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

Abstract

Extrahepatic bile duct cancer with an endocrine cell component has rarely been reported. We report here on a case of adenoendocrine cell carcinoma in the middle bile duct. An 82-year-old man was admitted to hospital for jaundice and anorexia. Computed tomography and magnetic resonance imaging examination showed a papillary low-density mass in the middle bile duct. Endoscopic retrograde cholangiography showed obstruction of the bile duct, and blushing cytology of the bile duct revealed an adenocarcinoma. We resected the extrahepatic bile duct with regional lymph node dissection. A pathological examination revealed a neuroendocrine component showing small cytoplasmic cells with hyperchromatic nuclei and a rosette-like structure in the middle of the tumor. In the peripheral mucosal region, there was a well-differentiated adenocarcinoma composed of columnar and cuboidal epithelial cells with clear and slightly granular eosinophilic cytoplasm. Immunohistochemical analysis showed positive staining for CD56, following the diagnosis of adenoendocrine cell carcinoma. The Ki-67 rate was >30% suggesting a small-cell endocrine carcinoma. The adenocarcinoma component infiltrated into the endocrine component, and some of the endocrine component was positive for cytokeratin, suggesting transdifferentiation of the adenocarcinoma into the endocrine component rather than originating from the common precursor cell. The patient experienced liver metastasis 3 months after the operation and died 6 months after the operation. Adenoendocrine tumor of the bile duct is extremely rare and adjuvant chemotherapy is necessary according to the malignant potential of the neuroendocrine tumor rather than the adenocarcinoma.

Mixed neuroendocrine–non-neuroendocrine neoplasm of the bile duct with long-term prognosis after neoadjuvant chemotherapy

“…Furthermore, the lack of characteristic imaging findings of biliary MiN-ENs may lead to the diagnosis of cholangiocarcinoma once the ADC component is detected, thus interrupting efforts to detect the NEC component. In MiNEN, NEC is often the cancer component that triggers vascular invasion, liver metastasis, and lymph node metastasis [10,12], suggesting that histological examination of lymph nodes and liver metastases is likely to detect NEC components. In our case, only the ADC component was detected by transpapillary examination; however, the NEC component was detected by EUS-FNA of the primary tumor and enlarged lymph nodes.…”

Small Cell Neuroendocrine Carcinoma of the Parotid Gland: An Uncommon Example of Incompletely Encapsulated Tumor Including S100 Protein-Positive Clear Cells

“…Many MiNENs consist of a superficial, well-differentiated adenocarcinoma and an invasive neuroendocrine carcinoma; there are also a few reports claiming that the neuroendocrine carcinoma component is transitionally derived from the adenocarcinoma component in the biliary tract [21,22]. The relationship between these two components closely resembles that of the dedifferentiated component and a low-grade carcinoma as a precursor lesion in dedifferentiated carcinoma of the salivary glands.…”

A long-term recurrence-free survival of a patient with the mixed adeno-neuroendocrine bile duct carcinoma: A case report and review of the literature

“…The Pub med and Japan Medical Abstracts Society databases were searched using each of the following search terms: “bile duct”, “biliary tract”, “MANEC”, “mixed adenoneuroendocrine carcinoma”, “neuroendocrine carcinoma”, “small cell carcinoma”, “large cell carcinoma”, “adenoneuroendocrine carcinoma”, or “adenocarcinoid”. These searches identified 34 patients who had undergone surgical resection of MANEC, including our patient [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] ( Table 1 ).…”