Primary hepatic neuroendocrine tumor: A rare entity

Jain, Richa; Sakpal, Mallikarjun; Asthana, Sonal; Shankar, Mythri; Agarwal, Vijay; Mn, Vidya; Panchal, Nijalingappa; Raichurkar, Karthik; Kale, S.B.

doi:10.1016/j.radcr.2020.09.011

Cited by 5 publications

(7 citation statements)

References 10 publications

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“…According to reports thus far, most PHNETs appeared in the form of a solid nodular lesion, and only a few cystic cases have been reported. [4][5][6] In the present case, however, it was a large multilocular cystic mass with septation, which led to the erroneous impression of hepatic mucinous cystadenoma or cystadenocarcinoma. A biopsy with an immunohistochemical examination can be helpful in diagnosis, but the cystic features of the mass made the authors hesitate to perform a biopsy.…”

Section: Discussionmentioning

confidence: 85%

Cystic Primary Hepatic Neuroendocrine Tumor

Kim

Lee

Shin

et al. 2021

Korean J Gastroenterol

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Neuroendocrine tumors (NETs) can arise throughout the body. Most NETs in the liver are metastatic tumors; primary hepatic NET (PHNET) is extremely rare. A diagnosis of PHNET is very difficult. No single modality can diagnose PHNET by itself, and it often resembles other hypervascular masses of the liver. This paper reports the case of a 51-year old female with a large hepatic mass. Unlike most of PHNETs reported previously, it was composed of a solid mass with mainly multiple cystic lesions, which led to an erroneous diagnosis of hepatic mucinous cystadenoma or cystadenocarcinoma. PHNET with cystic lesions is extremely rare, and the features are not well studied. This case may help physicians suspect PHNET in a differential diagnosis of an atypical hepatic mass.

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Section: Discussionmentioning

confidence: 85%

Cystic Primary Hepatic Neuroendocrine Tumor

Kim

Lee

Shin

et al. 2021

Korean J Gastroenterol

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“… 5 Primary hepatic NETs account for approximately 0.3% of primary NETs. 6 They were first described in 1958 by Edmondson et al, and around 150 cases have been reported. 2 The origin is debated, with theories including ectopic pancreatic or adrenal cells, neuroendocrine tissue in the intrahepatic biliary epithelium, or chronic inflammation in the biliary tract causing metaplasia.…”

Section: Discussionmentioning

confidence: 99%

“…2 The origin is debated, with theories including ectopic pancreatic or adrenal cells, neuroendocrine tissue in the intrahepatic biliary epithelium, or chronic inflammation in the biliary tract causing metaplasia. 6 Primary hepatic NETs typically occur in patients aged 40-50 years, with no specific gender predominance, and no risk factors have been identified. 6 They can be functional, causing skin flushing, diarrhea, and abdominal pain due to hormone secretion (carcinoid syndrome), or be non-functional.…”

Section: Discussionmentioning

confidence: 99%

“…6 Primary hepatic NETs typically occur in patients aged 40-50 years, with no specific gender predominance, and no risk factors have been identified. 6 They can be functional, causing skin flushing, diarrhea, and abdominal pain due to hormone secretion (carcinoid syndrome), or be non-functional. 5,7 They grow slowly without symptoms and are often discovered incidentally at advanced stages.…”

Section: Discussionmentioning

confidence: 99%

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Primary Hepatic Neuroendocrine Carcinoma: A Rare Entity

Tidwell,

Thakkar,

Nguyen

et al. 2024

ACG Case Rep J

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Neuroendocrine tumors are typically low-malignancy growths arising from neuroectodermal cells of neural crest origin. Neuroendocrine carcinoma, on the other hand, represents a high-malignancy form of these tumors. While rare in the liver, they often indicate metastasis when present. We present a unique case of incidentally discovered primary hepatic neuroendocrine carcinoma. Initially, the patient's management was based on misleading radiological findings. However, histopathology confirmed the diagnosis, with subsequent imaging ruling out an extrahepatic source. Despite this, the patient opted against surgical intervention, resulting in a fatal outcome. This case underscores the critical importance of prompt diagnosis and intervention to avert adverse outcomes.

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“…Therefore, early diagnosis holds paramount importance in treatment decision-making and favorable prognosis for patients with PHNENs. The imaging manifestations of PHNENs reported in previous literature mainly consist of case reports ( 12 , 13 ) and lack systematic analysis. Hence, this study retrospectively analyzed clinical, pathological, and imaging data from 12 patients with PHNENs to enhance the accuracy of preoperative diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Primary hepatic neuroendocrine neoplasms: imaging characteristics and misdiagnosis analysis

Yang,

Li,

et al. 2024

Front. Oncol.

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ObjectiveTo analyze the CT and MR features of Primary hepatic neuroendocrine neoplasms (PHNENs) in order to enhance the diagnostic accuracy of this disease.MethodsA retrospective analysis was conducted on patients diagnosed with hepatic neuroendocrine neoplasms, excluding other sites of origin through general examination and postoperative follow-up. The CT and MR signs were analyzed according to the 2018 version of Liver Imaging Reporting and Data System (LI-RADS), along with causes of misdiagnosis.ResultsTwelve patients, including 6 males and 6 females, were enrolled in this study. There was no significant increase in liver tumor markers among all cases. Most masses were multiple (9/12), exhibiting low attenuation on pre-contrast CT scans, T1-hypointense signal, T2-hyperintense signal, and restricted diffusion. The majority of these masses (7/10) demonstrated similar rim arterial phase hyper-enhancement as well as peripheral “washout” during venous portal phase and delayed phase imaging. Three cases had incomplete capsules while one case had a complete capsule. Cyst/necrosis was observed in 7 out of all cases following administration of contrast agent, with 5 mainly distributed in the periphery. All masses lacked fat, calcification, vascular or bile duct tumor thrombus formation.ConclusionThe imaging findings associated with PHNENs possess certain specificity, often presenting as multiple masses within the liver accompanied by peripheral cyst/necrosis, similar rim arterial phase hyper-enhancement during venous portal phase and delayed phase imaging.

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Primary hepatic neuroendocrine tumor: A rare entity

Cited by 5 publications

References 10 publications

Cystic Primary Hepatic Neuroendocrine Tumor

Cystic Primary Hepatic Neuroendocrine Tumor

Primary Hepatic Neuroendocrine Carcinoma: A Rare Entity

Primary hepatic neuroendocrine neoplasms: imaging characteristics and misdiagnosis analysis

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