Abstract

A 70-year-old Japanese woman who was treated for interstitial pneumonia (IP) with steroid therapy developed cholecystitis. A serial computed-tomography (CT) imaging showed irregular thickness of the fundus wall of the gallbladder and two rapidly enlarged lymph nodes (LNs): number (no.) 12 and no. 8a. Positron-emission tomography-computed tomography (PET-CT) scan showed an abnormal uptake at the site of the gallbladder tumor and those LNs. We subsequently performed open radical cholecystectomy and LN dissection of the no. 12 and 8a LNs, following complete remission of IP. The histology showed gallbladder adenocarcinoma, with a single focus of neuroendocrine carcinoma (NEC) component of less than 30%; Ki-67 index > 80%, synaptophysin (Syn) (+), chromogranin A (CgA) (+), and clusters of differentiation (CD) 56 (+) (T2bN1M0, Stage IIIB). LN no. 8a was diffusely metastatic with NEC components. LN no. 12c, which was adjacent to the cystic duct, revealed necrosis without apparent tumor cells, but was highly suspicious for tumor necrosis. The final diagnosis was adenocarcinoma of the gallbladder with focal NEC (< 30%), which did not meet the criteria for mixed neuroendocrine–non-neuroendocrine neoplasm (MiNEN). Postoperatively, she completed 4 cycles of adjuvant chemotherapy for NEC (Cisplatin plus Etoposide), and no recurrence was observed after 12 months.

Highlights

  • The occurrence of gallbladder neuroendocrine carcinoma (NEC) is very rare, which is reported to be 2.2% of all gallbladder cancers, which usually presents with advanced stage, lymph nodes (LNs) metastases, and poor prognosis [1]

  • While the patient was treated for interstitial pneumonia (IP), we were able to follow the rapid progression of the gallbladder adenocarcinoma with focal NEC (< 30%) and LN metastasis, over the course of 4 months with serial computed-tomography (CT) imaging

  • LN no. 8a was diffusely metastatic with NEC components, which were positive for Syn, chromogranin A (CgA), and clusters of differentiation (CD) 56, and was the only LN that showed clear metastasis based on histology (Fig. 4)

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Summary

Introduction

The occurrence of gallbladder neuroendocrine carcinoma (NEC) is very rare, which is reported to be 2.2% of all gallbladder cancers, which usually presents with advanced stage, lymph nodes (LNs) metastases, and poor prognosis [1]. While the patient was treated for interstitial pneumonia (IP), we were able to follow the rapid progression of the gallbladder adenocarcinoma with focal NEC (< 30%) and LN metastasis, over the course of 4 months with serial computed-tomography (CT) imaging. A follow-up CT scan, which was performed 2 months later, revealed irregular thickening of the gallbladder fundus wall in addition to two enlarged LNs; number (no.) 12 and no.8a, 1.5 cm and 0.7 cm, respectively.

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