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

Abstract

Background: Primary hepatic neuroendocrine carcinoma (PHNEC) is rarer than extrahepatic gastrointestinal neuroendocrine 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 patients were AFP -and CA199-. Seven patients had solid tumors with cystic changes on ultrasonography, CT and/or MRI. For the initial treatment, four patients received combined-therapy and six monotherapy. Considering overall treatment, six patients received combined-therapy and four patients monotherapy. Staging criteria of primary 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 clinical data were gathered. Results: The median follow-up duration was 38.5 months. The 1-year, 2-year, 3-year and 6-year disease-free survival was 80.0%, 46.2% and 46.2% and 0% respectively. The overall survival rates were 100%, 67.1%, 67.1% and 33.6% respectively. Patients in early-stages (Ⅰ/Ⅱ) had similar disease-free and overall survival as those in advanced-stages (Ⅲ/Ⅳ). Patients with monotherapy had significant shorter disease-free and overall survival than the patients with combination-therapy. Conclusions: PHNEC has a unique specificity during its occurrence and development. The staging criteria of PHC might not be suitable for the PHENT. More convenient 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 with PHNEC or recurrence to get the best effectiveness; they might extend the disease-free and overall survival.

Combination therapies for primary hepatic neuroendocrine carcinoma: a case report

“…Currently, surgery is the only curative option and provides the most favorable outcome, including long-term survival [ 15 ]. Only 33 reports of surgery in PHNEC patients have been published in the literature (Table 1 ) [ 2 , 4 , 7 , 10 , 11 , 13 – 15 , 19 , 20 , 22 – 24 , 26 ]. Park et al reported on three patients with resectable tumors who were alive 17.7 months after treatment (range, 15.2–36.9 months) and on nine patients whose tumors could not be surgically removed but who survived for 11.3 months (range, 0.7–41.7 months) [ 20 ].…”

“…It is a reason why adjuvant chemotherapy after curative resection should be considered, although no prospective studies are available to support this practice. While resection of all tumors could lead to a higher survival rate and better outcomes, many patients will still require combined therapy, such as transcatheter arterial chemoembolization, chemotherapy, and radiofrequency ablation [ 26 ].…”

“…While resection of all tumors could lead to a higher survival rate and better outcomes, many patients will still require combined therapy, such as transcatheter arterial chemoembolization, chemotherapy, and radiofrequency ablation [ 26 ].…”

“…There is still no report of typical treatment for recurrence in PHNEC. In Wang’s series, combined therapy resulted in better outcomes than monotherapy even in patients with recurrence [ 26 ]. Tumor progression can be controllable with antitumor agents, and tumor burden can be reduced without the evidence of other recurrence, allowing subsequent excision in the tumor.…”

“…Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with roughly 64 cases reported in the English-language literature until 2016 [ 1 – 26 ]. Because of the rarity of PHNEC, its clinical features and treatment outcomes are not well understood.…”