to pericardial hypotension.We report a rare case of gastrointestinal bleeding with hepatocellular carcinoma in a patient who presented with recurrent gastrointestinal bleeding.The initial diagnosis was gastric cancer with metastasis to the multiple lymph nodes of the lesser curvature.The patient underwent exploratory laparotomy,which identified two lesions in the gastric wall.Total gastrectomy and hepatic local excision was then performed.Pathological results indicated that the hepatocellular carcinoma had invaded the stomach directly,which was confirmed immunohistochemically.The patient is alive with a disease-free survival of 1 year since the surgery.Hepatocellular carcinoma with gastric invasion should be considered as a rare cause of upper gastrointestinal bleeding in hepatocellular carcinoma patients,especially with lesions located in the left lateral hepatic lobe.Surgery is the best solution.

Laparoscope resection of retroperitoneal ectopic insulinoma: A rare case

Ectopic insulinoma is a very rare and dormant tumor.Here we report the case of a 79-year-old female who presented with repeated episodes of hypoglycemia and was diagnosed with insulinoma based on laboratory and imaging examinations.Computed tomography and positron emission tomography revealed a tumor in the retroperitoneum under and left of the hepatoduodenal ligament, which was resected successfully using a laparoscopic approach.Pathologic results revealed an ectopic insulinoma, which was confirmed immunohistochemically.Ectopic insulinomas are accompanied by hypoglycemia that can be misdiagnosed as drug- or disease-induced.These tumors are difficult to diagnose and locate, particularly in atypical cases or for very small tumors.Synthetic or targeted examinations, including low blood glucose, elevated insulin, proinsulin, and C-peptide levels, 48-h fasting tests, and relevant imaging methods should be considered for suspected cases of insulinoma.Surgery is the treatment of choice for patients with insulinoma, and laparoscopic resection is a feasible and effective method for select ectopic insulinoma cases.

Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.RESULTS: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.CONCLUSION: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.

Culture of patient-derived multicellular clusters in suspended hydrogel capsules for pre-clinical personalized drug screening

A personalized medication regimen provides precise treatment for an individual and can be guided by pre-clinical drug screening.The economical and high-efficiency simulation of the liver tumor microenvironment(TME)in a drug-screening model has high value yet challenging to accomplish.Herein,we propose a simulation of the liver TME with suspended alginate-gelatin hydrogel capsules encapsulating patient-derived liver tumor multicellular clusters,and the culture of patient-derived tumor organoids(PDTOs)for personalized pre-clinical drug screening.The hydrogel capsule offers a 3D matrix environment with mechanical and biological properties similar to those of the liver in vivo.As a result,18 of the 28 patient-derived multicellular clusters were successfully cultured as PDTOs.These PDTOs,along with hepatocyte growth factor(HGF)of non-cellular components,preserve stromal cells,including cancer-associated fibroblasts(CAFs)and vascular endothelial cells(VECs).They also maintain stable expression of molecular markers and tumor heterogeneity similar to those of the original liver tumors.Drugs,including cabazitaxel,oxaliplatin,and sorafenib,were tested in PDTOs.The sensitivity of PDTOs to these drugs differs between individuals.The sensitivity of one PDTO to oxaliplatin was validated using magnetic resonance imaging(MRI)and biochemical tests after oxaliplatin clinical treatment of the corresponding patient.Therefore,this approach is promising for economical,accurate,and high-throughput drug screening for personalized treatment.