Primary Hepatic Neuroendocrine Tumor Research Articles

Extended Left Hemihapatectomy with Right Hepatic Artery Reconstruction for Primary Hepatic Neuroendocrine Neoplasm: A Brief Report

Extended Left Hemihapatectomy with Right Hepatic Artery Reconstruction for Primary Hepatic Neuroendocrine Neoplasm: A Brief Report

Surgical treatment of primary hepatic neuroendocrine tumor diagnosed by Al18F-NOTA-Octreotide PET/CT: a case report

Surgical treatment of primary hepatic neuroendocrine tumor diagnosed by Al18F-NOTA-Octreotide PET/CT: a case report

Primary Neuroendocrine Tumor of the Liver: A Case Report and Review of the Literature

Neuroendocrine tumors (NETs) of the digestive tract are rare tumors, but their prevalence is increasing. The anatomopathological examination is essential for establishing the diagnosis and for their histo-prognostic evaluation. Primary hepatic neuroendocrine tumors (PHNETs) are extremely rare, with only 200 cases described in the literature up to 2022. Given the rarity of PHNETs, there are no established algorithms for the diagnosis and management of these lesions, which presents a real medical challenge. Treatment is mainly surgical. The prognosis for these NETs appears to be better. The 10-year survival rate is around 70%. We report the case of a patient admitted for etiological evaluation of chronic right hypochondriac pain.

Comparison of primary hepatic neuroendocrine tumors and non-hepatitis B non-hepatitis C hepatocellular carcinoma on contrast-enhanced ultrasound.

The purpose of this study was to compare the sonographic features of primary hepatic neuroendocrine tumors (PHNETs) to those of non-hepatitis B and non-hepatitis C hepatocellular carcinoma (NBNC-HCC) on contrast-enhanced ultrasound (CEUS). Fourteen patients with a mean age of 56.9 ± 12.2 (SD) years with histopathologically confirmed PHNET were included in the study. Twenty-eight patients with a mean age of 58.5 ± 10.4 years with histopathologically confirmed NBNC-HCC were randomly selected as the control group. The clinical data, conventional ultrasound and CEUS features were retrospectively analyzed between PHNET and NBNC-HCC. PHNET was more common in women (57.1%, 8/14 cases), and NBNC-HCC was more common in men (75.0%, 21/28) (P=0.040). No significant differences were observed in etiology, tumor marker, and liver function between the two group (P>0.05). Conventional ultrasound revealed that the tumor size of PHNET (10.1 ± 4.7cm) was larger than that of NBNC-HCC (5.9 ± 3.8cm) (P=0.006). NBNC-HCC was predominantly hypoechoic, while the echogenicity of PHNET varied (P=0.001). On CEUS, 57.1% (8/14) of PHNETs showed heterogeneous hyperenhancement, whereas 77.0% (21/28) of NBNC-HCC presented homogeneous hyperenhancement (P=0.015). Furthermore, 35.7% (5/14) of PHNETs showed early washout (onset of washout <60 s), which was significantly different from that of NBNC-HCC (3.7%, 1/28) (P=0.005). CEUS is helpful in discriminating between PHNET and NBNC-HCC. PHNETs mainly present as a single mass with a large size (>10cm) in the liver. The CEUS showed that most PHNETs exhibited heterogeneous enhancement in the arterial phase, washout in the portal venous and late phases and early washout being more likely than NBNC-HCC. However, more imaging features need to be evaluated in a larger sample.

A rare primary hepatic neuroendocrine tumour with laparoscopic resection: a case report

IntroductionPrimary hepatic neuroendocrine tumours (PHNETs) are a rare form of hepatic neoplasms, and it is difficult to differentiate them from common hepatic malignancies in routine imaging studies.Presentation of the caseWe describe the case of a 60-year-old Indian male patient with a tentative preoperative diagnosis of hepatocellular carcinoma (HCC). Nevertheless, the definitive post-operative diagnosis was made by Histopathological and immunohistochemical assessment, which revealed a grade II neuroendocrine tumour (NET) of moderate differentiation. Surgical resection was performed through a minimally invasive approach with a favourable postoperative course and a short hospital stay. One-month Post-operative Octreotide scan showed no extrahepatic primary origin of the tumour.DiscussionPHNET is a rare entity, and multi modalities investigations, including imaging, serology, endoscopy series, and histopathology findings, aside from long-term follow-up to rule out another primary origin, are essential for the final diagnosis of PHNET. Surgical resection stands as the mainstay of treatment of PHNETs.ConclusionThe absence of primary liver diseases should expand our possible differential diagnosis. Laparoscopic surgical resection of PHNETs carries a favourable outcome.

