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

  • Wang, Li-Ming (Department of Abdominal Surgery, Cancer Institute & Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • An, Song-Lin (Department of Abdominal Surgery, Cancer Institute & Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College) ;
  • Wu, Jian-Xiong (Department of Abdominal Surgery, Cancer Institute & Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College)
  • 발행 : 2014.03.30

초록

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.

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피인용 문헌

  1. MR imaging of primary hepatic neuroendocrine neoplasm and metastatic hepatic neuroendocrine neoplasm: a comparative study vol.120, pp.11, 2015, https://doi.org/10.1007/s11547-015-0544-y
  2. Primary hepatic neuroendocrine neoplasm vol.97, pp.31, 2018, https://doi.org/10.1097/MD.0000000000011764
  3. Alpha-fetoprotein Secreting Neuroendocrine Carcinoma of the Liver: a Case Report and Literature Review pp.1941-6636, 2019, https://doi.org/10.1007/s12029-018-0140-1