Primary Hepatic Neuroendocrine Carcinoma Research Articles

Long‐term survival of a cat diagnosed with primary hepatic neuroendocrine carcinoma treated with surgical resection and several adjuvant chemotherapies

AbstractPrimary hepatic neuroendocrine carcinoma, previously referred to as carcinoid, is a malignant tumour arising from neuroendocrine cells and is a rare form of hepatic neoplasia in cats. This case report describes primary hepatic neuroendocrine carcinoma in a cat, with weight loss and vomiting as its primary clinical signs. Definitive diagnosis was made following surgical excision and histopathology of the affected liver lobes in addition to immunohistochemistry staining. Following surgery, the cat received different chemotherapy agents due to observation of progressive disease at restaging visits. The cat is currently receiving metronomic chemotherapy of cyclophosphamide and has no evidence of metastatic disease present 1009 days after initial presentation. Primary hepatic neuroendocrine carcinoma can occur in cats and should be a differential diagnosis for a hepatic mass in this species. Primary hepatic neuroendocrine carcinomas are rare in cats, and there is no standard of care for treatment in companion animals.

Combined Hepatocellular Neuroendocrine Carcinoma: A Rare Tumor

ABSTRACT Neuroendocrine tumors originate from neuroendocrine cells primarily located in the gastrointestinal tract. These tumors often metastasize to the liver. Primary hepatic neuroendocrine carcinomas are uncommon, and combined hepatocellular neuroendocrine carcinomas are exceedingly rare. There is a lack of data on the management of these rare tumors. Most cases have very poor prognosis secondary to aggressive behavior of the neuroendocrine tumor component. It is important for clinicians to be aware of this rare carcinoma to allow for early diagnosis and optimize potential treatment options.

Primary hepatic neuroendocrine carcinoma with colon adenoma: A case report with literature review.

Introduction and importancePrimary hepatic neuroendocrine tumors (PHNETs) are extremely rare, and the clinical symptoms, test results, and imaging characteristics are nonspecific in most patients; thus, it is difficult to differentiate from other liver masses before surgery. Histopathology and immunohistochemistry are the main basis for the diagnosis. PHNETs and colon tumors co-occur in a patient and are non-homologous, as reported in the English-language literature for the first time.Case presentationWe present a case of a 60-year-old woman with right hepatic lobe mass accidentally discovered on abdominal ultrasonography during a routine examination. Preoperative liver contrast-enhanced computed tomography suggested hepatocellular carcinoma; then, surgery were performed. Pathological results revealed a Grade 2 neuroendocrine tumor of the liver. In search of the primary tumor, upper and lower endoscopy of the GI tract was performed and revealed a mass in the ascending colon. Ascending colon cancer was considered; then, laparoscopic right hemicolectomy was performed. Pathological results suggested tubular villous adenoma of the ascending colon. The final diagnosis was not colon cancer with liver metastases but was PHNETs with colon adenoma.Clinical discussionPHNETs are rare cancers that are difficult to diagnose, requiring not only differentiation from other liver masses but also exclusion of metastases from extrahepatic sources. The pathological results play an important in making an accurate diagnosis.ConclusionPathology, postoperative follow-up, and comprehensive imaging examinations are powerful tools in the diagnosis of PHNETs. Currently, surgery is the best treatment to achieve a potential cure and prolong the patient's survival.

Primary hepatic neuroendocrine carcinoma: A postoperative diagnosis

Primary hepatic neuroendocrine carcinoma: A postoperative diagnosis

Efficacy of Chemotherapy versus Transcatheter Arterial Chemoembolization in Patients with Advanced Primary Hepatic Neuroendocrine Carcinoma and an Analysis of the Prognostic Factors: A Retrospective Study.