Role of Advanced Imaging in Primary Hepatic Neuroendocrine Tumour with Borderline Raised AFP and Negative Chromogranin Staining: A Case Report

Role of Advanced Imaging in Primary Hepatic Neuroendocrine Tumour with Borderline Raised AFP and Negative Chromogranin Staining: A Case Report

HER-2-positive primary neuroendocrine neoplasms of the breast with signet ring feature: A case report and review of literature.

Primary neuroendocrine neoplasm of the breast (BNEN) is an uncommon breast neoplasm, and in most cases, it presents as hormone receptors positive and HER-2 negative. Moreover, in neuroendocrine neoplasms (NENs), the signet ring feature is a rare morphological subtype, and only a few cases have been reported. Here, we report the case of a primary breast neuroendocrine neoplasm with an unusual signet ring cell appearance in this paper. The documentation of this case, combined with a review of the literature, may add to existing knowledge about the outcome and management of this rare tumor. In the present review, we describe a unique case of HER-2-positive primary BNEN with a signet ring feature that has not been reported in English. Additionally, we performed a literature search of the PubMed and Web of Science databases and calculated statistics for clinical data and follow-up. Our literature search, excluding non-English literature, identified 15 articles with data from 24 cases, including ours. The mean age was 51.25 years (range, 30-79 years), and there were 13 male patients (54%) and 11 female patients (46%). Of the 24 cases, some cases (11/24) were associated with lymph node metastases, a few cases (6/24) had distant metastasis, and the vast majority of cases (23/24) occurred in the digestive system. Primary hepatic signet ring cell neuroendocrine tumor showed slow progression and good prognosis. Lymph node involvement was identified in one of eight (12.5%) documented cases, and one of eight (12.5%) reported cases presented with distant metastatic disease. However, the prognosis of neuroendocrine tumors with signet ring cells in the pancreas and stomach was poor. Lymph node involvement was identified in 9 of 15 (60%) documented cases, and 5 of 15 (33.3%) reported cases presented with distant metastatic disease. NENs with a signet ring feature is uncommon, and this is the first case report of its occurrence in the breast. Current knowledge is limited to anecdotal experience based on case reports and small case series. We provide a literature review to summarize knowledge about this rare entity.

Developing a competing risk nomogram that predicts the survival of patients with a primary hepatic neuroendocrine tumor.

Primary hepatic neuroendocrine tumor (PHNET) is rare liver cancer and related prognostic factors are unclear. The aim of this study was to analyze the prognostic risk factors of patients with PHNETs and establish an assessment model for prognosis. The clinical information of 539 patients with PHNETs who met the criteria for inclusion was extracted from the Surveillance, Epidemiology, and End Results (SEER) database. These patients were randomly assigned to the training (269 cases) and validation sets (270 cases). Prognostic factors in patients with PHNETs were screened using the Cox proportional regression model and Fine-Gray competing risk model. Based on the training set analysis using the Fine-Gray competing risk model, a nomogram was constructed to predict cumulative probabilities for PHNET-specific death. The performance of the nomogram was measured by using receiver operating characteristic curves, the concordance index (C-index), calibration curves, and decision curve analysis (DCA). No differences in clinical baseline characteristics between the training and validation sets were observed, and the Fine-Gray analysis showed that surgery and more than one primary malignancy were associated with a low cumulative probability of PHNET-specific death. The training set nomograms were well-calibrated and had good discriminative ability, and good agreement between predicted and observed survival was observed. Patients with PHNETs with a high-risk score had a significantly increased risk of PHNET-specific death and non-PHNET death. Surgical treatment and the number of primary malignancies were found to be independent protective factors for PHNETs. The competing risk nomogram has high accuracy in predicting disease-specific survival (DSS) for patients with PHNETs, which may help clinicians to develop individualized treatment strategies.

Primary hepatic neuroendocrine tumor treated with liver transplantation.

An asymptomatic 45-year-old woman presented for evaluation of multiple liver lesions that were originally found accidentally 15 years ago. On clinical examination there were no abnormal findings; laboratory tests were also normal except for mildly elevated liver enzymes.

