Diagnostic problem of Primary Hepatic Neuroendocrine Tumor in 18-year-old woman: A case report

Primary Hepatic Neuroendocrine Tumor (PHNET) is a very rare case and it's quite challenging to diagnose and treat. There are approximately 300 cases reported. Among them, only two giant PHNET cases were found in children under 20 years old.

We reported an unusual giant PHNET that was found in 18 years old female followed by intra-tumoral bleeding, which was initially misdiagnosed as Hemangioma. Abdominal Computed Tomography (CT) imaging showed a large liver mass with the peripheral enhancing nodular solid part in the delayed phase, which resembled Hemangioma.

Preoperative diagnosis of a Primary Hepatic Neuroendocrine Tumor is difficult as the liver often becomes the site of Neuroendocrine Tumor metastasis from other gastrointestinal organs, rarely occurred in people under 20 years of age, and the CT scan looks like Hemangiomas. The clinical symptoms are not typical, so other specific diagnostic tools for Neuroendocrine Tumor such as Chromogranin A level and 24-hour 5-hydroxyindoleacetic acid urine test are not performed. The indication of hepatic resection is made based on the preoperative diagnosis of other conditions such as large tumor size or tumor complications. Intraoperative diagnosis is also not typical for PHNET, so the definitive diagnosis of PHENT is made from post-resection histopathology.

The preoperative diagnosis of this rare tumor is still a big challenge. The physicians should be alert when finding Giant Hemangioma of the Liver, and Neuroendocrine Tumor should be the differential diagnosis. Blood Chromogranin A, urine 5-hydroxyindoleacetic acid test, and Positron Emission Tomography (PET) scan should also be considered.