Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review

Primary hepatic neuroendocrine neoplasms (PHNENs) are a rare type of neuroendocrine tumors originating in the liver. These tumors are characterized by non-specific clinical symptoms and atypical imaging features, making differentiation from other primary hepatic masses, such as hepatocellular carcin...

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Main Authors: Xue Luo, Xingxing Bie, Na Luo, Wenting Huang
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-07-01
Series:Frontiers in Oncology
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Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2025.1555736/full
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author Xue Luo
Xingxing Bie
Na Luo
Wenting Huang
author_facet Xue Luo
Xingxing Bie
Na Luo
Wenting Huang
author_sort Xue Luo
collection DOAJ
description Primary hepatic neuroendocrine neoplasms (PHNENs) are a rare type of neuroendocrine tumors originating in the liver. These tumors are characterized by non-specific clinical symptoms and atypical imaging features, making differentiation from other primary hepatic masses, such as hepatocellular carcinoma (HCC) and parasitic lesions, challenging. The diagnosis of PHNENs is based on characteristic histological features associated with this condition and results from immunohistochemistry assays. Here, we report on a case of a 22-year-old female presenting with a large hepatic neoplastic lesion. Computed tomography (CT) imaging results revealed a highly vascularized mass with clear boundaries located in the right lobe of the liver, suggesting a diagnosis of HCC. The patient underwent a fine-needle aspiration biopsy and subsequent complete surgical resection of the tumor. Results from both the fine-needle aspiration and histology of the surgically resected specimen showed that the tumor cells were arranged in a solid structure with a trabecular pattern. The tumor cells exhibited positive expressions for the epithelial cell markers AE1/AE3, along with the neuroendocrine markers, synaptophysin (Syn), chromogranin (CgA), and CD56 as based on results from immunohistochemical staining. The Ki-67 proliferation index was > 20%, and the mitotic count was > 20 per 2 square millimeters, leading to a final diagnosis of a hepatic neuroendocrine neoplasms, Grade 3 (G3). PHNENs are extremely rare, and, to our knowledge, there have been no reports in the literature of adolescents or young adults diagnosed with the G3-type.
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spelling doaj-art-002b9f857d7c43aa976899ff8a8ed9b22025-08-20T02:38:23ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-07-011510.3389/fonc.2025.15557361555736Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature reviewXue Luo0Xingxing Bie1Na Luo2Wenting Huang3Department of Pathology, National Cancer Center, National Clinical Research Center for Cancer, Cancer Hospital and Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, Guangdong, ChinaDepartment of Thoracic Surgery, The Second Affiliated Hospital of Shenzhen University, Shenzhen, Guangdong, ChinaDepartment of Pathology, National Cancer Center, National Clinical Research Center for Cancer, Cancer Hospital and Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, Guangdong, ChinaDepartment of Pathology, National Cancer Center, National Clinical Research Center for Cancer, Cancer Hospital and Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, Guangdong, ChinaPrimary hepatic neuroendocrine neoplasms (PHNENs) are a rare type of neuroendocrine tumors originating in the liver. These tumors are characterized by non-specific clinical symptoms and atypical imaging features, making differentiation from other primary hepatic masses, such as hepatocellular carcinoma (HCC) and parasitic lesions, challenging. The diagnosis of PHNENs is based on characteristic histological features associated with this condition and results from immunohistochemistry assays. Here, we report on a case of a 22-year-old female presenting with a large hepatic neoplastic lesion. Computed tomography (CT) imaging results revealed a highly vascularized mass with clear boundaries located in the right lobe of the liver, suggesting a diagnosis of HCC. The patient underwent a fine-needle aspiration biopsy and subsequent complete surgical resection of the tumor. Results from both the fine-needle aspiration and histology of the surgically resected specimen showed that the tumor cells were arranged in a solid structure with a trabecular pattern. The tumor cells exhibited positive expressions for the epithelial cell markers AE1/AE3, along with the neuroendocrine markers, synaptophysin (Syn), chromogranin (CgA), and CD56 as based on results from immunohistochemical staining. The Ki-67 proliferation index was > 20%, and the mitotic count was > 20 per 2 square millimeters, leading to a final diagnosis of a hepatic neuroendocrine neoplasms, Grade 3 (G3). PHNENs are extremely rare, and, to our knowledge, there have been no reports in the literature of adolescents or young adults diagnosed with the G3-type.https://www.frontiersin.org/articles/10.3389/fonc.2025.1555736/fullliverneuroendocrine neoplasmssynaptophysinchromograninsurgery
spellingShingle Xue Luo
Xingxing Bie
Na Luo
Wenting Huang
Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
Frontiers in Oncology
liver
neuroendocrine neoplasms
synaptophysin
chromogranin
surgery
title Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
title_full Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
title_fullStr Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
title_full_unstemmed Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
title_short Primary giant hepatic neuroendocrine neoplasms in a young adult: case report and literature review
title_sort primary giant hepatic neuroendocrine neoplasms in a young adult case report and literature review
topic liver
neuroendocrine neoplasms
synaptophysin
chromogranin
surgery
url https://www.frontiersin.org/articles/10.3389/fonc.2025.1555736/full
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