BackgroundPrimary hepatic neuroendocrine carcinoma (PHNEC) is a rare liver tumor, and there is no clear therapeutic recommendation for patients with advanced PHNEC. This study aims to compare the efficacy of platinum-based chemotherapy (etoposide combined with cisplatin/carboplatin, EP/EC) and transcatheter arterial chemoembolization (TACE) in patients with advanced PHNEC, and to evaluate the relevant prognostic factors.Patients and MethodsThe clinical data of 41 patients with advanced PHNEC from June 2014 to October 2019 were retrospectively reviewed.ResultsAt a median follow-up time of 13.9 months, the median overall survival (OS) was 14.8 months in the EP/EC group and 12.2 months in the TACE group (P = 0.040). The median progression-free survival (PFS) was 4.4 months and 2.7 months in the EP/EC group and the TACE group, respectively (P = 0.005). No significant differences in the overall response rate and disease control rate were observed between the EP/EC group and the TACE group (26.1% vs 11.1%, P = 0.429; 73.9% vs 44.4%, P = 0.055, respectively). A univariate analysis indicated that the Eastern Cooperative Oncology Group performance status (ECOG PS), Ki-67, tumor number, and treatment options were prognostic factors for OS. A multivariate analysis further showed that ECOG PS (P < 0.001), Ki-67 (P = 0.003), and treatment options (P = 0.022) were independent prognostic factors for OS.ConclusionKi-67, ECOG PS, and treatment options were the independent prognostic factors for OS in patients with advanced PHNEC. EP/EC may be a better choice for patients with advanced PHNEC.

Primary hepatic neuroendocrine carcinoma diagnosed by needle biopsy: a case report

BackgroundPrimary hepatic neuroendocrine carcinomas (NECs) are extremely rare. The rate of recurrence after resection is extremely high, and the prognosis is poor. It is debatable whether chemotherapy or surgical resection is the optimal initial treatment for primary hepatic NECs. Therefore, selecting an appropriate therapeutic approach for patients with primary hepatic NECs remains clinically challenging. We present a case of primary hepatic NEC in a patient who developed recurrence after undergoing surgical resection.Case presentationA 78-year-old man with bone metastases of prostate cancer was referred to our department because of a solitary 66-mm tumor in the left lateral segment of the liver, which was detected on annual follow-up by computed tomography after prostate resection. A biopsy and preoperative diagnostic workup identified the lesion as a primary hepatic neuroendocrine carcinoma; therefore, left lateral segmentectomy was performed. Immunohistochemically, the tumor was positive for chromogranin A, synaptophysin, and CD 56, and the Ki-67 index was 40%. This neuroendocrine carcinoma was classified as a large cell type. Adjuvant chemotherapy with carboplatin + etoposide was initially administered a month after surgery. However, lymph node recurrence occurred 4 months after surgery, and the patient died of systemic metastases 15 months after surgical resection.ConclusionsDue to the lack of availability of abundant quantities of relevant, high-quality data, there is no standard therapy for primary hepatic NECs. Selecting the most appropriate treatment for patients depending on several factors, such as the stage and differentiation of a tumor and a patient’s performance status and clinical course, is consequently preferred. More cases need to be studied to establish the best treatment strategy for primary hepatic NEC.

Mucinous Cystic Neoplasm of the Liver With Associated Neuroendocrine Carcinoma: A Case Report

Abstract Mucinous cystic neoplasms (MCNs) of the liver are relatively rare tumors that constitute less than 5% of liver cysts and are subcategorized into low/intermediate- and high-grade lesions based on the degree of epithelial dysplasia. An associated invasive carcinoma component occurs in 6% of cases, usually as adenocarcinoma with tubular growth. The liver is a frequent metastatic site for neuroendocrine carcinomas (NECs), whereas primary hepatic NECs are extremely rare. We present the case of a 63-year-old woman with a history of choledochal cyst, who now presented with abdominal pain. Computed tomography showed a multilocular cystic lesion in the liver. The cyst was surgically enucleated, and the histological examination revealed an MCN with low-grade dysplasia, with an associated invasive large cell NEC. To our knowledge, this is the first report in the literature of a biliary MCN with associated NEC.

Diagnosis of Non-Hepatocellular Carcinoma Malignancies in Patients With Risks for Hepatocellular Carcinoma: CEUS LI-RADS Versus CT/MRI LI-RADS.

