Published online Jun 28, 2018. doi: 10.3748/wjg.v24.i24.2640
Peer-review started: March 22, 2018
First decision: April 24, 2018
Revised: May 12, 2018
Accepted: May 26, 2018
Article in press: May 26, 2018
Published online: June 28, 2018
The female patient suffered a giant but slow-growing, mild symptom right hepatic tumor for 26 years before definitive surgery, and the diagnosis was confirmed as primary hepatic neuroendocrine tumor (PHNET). Surgical removal leads to a good long-term disease free survival.
The female patient had no obvious symptoms but abdominal distension and intermittent abdominal pain.
In the first surgery in 1984, tumor biopsy was performed and three differential diagnoses were considered: (1) Hepatocellular adenoma; (2) highly differentiated hepatocellular carcinoma; and (3) sinusoidal dilatation and hemorrhage.
No abnormal findings were found in preoperative laboratory tests, including blood regular test, liver and renal function, tumor markers, etc.
Computed tomography scan showed a huge space-occupying lesion of the right liver, with the heterogeneous internal density and multiple enveloped calcifications.
Histological and immunochemical exams confirmed acinar and daisy clumpy structures under light microscope. Some areas were beam-like and had rich sinusoids, and mitotic phases were rare (0-1/10 HPF). Cluster of differentiation 56 and synaptophysin were positive, and Ki-67 was < 1% in the tumor.
Patient accepts definitive surgery, and the tumor was removed thoroughly.
PHNET is a kind of very rare primary hepatic lesion. The symptoms, radiological findings, clinical course, therapy methods, and patients’ survival conditions are variable between different cases. Although PHNET should be a differential diagnosis in liver tumors, it is very difficult to confirm this diagnosis before pathological examination. Different therapy methods have been used to treat PHNET. Surgery should be the first choice if possible.
Neuroendocrine Tumor (NET), previously named carcinoid tumor, is a kind of rare tumor. NETs always originate from gastrointestinal tracts or bronchial lungs. NETs in liver are rare, and about 80% of them are metastasis tumors. PHNET is extremely rare, accounting for roughly 0.4% of all cases of NETs, and it is difficult to differentiate from other liver tumors, such as hepatocellular carcinoma and adenoma.
In this rare, slow growing PHNET case, we find that methods and the possibility of treatment may change along with technology progress, such that definitive treatment may be achieved after long-time conservative treatment.