Intraductal papillary neoplasm of the bile duct accompanying biliary mixed adenoneuroendocrine carcinoma

Abstract

We present the first case of an intraductal papillary neoplasm of the bile duct (IPNB) accompanying a mixed adenoneuroendocrine carcinoma (MANEC). A 74-year-old woman presented with fever of unknown cause. Laboratory data revealed jaundice and liver injury. Contrast-enhanced computed tomography revealed a 20 mm polypoid tumor in the dilated distal bile duct, which exhibited early enhancement and papillary growth. Upper gastrointestinal endoscopy revealed mucus production from the papilla of Vater, characterized by its protruding and dilated orifice. Endoscopic ultrasonography visualized the polypoid tumor in the distal bile duct, but no invasive region was suggested by diagnostic imaging. Therefore, the initial diagnosis was IPNB. After endoscopic nasobiliary drainage, a pylorus-preserving pancreaticoduodenectomy was performed. Pathological examination of the resected bile duct revealed papillary proliferation of biliary-type cells with nuclear atypia, indicating pancreaticobiliary-type IPNB. In addition, solid portions comprised of tumor cells with characteristic salt-and-pepper nuclei were evident. Immunohistochemistry revealed expression of the neuroendocrine marker synaptophysin in this solid component, diagnosing it as a neuroendocrine tumor (NET). Furthermore, the MIB-1 proliferation index of NET was higher than that of IPNB, and microinvasion of the NET component was found, indicating neuroendocrine carcinoma (NET G3). This unique case of MANEC, comprising IPNB and NET, provides insight into the pathogenesis of biliary NET.

Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review

“…Clinical laboratory tests can show biliary obstruction, with elevated bilirubin, alkaline phosphatase, 5 0 nucleotidase, and gamma-glutamyl transpeptidase, especially with involvement of the common bile duct. 20,26 Although one case had a significantly elevated level of hepatic enzymes without liver involvement, contiguous liver invasion and metastases should be ruled out in such a scenario, particularly with elevated alkaline phosphatase. 24,25 Carbohydrate antigen 19-9 (CA 19-9) is probably the most useful serologic tumor marker, but elevation of serum carcinoembryonic antigen and chromogranin A have also been documented.…”

Carcinoma adenoneuroendocrino mixto de la ampolla de Vater: reporte de caso

“…La serie más grande publicada de 2.564 adenocarcinomas periampulares resecados mostró una supervivencia media estimada de 22 meses 9 , sin embargo, no menciona la presencia de tumores mixtos con componente neuroendocrino, por lo que la cifra de supervivencia media no se puede aplicar al caso clínico específicamente. En otros reportes la supervivencia en 5 años varía de 33 a 53% 10 . A su vez, el compromiso de ganglios linfáticos, los márgenes quirúrgicos positivos, el estadio avanzado y tumores poco diferenciados se asocian con una supervivencia significativamente menor después de la resección quirúrgica 11,12 .…”

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 ).…”