ObjectiveData regarding direct comparison of contrast-enhanced ultrasound (CEUS) Liver Imaging Reporting and Data System (LI-RADS) and Computed Tomography/Magnetic Resonance Imaging (CT/MR) LI-RADS in diagnosis of non-hepatocelluar carcinoma (non-HCC) malignancies remain limited. Our study aimed to compare the diagnostic performance of the CEUS LI-RADS version 2017 and CT/MRI LI-RADS v2018 for diagnosing non-HCC malignancies in patients with risks for HCC.Materials and MethodsIn this retrospective study, 94 liver nodules pathologically-confirmed as non-HCC malignancies in 92 patients at risks for HCC from January 2009 to December 2018 were enrolled. The imaging features and the LI-RADS categories on corresponding CEUS and CT/MRI within 1 month were retrospectively analyzed according to the ACR CEUS LI-RADS v2017 and ACR CT/MRI LI-RADS v2018 by two radiologists in consensus for each algorithm. The sensitivity of LR-M category, inter-reader agreement and inter-modality agreement was compared between these two standardized algorithms.ResultsNinety-four nodules in 92 patients (mean age, 54 years ± 10 [standard deviation] with 65 men [54 years ± 11] and 27 women [54 years ± 8]), including 56 intrahepatic cholangiocarcinomas, 34 combined hepatocellular cholangiocarcinomas, two adenosquamous carcinomas of the liver, one primary hepatic neuroendocrine carcinoma and one hepatic undifferentiated sarcoma were included. On CEUS, numbers of lesions classified as LR-3, LR-4, LR-5 and LR-M were 0, 1, 10 and 83, and on CT/MRI, the corresponding numbers were 3, 0, 14 and 77. There was no significant difference in the sensitivity of LR-M between these two standardized algorithms (88.3% of CEUS vs 81.9% of CT/MRI, p = 0.210). Seventy-seven lesions (81.9%) were classified as the same LI-RADS categories by both standardized algorithms (five for LR-5 and 72 for LR-M, kappa value = 0.307). In the subgroup analysis for ICC and CHC, no significant differences were found in the sensitivity of LR-M category between these two standardized algorithms (for ICC, 94.6% of CEUS vs 89.3% of CT/MRI, p = 0.375; for CHC, 76.5% of CEUS vs 70.6% of CT/MRI, p = 0. 649).ConclusionCEUS LI-RADS v2017 and CT/MRI LI-RADS v2018 showed similar value for diagnosing non-HCC primary hepatic malignancies in patients with risks.

Treating leptomeningeal metastases from primary hepatic neuroendocrine carcinoma with combined radiotherapy and immunotherapy: a case report

Treating leptomeningeal metastases from primary hepatic neuroendocrine carcinoma with combined radiotherapy and immunotherapy: a case report

Combined primary hepatic neuroendocrine carcinoma and hepatocellular carcinoma: case report and literature review

BackgroundHepatocellular carcinoma (HCC) can grow in a mosaic pattern, often combined with various non-hepatocellular cells. However, HCC combined with a neuroendocrine carcinoma (NEC) component is rarely reported, and its clinical features, origin, diagnosis, and behavior have not been established. In the literature, mixed HCC–NEC tumors are categorized as either collision type or combined type, depending on their microscopic features. Here, we report a patient with a combined-type HCC–NEC tumor.Case presentationAn asymptomatic 84-year-old woman was found to have a solid mass in the right lobe of the liver. Laboratory and radiologic examinations showed typical findings of HCC, including arterial-phase enhancement, and portal- and delay-phase washout. She was treated by partial laparoscopic hepatectomy of segment 5. Pathological examination showed that the tumor was predominantly HCC, partly admixed with an NEC component. A transitional zone between the HCC and NEC tissues was also observed. The tumor was finally diagnosed as a combined-type primary mixed NEC–HCC tumor. After the preoperative diagnosis, the patient underwent somatostatin receptor scintigraphy to detect the primary NEC lesion, but no accumulation was found in any other part of her body. She has been free of recurrence for 9 months since the surgery.ConclusionMixed HCC–NEC tumors are extremely rare, and correct diagnosis requires multidisciplinary collaboration. The accumulation of further cases is needed to help understand the exact pathology, diagnosis, and treatment of this disease.

Elevated procalcitonin levels in primary hepatic neuroendocrine carcinoma

Rationale:Procalcitonin (PCT) has been identified as a tumor biomarker in medullary thyroid carcinoma. Other neuroendocrine carcinomas with elevated PCT levels are relatively rare, and are mainly reported in the lung, digestive tract, and pancreas. No studies in the literature have reported a case of primary hepatic carcinoma complicated with unexpectedly elevated PCT levels.Patient concerns:A 78-year-old man with persistent fatigue and mild fever was complicated with an extremely high PCT level. Radiological examination revealed a single hypodense lesion in the left lobe of the liver with a “rapid enhancement and rapid washout” pattern. Pathological analysis showed a poorly differentiated neuroendocrine carcinoma (grade 3) with multiple genetic mutations.Diagnosis:Primary hepatic neuroendocrine carcinoma.Interventions:The patient received antibiotic therapy and subsequent transcatheter hepatic arterial chemoembolization; a PCT assessment and computed tomography were performed during the follow-up.Outcomes:The PCT level did not decline after antibiotic therapy but greatly declined in response to effective transcatheter hepatic arterial chemoembolization. The patient survived and is still being followed up.Lessons:An extremely elevated PCT level may raise a suspicion of a neuroendocrine carcinoma and plays an indicative role as a biomarker during therapy.

