Abstract
We describe a primary hepatic neuroendocrine tumor of a 57-year-old Thai woman who presented in 2004 with a suspicious mass in the left hepatic lobe. She underwent left hepatectomy for the 10.5-cm mass, called intermediate grade neuroendocrine carcinoma of unknown origin, likely metastatic. The tumor recurred in 2007, then called recurrent primary hepatic neuroendocrine tumor (PHNET), and the patient underwent liver transplant. Because of similarity between the neuroendocrine tumor and a thyroid tumor—specifically, follicular-like characteristics—immunohistochemical stains for thyroglobulin, TTF1, and calcitonin were performed. However, all were negative. All imaging studies revealed no evidence of a primary lesion other than the liver mass. In 2008, the patient's liver transplant failed because of ischemic cholangiopathy, and she underwent a second liver transplant. Seven years later, in 2015, she presented with metastatic neuroendocrine tumor of intermediate grade to the lung, consistent with metastatic PHNET. She underwent left upper-lobe wedge resection to remove the tumor. The patient is alive with no evidence of disease at 13 years after initial diagnosis. This rare variant of PHNET had thyroid-like morphologic characteristics but there is no evidence of primary thyroid tumor or thyroid markers in the primary and recurrent hepatic tumors and lung metastasis.
Highlights
Primary hepatic neuroendocrine tumors (PHNETs) are rare and poorly described entities, with fewer than 150 cases described in the literature [1]
Neuroendocrine tumors (NETs) can arise virtually anywhere in the body, and approximately 70% of these rare tumors arise in the gastrointestinal tract and lungs [4]; the liver is the common site for metastasis, yet it is an uncommon site for tumor origin
PHNET primarily occurs in patients aged 40 to 50 years and is usually located in the right lobe of the liver [3]
Summary
Primary hepatic neuroendocrine tumors (PHNETs) are rare and poorly described entities, with fewer than 150 cases described in the literature [1]. Neuroendocrine tumors (NETs) in general are relatively rare tumors with an incidence rate of 2 per 100,000 cases of all gastrointestinal tract tumors [3]. In the few reported cases of PHNET and for the present patient, a palpable abdominal mass or abdomen discomfort may be the only presenting concern [1]. This presentation is in contrast to the classic symptoms of carcinoid syndrome that accompany 10% of all NET liver metastasis but rarely occur in PHNET. PHNETs tend to appear more vascular than metastatic because of a rich vascular supply from the hepatic artery [6], consistent with the magnetic resonance imaging (MRI) findings of the recurrent PHNET in this case
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