Primary Hepatic Neuroendocrine Tumor Arising at a Young Age: Rare Case Report and Literature Review

SONG, Sanghwa; Koh, Yang Seok

doi:10.4166/kjg.2021.139

Cited by 5 publications

(4 citation statements)

References 19 publications

(25 reference statements)

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“…The size of the tumor is 1.5–27 cm, presenting as single (76.3%) and multiple in 23.7% of cases, located mainly in the right lobe in 48.4% of cases, and as bilobular in 18.5%. Knox et al [54] reported extrahepatic involvement in 18.6% of cases, such as bone (60%), lymph node (60%), and lung (40%) [9‒55]. The form of tumor presentation was two liver tumors that occupied both lobes of approximately 20 cm with metastasis to the mesentery.…”

Section: Discussionmentioning

confidence: 99%

“…Indications for RFA are the presence of three or fewer tumors and a tumor diameter of ≤5 cm. Tumors located near the major branches of the portal and hepatic veins have a higher potential for incomplete ablation [ 10 , 55 , 56 ]. Approximately 20–37% of patients are diagnosed at the metastatic or advanced stage, where platinum-based chemotherapy (etoposide + cisplatin [EP] or carboplatin [EC]) is the first-line treatment according to The European Society for Medical Oncology (ESMO) guideline [ 57 ].…”

Section: Discussionmentioning

confidence: 99%

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Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report

Fernández-Ferreira,

Romero-López,

Robles-Aviña

et al. 2023

Case Rep Oncol

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Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 90 cases having been reported in the English-language literature. Among all neuroendocrine neoplasms, primary hepatic neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) are extremely rare, accounting for 0.3% of NETs and 0.28–0.46% of malignant liver tumors. Additionally, primary hepatic NECs occur infrequently. The clinical diagnosis of primary hepatic NEC remains challenging because of its rarity and the lack of information about its characteristic appearance on images. Consequently, pathological examination through the performance of a preoperative liver tumor biopsy is essential for diagnosis. Due to the lack of availability of substantial high-quality data, there is no standard therapy for primary hepatic NEC. We present the first case of PHNEC metastasized to the mesentery reported in the English-language literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report

Fernández-Ferreira,

Romero-López,

Robles-Aviña

et al. 2023

Case Rep Oncol

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“…In contrast, a different investigation revealed that 83.3% of PHNETs displayed mixed echogenicity, and suggested that cystic changes frequently occur in PHNETs ( 8 ). Therefore, cystic change may represent a feature of PHNETs ( 19 ), although this requires confirmation in a larger cohort. Occasionally, calcifications (21.4%) were also present in PHNETs, which is different from NBNC-HCC.…”

Section: Discussionmentioning

confidence: 99%

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

Tan

et al. 2023

Front. Oncol.

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ObjectiveThe 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).Materials and methodsFourteen 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.ResultsPHNET 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 ± 4.7 cm) was larger than that of NBNC-HCC (5.9 ± 3.8 cm) (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).ConclusionCEUS is helpful in discriminating between PHNET and NBNC-HCC. PHNETs mainly present as a single mass with a large size (>10 cm) 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.

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“…The multivariate Cox analysis in this study showed that older age (>76 years), neuroendocrine carcinoma, and chemotherapy treatment predicted poorer OS and DSS, while surgery predicted better OS and DSS. It is known that a PHNET is generally diagnosed at an older age ( 21 ); however, a case of a PHNET that occurred at a young age, which was misdiagnosed as hepatocellular carcinoma preoperatively, was reported in South Korea ( 22 ). This report was consistent with our results that age is an independent risk factor affecting the prognosis of patients with PHNETs.…”

Section: Discussionmentioning

confidence: 99%

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

Lin

Ding

et al. 2022

Front. Med.

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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.

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Primary Hepatic Neuroendocrine Tumor Arising at a Young Age: Rare Case Report and Literature Review

Cited by 5 publications

References 19 publications

Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report

Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report

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

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

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