Characteristics of Neuroendocrine Carcinomas of the Gallbladder

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In a Korean study reported in the American Journal of Surgical Pathology, Lee et al identified clinicopathologic and immunohistochemical characteristics of neuroendocrine carcinomas of the gallbladder.

Study Details

The study involved data from 34 patients with gallbladder neuroendocrine carcinomas who had undergone surgery between January 2008 and December 2016 at Asan Medical Center, Seoul. Overall, 68% of patients were women, and median age at diagnosis was 63 (range = 37–82 years). 

Key Findings

Histologic features included the following:

  • Tumor location was the fundus in 44% of cases, body/fundus in 24%, body in 18%, and neck in 15%.
  • In total, 65% were mass-forming lesions and 35% infiltrating lesions.
  • Half were of small-cell type and half were of large-cell type.
  • Twenty-three cases (68%) were associated with biliary intraepithelial neoplasia (38%) and intracholecystic papillary neoplasm (29%).
  • Growth pattern was diffuse in 74% and organoid in 24%.
  • Perilobular pseudopalisading, nuclear molding, or rosette/pseudorosette formation was present in 88%. 

Immunohistochemical characteristics included the following:

  • Cytokeratin 7 was expressed in 56% of cases and cytokeratin 20 in 24%.
  • Diffuse or focal membranous or cytoplasmic labeling for CD117 was present in 74%.
  • Loss of Rb1 expression and accompanying overexpression of p16 were observed in 74% of cases, with three tumors (9%) expressing both Rb1 and p16.
  • No BRAF V600E mutations were identified in any tumors.  

Complete resection was achieved in 91% of patients. Twenty-five patients (68%) received adjuvant chemoradiation. Among all patients, 1-, 3-, and 5-year overall survival rates were 64%, 35%, and 19%, respectively. On univariate analysis, increasing American Joint Committee on Cancer stage (hazard ratio [HR] = 1.79, P = .032) and receipt of adjuvant chemoradiation (HR = 0.25, P = .002) were significantly associated with survival; on multivariate analysis, only receipt of adjuvant therapy remained significant (HR = 0.295, P = .007). For patients who received vs had not received chemoradiation, 1-year overall survival rates were 68% vs 22% and median overall survival was 19 months vs 10 months.

The authors stated, “The complete resection and application of postoperative adjuvant therapy may influence a better clinical outcome in patients with neuroendocrine carcinomas of the gallbladder.”

Sun Mi Lee, MD, of the Department of Pathology, Jeju National University Hospital, Jeju-si, is the corresponding author for the American Journal of Surgical Pathology article.

Disclosure: The study was supported by a grant from Jeju National University Hospital. For full disclosures of the study authors, visit

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