Small-cell carcinoma of the extrahepatic bile duct: a case report and review of the literature

Abstract

A small-cell carcinoma of the extrahepatic bile duct in a 69-year-old woman is herein reported. A tumor measuring approximately 3 cm in diameter was located at the confluence of the common bile duct, cystic duct, and common hepatic duct. Histopathologically, the tumor was small-cell neuroendocrine carcinoma without any gland formation or differentiation to squamous cell carcinoma. Tumor cells were immunoreactive for epithelial markers such as epithelial membrane antigen and cytokeratin and for the neuroendocrine markers such as neuron-specific enolase, chromogranin A, and synaptophysin. Although the carcinomas in more than half of the reported cases have been reported to be associated with well-to-moderately differentiated squamous or glandular components, seven cases, including our case, showed the carcinomas without squamous or glandular components. According to the review of 16 previously reported cases and our case of small-cell carcinoma of the extrahepatic bile ducts, there is no significant difference in the clinicopathological findings, namely, age, sex, site of carcinoma, and prognosis between the cases with or without squamous or glandular components. No CD34-positive multipotent adult progenitor cells, which might be the origin of the small-cell carcinoma, were detected in the bile duct epithelium in our case.

Large-cell neuroendocrine carcinoma of the common bile duct: a case report and a review of literature

“…(autopsy)18Arakura [20]75MSCNECBh-Bs6.5 cmChemo. and irradiation10 m, dead19Hosonuma [21]69FSCNECBs-Bm3 cmBiliary drainage2 m, alive20Okamura [22]62MSCNECBm3 cmNAC, resection, and irradiation20 m, dead21Yamaguchi [23]77FNECBiN.A.Resection and chemo.27 m, alive22Demoreuil [24]73MLCNEC + Adenoca.Bh-Bs3 cmResection and chemo.12 m, dead23Cho [25]59FSCNECBm3 cmResection6 m, dead24Sasatomi [26]76MLCNECBh-Bs5 cmResection21 days, dead25Ninomiya [27]75FLCNECBm3 cmResection14 m, alive26Park [28]75FLCNECBm2.7 cmResection and chemo.12 m, dead27Kihara [29]70FSCNECBh5 cmResection and chemo.10 m, alive28Current report79MLCNEC + Adenoca.Pm2.9 cmResection3 m, dead NEC neuroendocrine carcinoma, NAC neoadjuvant chemotherapy, Adenoca. adenocarcinoma, CBD common bile duct, Bh hilar bile duct, Bs superior portion of common bile duct, Bm mid-portion of bile duct, Bi inferior portion of bile duct, chemo .…”

“…Because peritoneal, lung, and liver metastases developed during the early postoperative period, it was hard to attempt any adjuvant chemotherapy in LCNEC. If a diagnosis of LCNEC of the CBD was possible preoperatively, we could consider neoadjuvant multimodal treatments before resection to improve the prognosis [11, 21, 32]. However, it was difficult to diagnose NEC of the CBD preoperatively, because there is no difference between adenocarcinoma and NEC in symptoms, blood tests, and imaging studies.…”

Composite small cell and mucinous carcinoma originating from the intrahepatic bile duct: report of a case

“…Extrahepatic biliary small cell carcinomas have been categorized into two histological types, including the pure and mixed types [2][3][4][5][6][7][8][9][10][11][12]. Of the 10 extrahepatic cases reported previously, four were pure and six cases were mixed types.…”

“…The biliary tract is a very rare site for extrapulmonary small cell carcinoma [2][3][4][5][6][7][8][9][10][11][12]. So far, only 10 cases of small cell carcinoma with an extrahepatic bile duct origin have been reported in the English literature [2][3][4][5][6][7][8][9][10][11][12]. Only one case with an intrahepatic bile duct origin has been reported to date, by Koplin et al [12], although they used the term ''hepatic carcinoid tumor''.…”

Management of primary hepatopancreatobiliary small cell carcinoma

“…SCC arises in cells of amine precursor and decarboxylation (APUD) origin, which have been identified in almost every organ system 1, 2. Unlike neuroendocrine cancers that also arise from APUD cells, SCC is predominantly composed of anaplastic or poorly differentiated cells, although segments of well‐differentiated squamous or glandular tissue may also be present 1, 3. Since its original description as a primary pulmonary neoplasm in 1926, several reports have confirmed primary SCC in the pancreas, bile ducts, and gallbladder 1–7.…”