Primary hepatic neuroendocrine tumor with sarcoid like granulomas: A case report

Primary hepatic neuroendocrine tumor (PHNET) is a rare entity comprising 0.3% of all neuroendocrine tumors. Tumors coexisting with granulomas are occasionally noted with malignancies in the lymph nodes draining the tumor or within the primary tumor itself. Tumor-related sarcoid reactions are noted only in carcinomas and its association with a NET has been extremely rare.: We report a case of PHNET in a 63-year male who presented with various comorbidities who was incidentally detected with a liver space-occupying lesion (SOL). Contrast enhanced CT scan of the abdomen revealed a well-defined exophytic mass in the liver involving segment 6. Subsequently, 68Ga DOTANOC PET/CT scanning revealed somatostatin receptor-expressing well-defined lesion measuring 5.1 x 5.0 cm in segment 6 (SUV MAX 19). A diagnosis of either a primary or a metastatic neuroendocrine tumor was made and non-anatomical wedge resection of the tumor was done with a detailed exploration of the abdominal cavity to look for a primary focus that did not reveal any other lesion or mass. Histologically, the tumor was confirmed to be a Neuroendocrine Tumor along with the presence of tumor-related sarcoid like granulomas in the adjoining hepatic parenchyma.: Diagnosis of a PHNET requires a combination of a negative abdominal survey at the time of the operative procedure, pathologic evaluation of specimen, and a negative imaging workup for extrahepatic NET in both the pre and postoperative settings.

Molecular deciphering of primary liver neuroendocrine neoplasms confirms their distinct existence with foregut-like profile.

Isolated hepatic localizations of neuroendocrine tumors (NETs) are generally considered as metastatic NETs of unknown primary but could correspond to primary hepatic NETs (PHNETs), a poorly explored entity. We aimed to describe the clinicopathological and molecular features of PHNETs and compare them with other primary NETs. We assembled a retrospective cohort of patients managed for hepatic localization of NET without extra-hepatic primary tumor after exhaustive clinical, imaging, and immunohistochemical characterization. We performed whole-exome sequencing with mutational and copy number analysis. Transcriptomic profiles were compared with pancreatic (n = 31), small-bowel (n = 22), and lung (n = 15) NETs using principal component analysis, unsupervised clustering, and gene set enrichment analysis. Among 27 screened patients, 16 had PHNET (solitary tumor in 63%, median size 11 cm, G2 NETs in 81%) following clinical and pathological review. DNA analyses showed 'foregut-like' genomic profiles with frequent alterations in pathways of Fanconi DNA repair (75%), histone modifiers (58%), adherens junctions (58%), and cell cycle control (50%). The most frequently involved genes were KMT2A (58%), ATM (42%), CDH1, CDKN2C, FANCF, and MEN1 (33% each). Transcriptomic analyses showed that PHNETs clustered closer to foregut (pancreatic, lung) NETs than to midgut (small-bowel) NETs, while remaining a distinct entity with a specific profile. Assessment of potentially predictive biomarkers suggested efficacy of treatments usually active in foregut NETs. In conclusion, PHNETs display a foregut-like molecular profile distinct from other types of NETs, with recurrent molecular alterations. Upon exhaustive work-up to exclude an unrecognized primary tumor, PHNETs should not be considered metastatic NETs from an unknown primary. © 2022 The Pathological Society of Great Britain and Ireland.

Primary hepatic neuroendocrine tumor: A case report

Primary neuroendocrine tumors (NET) of the liver are extremely rare, with approximately 150 published cases [2]. For the diagnosis is necessary to exclude its origin and presence in other locations and histological confirmation. When resection is feasible surgery is usually indicated. we present the case of a 57 year old patient with a primary NET on the liver.

Diagnostic problem of Primary Hepatic Neuroendocrine Tumor in 18-year-old woman: A case report.

Introduction and importancePrimary Hepatic Neuroendocrine Tumor (PHNET) is a very rare case and it's quite challenging to diagnose and treat. There are approximately 300 cases reported. Among them, only two giant PHNET cases were found in children under 20 years old.Case presentationWe reported an unusual giant PHNET that was found in 18 years old female followed by intra-tumoral bleeding, which was initially misdiagnosed as Hemangioma. Abdominal Computed Tomography (CT) imaging showed a large liver mass with the peripheral enhancing nodular solid part in the delayed phase, which resembled Hemangioma.Clinical discussionPreoperative diagnosis of a Primary Hepatic Neuroendocrine Tumor is difficult as the liver often becomes the site of Neuroendocrine Tumor metastasis from other gastrointestinal organs, rarely occurred in people under 20 years of age, and the CT scan looks like Hemangiomas. The clinical symptoms are not typical, so other specific diagnostic tools for Neuroendocrine Tumor such as Chromogranin A level and 24-hour 5-hydroxyindoleacetic acid urine test are not performed. The indication of hepatic resection is made based on the preoperative diagnosis of other conditions such as large tumor size or tumor complications. Intraoperative diagnosis is also not typical for PHNET, so the definitive diagnosis of PHENT is made from post-resection histopathology.ConclusionThe preoperative diagnosis of this rare tumor is still a big challenge. The physicians should be alert when finding Giant Hemangioma of the Liver, and Neuroendocrine Tumor should be the differential diagnosis. Blood Chromogranin A, urine 5-hydroxyindoleacetic acid test, and Positron Emission Tomography (PET) scan should also be considered.