A case of primary hepatic high-grade neuroendocrine carcinoma

A case of primary hepatic high-grade neuroendocrine carcinoma

A Case of Primary Hepatic Neuroendocrine Carcinoma that Underwent Hepatectomy with Portal Vein Resection and Reconstruction

A Case of Primary Hepatic Neuroendocrine Carcinoma that Underwent Hepatectomy with Portal Vein Resection and Reconstruction

Primary hepatic neuroendocrine carcinoma: report of two cases and literature review

BackgroundPrimary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare. The diagnosis of PHNEC remains challenging—partly due to its rarity, and partly due to its lack of unique clinical features. Available treatment options for PHNEC include surgical resection of the liver tumor(s), radiotherapy, liver transplant, transcatheter arterial chemoembolization (TACE), and administration of somatostatin analogues.Case presentationWe report two male PHNEC cases and discuss the diagnosis and treatment options. Both cases presented with abdominal pain; case two also presented with symptoms of jaundice. The initial diagnosis for both cases was poorly differentiated grade 3 small-cell neuroendocrine carcinoma, based on imaging characteristics and the pathology of liver biopsies. Final diagnoses of PHNEC were arrived at by ruling out non-hepatic origins. Case one presented with a large tumor in the right liver lobe, and the patient was treated with TACE. Case two presented with tumors in both liver lobes, invasions into the left branch of hepatic portal vein, and metastasis in the hepatic hilar lymph node. This patient was ineligible for TACE and was allergic to the somatostatin analogue octreotide. This limited treatment options to supportive therapies such as albumin supplementation for liver protection. Patient one and two died at 61 and 109 days, respectively, following initial hospital admission.ConclusionsWe diagnosed both cases with poorly differentiated grade 3 small-cell PHNEC through imaging characteristics, immunohistochemical staining of liver biopsies, and examinations to eliminate non-hepatic origins. Neither TACE nor liver protection appeared to significantly extend survival time of the two patients, suggesting these treatments may be inadequate to improve survival of patients with poorly differentiated grade 3 small-cell PHNEC. The prognosis of poorly differentiated grade 3 small-cell PHNEC is poor due to limited and ineffective treatment options.

Combination therapies for primary hepatic neuroendocrine carcinoma: a case report

BackgroundPrimary hepatic neuroendocrine carcinomas are extremely rare. Because of the rarity of PHNEC, its clinical features and treatment outcomes are not well understood. A proper diagnosis and the correct therapeutic approach therefore remain clinically challenging.Case presentationA 67-year-old man was admitted to our department because of a liver tumor. Computed tomography revealed a single liver tumor 50 mm in diameter and located in the S3 region. Biopsy and imaging findings resulted in a diagnosis of primary hepatic neuroendocrine carcinoma. Left lateral segmentectomy was performed. Immunohistochemically, the tumor cells were positive for synaptophysin, chromogranin A, and CD56. Ki-67 was positive in > 90% of the tumor cells. The final diagnosis was primary hepatic neuroendocrine carcinoma. The patient suffered two episodes of lymph node recurrence. Nonetheless, the tumor was excised to prolong survival. Thus, after lymphadenectomy, he received adjuvant chemotherapy for 6 months. Two years after surgery, the patient remains alive and in good general condition.ConclusionsIn most cases, primary hepatic neuroendocrine carcinoma, while extremely rare, has a poor prognosis. At present, surgical resection is a priority for curative treatment, but in patients with recurrence, combined therapies are recommended.

