Two case reports and literature review for hepatic epithelioid angiomyolipoma: Pitfall of misdiagnosis

BACKGROUND Hepatic epithelioid angiomyolipoma(HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.CASE SUMMARY We presented two cases of HEAML in Changzheng Hospital, Naval Medical University, and then collected and analyzed all reports about HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. A total of 409 cases of HEAML in 97 reports were collected, with a ratio of men to women of 1:4.84 and an age range from 12 years to 80 years(median 44 years). Among the patients with clinical symptoms mentioned, 61.93%(205/331) were asymptomatic, 34.74%(115/331) showed upper or right upper quadrant abdomen discomfort, while a few of them showed abdominal mass, gastrointestinal symptoms, low fever, or weight loss. The misdiagnosis rate of HEAML was as high as 40.34%(165/409) due to its nonspecific imaging findings. Most of the tumors were solitary and round in morphology, with clear boundaries. Ultrasound scan indicated low echo with internal nonuniformity and rich blood supply in most cases. Computer tomography/magnetic resonance imaging enhanced scan showed varied characteristics. The ratio of fast wash-in and fast wash-out, fast wash-in and slow wash-out, and delayed enhancement was roughly 4:5:1. A definite diagnosis of HEAML depended on the pathological findings of the epithelioid cells in lesions and the expression of human melanoma black 45, smooth muscle actin,melanoma antigen, and actin by immunohistochemical staining. HEAML had a relatively low malignant rate of 3.91%. However, surgical resection was the main treatment for HEAML, due to the difficulty diagnosing before operation.CONCLUSION HEAML is a rare and easily misdiagnosed disease, and it should be diagnosed carefully, taking into account clinical course, imaging, pathological,and immunohistochemical findings.

Immunotherapy in liver transplantation for hepatocellular carcinoma:Pros and cons

Liver transplantation(LT)has emerged as a curative strategy for hepatocellular carcinoma(HCC),but contributes to a higher predisposition to HCC recurrence in the immunosuppression context,especially for tumors beyond the Milan criteria.Although immunotherapy has dramatically improved survival for immunocompetent patients and has become the standard of care for a variety of tumors,including HCC,it is mainly used outside the scope of organ transplantation owing to potentially fatal allograft rejection.Nevertheless,accumulative evidence has expanded the therapeutic paradigms of immunotherapy for HCC,from downstaging or bridging management in the pretransplant setting to the salvage or adjuvant strategy in the posttransplant setting.Generally,immunotherapy mainly includes immune checkpoint inhibitors(ICIs),adoptive cell transfer(ACT)and vaccine therapy.ICIs,followed by ACT,have been most investigated in LT,with some promising results.Because of the complex tumor microenvironment and immunoreactivity when immunosuppressants are combined with immunotherapy,it is difficult to reach formulations for immunosuppressant adjustment and the optimal selection of immunotherapy as well as patients.In addition,the absence of effective biomarkers for identifying rejection and tumor response is still an unresolved barrier to successful clinical immunotherapy applications for LT.In this review,we comprehensively summarize the available evidence of immunotherapy used in LT that is specific to HCC.Moreover,we discuss clinically concerning issues regarding the concurrent goals of graft protection and antitumor response.

Two-in-one:A pooled analysis of primary hepatic neuroendocrine carcinoma combine d/collide d with hepatocellular carcinoma

To the Editor:Primary hepatic neuroendocrine carcinoma(PHNEC)combined/collided with hepatocellular carcinoma(HCC)is a rare and intriguing disease with characteristics of two histologically different cancers.Usually,a combined type refers to the cases in which hepatic neuroendocrine carcinoma(HNEC)and HCC components intermingle with each other and cannot be clearly separated in the transitional areas,while a collision type refers to those in which the two distinct cancers occurred simultaneously and independently.Sometimes it is difficult to readily distinguish these two types.We searched PubMed,Medline,China Science Periodical Database,and VIP Database with the publication from January 1980 to January 2019,using the keywords(“hepatic neuroendocrine carcinoma”or“HNEC”)and(“hepatocellular carcinoma”or“HCC”).After manually excluding records on secondary HNEC and other types of liver cancer,a total of 28 cases in 24 reports[1–24]were identified and summarized in Table 1.

Primary hepatic squamous cell carcinoma with abdominal incision metastasis after hepatectomy

To the Editor:Primary hepatic squamous cell carcinoma(PHSCC)is a rare and easily misdiagnosed disease.We reported a PHSCC case with abdominal incision metastasis after hepatectomy,and reviewed the clinical course,imaging,pathology and immunohistochemical findings.Our case may provide a better understanding of PHSCC.

Immunometabolic inflammation and hepatocellular carcinoma

To the Editor: Inflammation-cancer transformation and metabolomics are hot topics in hepatocellular carcinoma (HCC). Cancer-related inflammation and anti-cancer immunity co-exist in cancer progression and the microenvironmental conditions dictate the direction [1].

Criteria-specific long-term survival prediction model for hepatocellular carcinoma patients after liver transplantation

AIM: To establish a model to predict long-term survival of hepatocellular carcinoma(HCC) patients after liver transplantation(MHCAT).METHODS: Two hundred and twenty-three patients with HCC were followed for at least six years to identify independent risk factors for long-term survival after liver transplantation(LT). The criteria for HCC liver transplantation included the Milan, University of California San Francisco, Hangzhou and Shanghai Fudan criteria. The Cox regression model was used to build MHCAT specifying these criteria. A survival analysis was carried out for patients with high or low risk.RESULTS: The one-, three- and five-year cumulativesurvival of HCC patients after LT was 78.9%, 53.2% and 46.4%, respectively. Of the HCC patients, the proportion meeting the Hangzhou and Fudan criteria was significantly higher than the proportion meeting the Milan criteria(64.6% vs 39.5%, 52.0% vs 39.5%, P < 0.05). Moreover, the proportion meeting the Hangzhou criteria was also significantly higher than the proportion meeting other criteria(P < 0.01). Pre-operative alfa-fetoprotein level, intraoperative blood loss and retransplantation were common significant predictors of long-term survival in HCC patients with reference to the Milan, University of California San Francisco and Fudan criteria, whereas in MHCAT based on the Hangzhou criteria, total bilirubin, intraoperative blood loss and retransplantation were independent predictors. The c-statistic for MHCAT was 0.773-0.824, with no statistical difference among these four criteria. According to the MHCAT scoring system, patients with low risk showed a higher five-year survival than those with high risk(P < 0.001).CONCLUSION: MHCAT can effectively predict longterm survival for HCC patients, but needs to be verified by multi-center retrospective or randomized controlled trials.