A Case of Primary Hepatic Neuroendocrine Tumor Slowly Growing During Long-term Follow-up

A Case of Primary Hepatic Neuroendocrine Tumor Slowly Growing During Long-term Follow-up

Re-Recognizing the Cellular Origin of the Primary Epithelial Tumors of the Liver.

The primary epithelial tumors of the liver (PETL) are composed of a series of heterogeneous tumors. Although the classification of PETLs has been updated several times by the World Health Organization, the cellular origins of some tumors in this family remain to be precisely depicted. In addition, certain tumors in different categories have similar histology, molecular phenotypes and biological characteristics, suggesting that they may have the same cellular origin. In this work, a narrative review method was adopted to review the relevant papers. By comparing the expression profiles of biomarkers of liver epithelium at different lineages and stages of differentiation, the cells-of-origin of some major members of the PETL family were reassessed. We propose that 1) hepatic adenomas, hepatocellular carcinomas (HCCs) and pure fetal hepatoblastomas (HBs) share the same spectrum in their cellular origin including the hepatocytic-committed progenitors (HCP) and their differentiated descendants. 2) Bile duct adenomas, peribiliary cysts and intrahepatic cholangiocellular carcinomas (ICCs) can share the same spectrum in their cellular origin including the cholangiocytic-committed progenitors (CCP) and their differentiated descendants. 3) The cells-of-origin of embryonal HBs include liver stem cells (LSCs), hepatoblasts, and transitional cells between them. Embryonal HB with small cell element, small cell undifferentiated HB and small cell neuroendocrine carcinoma of the liver can have the same or similar cells-of-origin from LSC. Embryonal HB lacking the small cell component of the LSC phenotype and presenting both hepatocytic and bile duct/ductule components may originate from actual hepatoblasts/hepatic progenitor cells (HPCs) as the combined HCC-ICC does. 4) Teratoid hepatoblastoma and mixed epithelial/mesenchymal HBs can be derived from the LSCs or even less committed extrahepatic pluripotent stem cell. 5) Many members of the PETLs family, including those derived from LSCs, hepatoblasts/HPCs, early HCPs and CCPs, have neuroendocrine potentiality. Except for those primary hepatic neuroendocrine tumor (PHNET) exhibit hepatocytic and/or cholangiocytic phenotypes, other PHNETs subtype may be derived from the descendants of LSC that differentiate towards the upper digestive tract, pancreas or other lineages.

Primary hepatic neuroendocrine tumour with multiple liver metastases: A case report with literature review.

Introduction and importanceTo describe an unusual case with a primary hepatic neuroendocrine tumour (PHNET) with multiple liver metastases.Case presentationWe reported a 65-year-old woman with PHNET with multiple liver metastases. She was highly suspected of having primary liver cancer with multiple intrahepatic metastases before liver biopsy, but was diagnosed with PHNET with multiple liver metastases after histopathology and immunohistochemistry (IHC) examinations. The patient successfully underwent three times of transcatheter arterial chemoembolization (TACE), and is currently living in a good state without related complications.Clinical discussionNeuroendocrine tumors (NETs), also known as carcinoids or argyrophilic tumors, are very rare malignant tumors. The liver is the main metastasis site of NETs, but primary hepatic neuroendocrine tumors (PHNETs) are extremely rare. Histopathology and immunohistochemistry (IHC) examinations are still the main methods used for diagnosing NETs. There are no treatment guidelines for PHNETs, and surgical resection is generally the preferred treatment. For PHNET patients who are not suitable for surgery, TACE has been proven to be an effective alternative treatment that can effectively reduce the tumour burden and relieve symptoms, but the current evidence is still limited.ConclusionThe clinical diagnosis of PHNET still faces great challenges, imaging examinations often lead to misdiagnosis, and its diagnosis mainly depends on histopathology and immunohistochemical examinations. For PHNET patients who are not suitable for surgery, TACE may be an effective alternative therapy.

