Clinical characteristics and outcome of primary hepatic neuroendocrine tumors after comprehensive therapy

BACKGROUND Primary hepatic neuroendocrine tumors(PHNETs),a group of neuroendocrine neoplasms,are extremely rare.There are only few case reports about PHNETs in the literature.The lack of large samples and multicenter research results in poor diagnostic and therapeutic approaches.AIM To discuss the clinical characteristics,diagnosis,and treatment of PHNETs and risk factors related to survival.METHODS We retrospectively analyzed the clinical data,imaging features,immunohistochemistry data,and treatment efficacy of 40 patients who were pathologically diagnosed with PHNETs and admitted to The First Affiliated Hospital of Zhengzhou University from January 1,2014 to November 15,2019.Finally,survival analysis was performed to identify the risk factors for survival.RESULTS The main symptoms and signs included intermittent abdominal pain(19 patients,47.5%)and bloating(8 patients,20.0%).The positive rates of tested tumor markers were recorded as follows:Carbohydrate antigen 19-9(CA19-9)(6 patients,15.0%),CA72-4(3 patients,7.5%),carcinoembryonic antigen(7 patients,17.5%),and alpha-fetoprotein(6 patients,15.0%).Immunohistochemical staining results showed positivity for Syn in 38(97.4%)of 39 patients,for chromogranin A in 17(65.4%)of 26 patients,for CD56 in 35(94.6%)of 37 patients,for AE1/AE3 in 28(87.5%)of 32 patients,and for Ki-67 in all 40(100.0%)patients.The overall survival rate was significantly related to the tumor grade,AE1/AE3,and Ki-67.tumor number,tumor size,metastasis,and treatment)and overall survival.CONCLUSION Higher grade,negative AE1/AE3,and higher Ki-67 are associated with a worse survival rate.Kinds of treatment and other parameters have no significant influence on overall survival.

Primary hepatic neuroendocrine tumor with a suspicious pulmonary nodule:A case report and literature review

BACKGROUND Primary hepatic neuroendocrine tumors(PHNETs)are extremely rare tumors originating from neuroendocrine cells.Due to lack of neuroendocrine symptoms and specific radiographic characteristics,PHNETs are challenging to differentiate from other liver tumors.CASE SUMMARY This case involved a 67-year-old male who was admitted with a discovered hepatic mass and a suspicious lung lesion.Primary hepatic carcinoma was initially speculated based on the characteristic magnetic resonance imaging findings.The patient underwent a laparoscopic right partial hepatectomy,and subsequent immunohistochemical examination revealed a HNET.To exclude other potential origins,a positron emission tomography-computed tomography scan and gastrointestinal endoscopy were performed,leading to a final diagnosis of PHNETs.Then we conducted a literature review using the PubMed database,identifying 99 articles and 317 cases related to PHNETs.The characteristics,diagnostic methods,and treatment of PHNETs have been described.Finally,we elaborate on the presumed origins,pathological grades,clinical features,diagnosed methods,and treatments associated with PHNETs.CONCLUSION The diagnosis of PHNETs was primarily an exclusionary process.A definitive diagnosis of PHNETs relied mainly on immunohistochemical markers(chromogranin A,synaptophysin,and cluster of differentiation 56)and exclusion of primary foci in other organs.Radical surgery was the preferred treatment for early-stage tumors.

Rare and lacking typical clinical symptoms of liver tumors:Four case reports

BACKGROUND Rare liver tumors(RLTs)have an extremely low likelihood of forming,and some have been recorded only in isolated cases.The lack of normal clinical symptoms in RLTs makes preoperative diagnosis extremely challenging,which results in frequent misinterpretation.The present case report helps enhance our ability to recognize and treat uncommon liver tumor disorders.CASE SUMMARY We describe four distinct examples of rare liver tumor diseases.These cases were all true cases with no conventional clinical signs or imaging findings.In all patients,hepatic occupancy was discovered on physical examination,which raised the preoperative suspicion of hepatic cancer.All tumors were surgically removed,and postoperative histology and immunohistochemistry were performed to confirm the diagnosis.The first patient had primary hepatic fibrosarcoma.The second case involved a primary hepatic neuroendocrine tumors.These two patients had malignant liver tumors,and both had extremely satisfactory surgical outcomes.The third case involved focal hepatic steatosis,and the fourth case involved a single necrotic nodule in the liver.These two patients had benign liver tumors,but they had already undergone surgery and did not require any postoperative care.CONCLUSION The number of patients with RLTs is small,and the clinical and imaging results are vague.Preoperative diagnosis is challenging,and patients are sometimes mistakenly diagnosed with liver cancer,which leads to unnecessary surgical therapy in certain individuals.