Gallbladder neuroendocrine neoplasm: a case report and critical evaluation of WHO classification

Abstract

Abstract. Gallbladder neuroendocrine neoplasms (GB-NENs) are rare. The majority of GB-NENs are poorly differentiated, with increased mitotic activity and clinically aggressive course. Surgery is the only curative approach and the optimal medical treatment is uncertain. In this report we describe the case of a woman affected by metastatic well differentiated GB-NEN with increased Ki 67. The patient underwent surgical removal of the gallbladder neoplasm and showed disease recurrence with pulmonary and liver metastases. After achieving a partial chemotherapy response, the patient rapidly died due to progressive disease. This case raises important issues. Well differentiated NENs with a high proliferative index are not included as a specific entity in any of the most widely used nomenclature systems. Moreover considering the proliferative index of the disease, it is reasonable to consider the patient a candidate for chemotherapy. Nevertheless, recently published papers raise the possibility that well differentiated NENs and specific proliferative index cutoff can predict low chemosensitivity in patients with highly proliferative neuroendocrine carcinoma.

Age‐specific clinicopathological characteristics and prognostic analysis of neuroendocrine carcinomas of the gallbladder

“…7 , 13 , 28 Possible reasons include the high proportion of advanced AJCC stages (stage III: 28.6%; Stage IV: 50%), poor histological grade (100% poorly differentiated), low rate of adjuvant therapy (42.9%), and poor margin status (75% R0 resection). 29 , 30 Yan et al reported that the median survival time of NEC patients was 20.4 months, which is longer than that of this study (10.0 months). Their AJCC staging (stage III: 46.7%; stage IV: 26.7%) and histological grade (66.7% well differentiated) were relatively better than in patients in our study, which could lead to a longer survival time.…”

“…The overall survival rates of NEC patients in this study were relatively lower than in previous studies 7,13,28 . Possible reasons include the high proportion of advanced AJCC stages (stage III: 28.6%; Stage IV: 50%), poor histological grade (100% poorly differentiated), low rate of adjuvant therapy (42.9%), and poor margin status (75% R0 resection) 29,30 . Yan et al reported that the median survival time of NEC patients was 20.4 months, which is longer than that of this study (10.0 months).…”

“…7,13,28 Possible reasons include the high proportion of advanced AJCC stages (stage III: 28.6%; Stage IV: 50%), poor histological grade (100% poorly differentiated), low rate of adjuvant therapy (42.9%), and poor margin status (75% R0 resection). 29,30 T A B L E 4 Characteristics of different surgical groups of NEC patients relatively better than in patients in our study, which could lead to a longer survival time. 7,13 Chen et al 18 reported that the median survival time in their study was 3 months, and only two of 10 patients in their study underwent radical resection and the rest got palliative therapy.…”

Clinicopathological characteristics and prognostic factors of patients with primary gallbladder neuroendocrine carcinomas

“…The exclusion criteria of the studies for literature review were as follows: (a) primary lesion arising from organs other than the gallbladder; (b) histological subtype of GB‐NET was not pathologically confirmed; (c) studies on GB‐NET or well‐differentiated MiNEN; (d) review articles, meta‐analyses, editorials, commentaries, or animal studies, etc; (e) the required clinicopathological data could not be extracted; and (f) insufficient data on the follow‐up or patient outcomes. After literature search data of additional 47 patients from 38 studies were obtained 5,7,9–44 . Finally, a total of 62 patients with GB‐NEC including poorly differentiated MiNEN were finally enrolled.…”