Diagnosis and Therapy of Primary Hepatic Neuroendocrine Carcinoma: Clinical Analysis of 10 Cases

Background: Primary hepatic neuroendocrine carcinoma (PHNEC) is rarer than extrahepatic gastrointestinalneuroendocrine carcinoma (NEC). It is difficult to make a correct diagnosis and poses a challenge for management.Materials and
Methods: Ten PHNEC patients were admitted to our hospital from June 2006 to June 2011.Laboratory tests and imaging scans were performed for diagnosis and exclusion of extrahepatic NEC. All patientswere AFP - and CA199- . Seven patients had solid tumors with cystic changes on ultrasonography, CT and/orMRI. For the initial treatment, four patients received combined-therapy and six monotherapy. Consideringoverall treatment, six patients received combined-therapy and four patients monotherapy. Staging criteria ofprimary hepatocellular carcinoma (PHC, AJCC 7th edition) were used to differentiate the stage of all patients:3 patients were stage Ⅰ, 2 stageⅡ, 4 patients stageⅢ and 1 stageⅣ. All patients were followed up and clinicaldata were gathered.
Results: The median follow-up duration was 38.5 months. The 1-year, 2-year, 3-year and6-year disease-free survival was 80.0%, 46.2% and 46.2% and 0% respectively. The overall survival rates were100%, 67.1%, 67.1% and 33.6% respectively. Patients in early-stages (Ⅰ/Ⅱ) had similar disease-free and overallsurvival as those in advanced-stages (Ⅲ/Ⅳ). Patients with monotherapy had significant shorter disease-freeand overall survival than the patients with combination-therapy.
Conclusions: PHNEC has a unique specificityduring its occurrence and development. The staging criteria of PHC might not be suitable for the PHENT. Moreconvenient and effective features need to be found in imaging and laboratory detection. Surgical resection, TACE,chemotherapy and radiofrequency ablation should be performed in combination and actively for patients withPHNEC or recurrence to get the best effectiveness; they might extend the disease-free and overall survival.