Ann Surg Treat Res.  2019 Oct;97(4):176-183. 10.4174/astr.2019.97.4.176.

Long-term postresection prognosis of primary neuroendocrine tumors of the liver

Affiliations
  • 1Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. shwang@amc.seoul.kr
  • 2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 3Department of Surgery, Inje University Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.

Abstract

PURPOSE
Primary hepatic neuroendocrine tumor (PHNET) is a very rare neoplasm, requiring strict exclusion of metastasis from possible extrahepatic primary sites for its diagnosis.
METHODS
We reviewed our clinical experience of 13 patients with primary hepatic NET who underwent liver resection from January 1997 to December 2015.
RESULTS
The mean age of the 13 patients (8 males and 5 females) was 51.1 ± 12.8 years; the most common clinical manifestation was vague, nonspecific abdominal pain (n = 9). Of them, 11 patients underwent preoperative liver biopsy, 7 of which correctly diagnosed as neuroendocrine tumor (NET). Ten patients underwent R0 resection, and 3 underwent R1 resection. Diagnosis of PHNET was confirmed both immunohistochemically and by absence of extrahepatic primary sites. All tumors were single lesions, with a mean size of 9.6 ± 7.6 cm and a median size of 4.3 cm; all showed positive staining for synaptophysin and chromogranin. During a mean follow-up period of 95.1 ± 86.6 months, 7 patients died from tumor recurrence, whereas the other 6 remain alive to date, making the 5-year tumor recurrence rate 56.0% and the 5-year patient survival rate 61.5%. When confined to R0 resection, 5-year recurrence and survival rates were 42.9% and 70.0%, respectively. Univariate analysis showed that Ki-67 proliferative index was the only risk factor for tumor recurrence.
CONCLUSION
PHNET is a very rare tumor with no specific clinical features, and its final diagnosis depends primarily on pathology, immunohistochemistry, and exclusion of metastasis from other sites. Aggressive surgical treatment is highly recommended for PHNET because of acceptably favorable postresection outcomes.

Keyword

Carcinoid tumor; Hepatectomy; Liver; Metastasis; Neuroendocrine tumor

MeSH Terms

Abdominal Pain
Biopsy
Carcinoid Tumor
Diagnosis
Follow-Up Studies
Hepatectomy
Humans
Immunohistochemistry
Liver*
Male
Neoplasm Metastasis
Neuroendocrine Tumors*
Pathology
Prognosis*
Recurrence
Risk Factors
Survival Rate
Synaptophysin
Synaptophysin

Figure

  • Fig. 1 CT and gross findings. In the case 10 patient, the CT image of the arterial phase shows a huge hypervascular mass identified with a background of chronic liver disease (A); and the portal-phase CT image shows tumor hypervascularity with central necrosis (B). In the case 12 patient, the CT image of the arterial phase shows a 5-cm-sized well-demarcated mass in the left liver (C); and the gross photograph of the resected liver specimen shows a well-demarcated intrahepatic mass (D).

  • Fig. 2 Microscopic examination of a primary neuroendocrine tumor of the liver. (A) Neoplastic cells are arranged in combined patterns as trabecular arrangement structures and solid nests (H&E, ×100). (B) Immunohistochemical staining with an antibody to synaptophysin shows neuroendocrine differentiation (×200).

  • Fig. 3 Cumulative tumor recurrence and overall patient survival curves in all 13 patients (A) and in the 10 patients who underwent R0 resection (B).


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젊은 나이에 발생하는 원발성 간 신경내분비 종양 1예
Sanghwa Song, Yangseok Koh
Korean J Gastroenterol. 2022;79(1):35-40.    doi: 10.4166/kjg.2021.139.


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