Primary Hepatic Neuroendocrine Carcinoma with Multiple Liver Metastases Presenting as Jaundice Due to CBD Obstruction

Background: Whereas more than 80% of NETs found in the liver are metastatic, primary hepatic neuroendocrine tumors (PHNETs) are very rare. We experienced a case of primary hepatic neuroendocrine carcinoma (PHNEC) with multiple liver metastases presenting as jaundice due to the mid CBD obstruction. To the best of our knowledge, few studies have reported similar cases like ours in the English literature. Case presentation: A 81-year-old man was admitted with jaundice of two week's duration. His initial laboratory findings were total bilirubin 19.75 mg/dl, direct bilirubin 15.72 mg/dl, aspartate aminotransferase 65 IU/L, alanine aminotransferase 71 IU/L, alkaline phosphatase 525 U/L, gamma-glutamic transpeptidase 313 U/L and CA19-9 529 U/ml. Abdominal CT revealed marked dilatation of intrahepatic bile duct (IHD) and proximal CBD due to CBD invasion by conglomerated lymphadenopathies in the hepatoduodenal ligament and showed subtly enhancing lesion with central hypodense portion in the liver segment 8/5. MRI with MRCP showed multiple variable sized T2 high signal intensity masses (the largest 3.6 cm) in the both hemiliver, which showed diffusion restriction and delayed enhancement, and diffuse IHD and proximal CBD dilatation with conglomerated lymph nodes at portocaval space and hepatoduodenal ligament. Percutaneous transhepatic biliary drainage was performed, but biopsy could not be done because of concomitant hemobilia. So, we performed sono-guided liver biopsy at the intrahepatic masses. Histopathology revealed poorly differentiated carcinoma. The immunohistochemical staining for CD56, synaptophysin and thyroid transcription factor-1 (TTF-1) revealed positive reaction. So, the diagnosis of neuroendocrine carcinoma, large cell type was confirmed. Chest CT for excluding metastasis from extrahepatic primary site or distant metastatic involvement showed no mass-like lesion in the both lung fields. He refused any methods of treatment such as chemotherapy or transhepatic arterial chemoembolization, and wanted only conservative treatment. Therefore, he was discharged after insertion of self-expandable metal stent at the mid CBD obstruction site and he was died 5 months later. Conclusion: Our case suggests that PHNEC can be presented as multiple intrahepatic masses with CBD obstruction even though PHNETs are very rare. Therefore, it is important to confirm the pathology even in such cases.Figure 1Figure 2

Primary hepatic neuroendocrine carcinoma invading the fundus of gallbladder: a report of one case

Primary hepatic neuroendocrine carcinoma invading the fundus of gallbladder: a report of one case

One case of primary hepatic neuroendocrine carcinoma with liver cirrhosis

One case of primary hepatic neuroendocrine carcinoma with liver cirrhosis

[Diagnosis and treatment of primary hepatic neuroendocrine carcinoma].

To investigate the clinical features, diagnostic and therapeutic methods of primary hepatic neuroendocrine carcinoma. The clinicopathological data of fourteen patients with primary hepatic neuroendocrine carcinoma confirmed by pathology were analyzed retrospectively and related literatures were reviewed. The fourteen patients, including eight males and six females, had an age range of 23-58 years (mean 45.9 years). Four tumors were located in the right liver lobe, four in the left liver lobe and six in both. The clinical manifestations were nonspecific and variable. The most common clinical manifestation was abdominal distention or right upper quadrant pain. Radiological findings were not specific and could not distinguish primary hepatic neuroendocrine tumor from hepatocellular carcinoma. Diagnosis of primary hepatic neuroendocrine tumor was confirmed by pathology using immunohistochemical staining and by the absence of extrahepatic primary lesions. Extrahepatic primary neuroendocrine carcinoma was ruled out by ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT), preoperative gastrointestinal endoscopy and long-term postoperative follow up. Three patients received surgical treatment, two cases received surgical resection and radiofrequency ablation (RFA), six patients received transarterial chemoembolization, one case received orthotopic liver transplantation, one case only received exploratory laparotomy, and one case received chemotherapy. All 14 patients were followed up and seven of them are still alive, the others died of liver failure or recurrence. Primary hepatic neuroendocrine carcinomas are extremely rare. Its diagnosis should be confirmed by pathology. Preoperative fine needle biopsy is strongly recommended. Prognosis is relatively favorable. Surgical resection is treatment of first choice, and TACE, RFA, and chemotherapy can be used for unresectable patients.

Metastasising hepatic neuroendocrine carcinoma in a pygmy goat (Capra hircus hircus)

An adult, female pygmy goat showed clinical injury and later lameness caused by marked swelling and ulceration of the left lateral hind claw. Conservative treatments failed and due to the...