Primary Hepatic Neuroendocrine Tumor and its Metastasis to Breast; Case Report of an Unusual Malignancy

Introduction: Neuroendocrine tumors (NETs) are rare tumors with varied clinical presentations. Entero-pancreatic and respiratory systems are usually involved but it can also affect unusual sites like the liver.&#x0D; Purpose: This paper presents a case of a 45 years female who developed progressive and disabling symptoms of mass effect and carcinoid but remained undiagnosed for many years due to extremely low suspicion of such a tumor in liver.&#x0D; Methods: Diagnosis was made after extensive radiological, histopathological, and biochemical investigations. By that time, disease had spread to her breast which is also not a typical feature of NETs.&#x0D; Findings: Diagnosis of rare tumors at an unusual site is challenging and requires high clinical suspicion and appropriate workup.

Two cases of primary hepatic neuroendocrine tumor with various imaging findings

Two cases of primary hepatic neuroendocrine tumor with various imaging findings

Mixed Neuroendocrine Carcinoma and Hepatocellular Carcinoma: A Case Report and Literature Review

Background: Neuroendocrine tumors are heterogeneous malignancies that originate from the neuroendocrine system. Previous studies show that this cancer type mainly localizes in the gastrointestinal tract and often metastasizes to the liver. Primary liver neuroendocrine tumors are very rare and primary hepatic neuroendocrine tumors (PHNET) with concurrent hepatocellular carcinoma (HCC) are extremely rare. To the best of our knowledge, only few PHNET cases have been identified, making their diagnosis difficult. Here, we report the biggest ever reported and “deceiving” lesion of a mixed neuroendocrine-non-neuroendocrine neoplasm in the liver, aiming to raise awareness and improve treatment of the disease.Case Presentation: Here, we report a preoperative misdiagnosed case that presented with hepatocellular carcinoma clinical features and no extrahepatic tumors. Postoperative pathology confirmed that it was a mixed neuroendocrine-non-neuroendocrine neoplasm. The patient was then referred for etoposide and cisplatin-based chemotherapy. No disease recurrence was observed at the 6-month follow-up.Conclusion: We report a very rare and easily misdiagnosed case and we speculate that there were “undifferentiated cells” undergoing neuroendocrine and hepatocellular carcinoma differentiation, during which some hepatocellular carcinoma cells express neuroendocrine features. We recommend proper surgery and postoperative platinum-based chemotherapy in the management of this disease.

Development of a nomogram model to predict survival outcomes in patients with primary hepatic neuroendocrine tumors based on SEER database

BackgroundPrimary hepatic neuroendocrine tumors (PH-NETs) are extremely rare and unknown. Because of its rarity, its prognosis features and influencing factors are not well established.MethodsData of 140 patients with PH-NETs diagnosed in the SEER database from 1975 to 2016 were collected. The demographics and clinic-pathological features were described. By using propensity-score matching (PSM) analysis, three associated cohorts were selected to describe the malignancy of PH-NETs and univariate analysis was conducted. Then, multivariate Cox analyses were performed and a predicting nomograph was constructed. C-index, receiver operating characteristic (ROC) curve and calibration curves were used to evaluate the predictive value of nomogram.ResultsThe overall survival outcomes of PH-NETs were superior to hepatocellular carcinoma (HCC) with a mean survival time 30.64 vs 25.11 months (p = 0.052), but inferior to gastrointestinal tract neuroendocrine tumors in situ (GI-NETs in situ) with a mean survival time 30.64 vs 41.62 months (p = 0.017). With reference to gastrointestinal neuroendocrine tumors with liver metastasis (GI-NETs-LM), GI-NETs-LM had better outcomes in short time (1-year survival rate: 64.75% vs 56.43%) but was worse in long time (5-year survival rate: 8. 63% vs 18.57%). Multivariate Cox analyses showed that tumor grade and surgery were two independent factors for prognosis of the patients (p < 0.00). Tumor grade and surgery were used to construct the predicting nomogram. The C-index was 0.79 (95%CI = 0.75–0.83). The area under curve (AUC) values in ROC were 0.868 in 1-year and 0.917 in 3-year survival and the calibration curves showed good consistency.ConclusionsThe overall prognosis PH-NETs is generally favorable, better than HCC and GI-NETs-LM in long term. Preoperative biopsy and complete pathological diagnosis were recommended. Radical surgical intervention including transplantation was the first choice in PH-NETs therapy.