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

Murakami, Makoto; Katayama, Kanji; Kato, Shigeru; Fujimoto, Daisuke; Morikawa, Mitsuhiro; Kono, Kenji; Hirono, Yasuo; Goi, Takanori

doi:10.1186/s40792-016-0269-8

Cited by 12 publications

(9 citation statements)

References 23 publications

(21 reference statements)

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“…NECs could also exhibit an abnormal high uptake of 18 F-fluorodeoxyglucose on positron emission tomography. [ 30 ] Upstream bile duct dilations, lymph node involvement and adjacent stricture invasions are frequently observed on imaging. Similar to previous cases of NECs in the EHBT, our patient displayed obstructive jaundice, elevated serum CEA and CA19-9, and without hormone-related signs or specific imaging features.…”

Section: Discussionmentioning

confidence: 99%

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Neuroendocrine carcinoma in the extrahepatic biliary tract

et al. 2018

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Rationale:Neuroendocrine carcinoma (NEC) arising from the extrahepatic biliary tracts (EHBTs) is rare, and thus its management and prognosis remain poorly clarified. We herein describe a case of NEC in the perihilar EHBTs, and review the literature, together with a comparison between NECs in the perihilar and distal EHBTs, to elucidate the management strategy and oncological outcome of this rare entity.Patient concerns:A 62-year-old Chinese male was admitted with complaints of painless jaundice. Imaging studies revealed a 2-cm mass in the hepatic hilum, regional lymph node involvement, and severe stenosis at the junction of the common hepatic ducts.Diagnoses:The histopathological examination of the resected specimen demonstrated small tumor cells with round hperchromatic nuclei and scant cytoplasm. A detailed immunohistochemical analysis showed that the tumor was strongly positive for synaptophysin, CD56 and chromogranin A, with a Ki-67 labeling index greater than 80%. These results led to a diagnosis of NEC in the perihilar bile duct.Interventions:The patient underwent surgical resection including a left hemihepatectomy, cholecystectomy, lymphadenectomy and Roux-en-Y hepaticojejunostomy.Outcomes:During the two months of follow-up, repeated imaging studies indicated tumor recurrence in the liver. The patient died 6 months after surgery.Lessons:NEC in the EHBTs is extremely challenging to diagnose preoperatively because of mimicking other bile duct cancers. The prognosis of this disease entity is dismal, and most patients die within 2 years after diagnosis. Subtyping of NECs into perihilar NECs and distal NECs is beneficial for clinical applications, including guiding therapy selection and predicting survival.

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Section: Discussionmentioning

confidence: 99%

“…Only 5 cases of LCC have been reported in the literature. [ 21 , 27 , 28 , 30 , 32 ] Compared to SCC, LCC tumor cells are larger in size and have a lower nuclear to cytoplasmic ratio. IHC analysis is required to confirm histologic identification.…”

Section: Discussionmentioning

confidence: 99%

Neuroendocrine carcinoma in the extrahepatic biliary tract

et al. 2018

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“…in the extrahepatic bile duct. 6 The etiology of NENs in the extrahepatic bile duct is unclear, though it might be argued they originate from neuroendocrine cells at sites of intestinal or gastric-type metaplasia induced by chronic inflammation. [7][8][9] Indeed, intestinal or gastric-type metaplasias are not uncommon in non-neoplastic mucosa adjacent to a variety of NENs of the gallbladder, including carcinoid tumor, small cell NEC, and LCNEC.…”

Section: Discussionmentioning

confidence: 99%

Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct

Park

Jeon

2018

Korean J Gastroenterol

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Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT. (Korean J Gastroenterol 2018;72:318-321)

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“…Diagnosis is usually made after cholecystectomy for symptomatic cholelithiasis with histo-pathological and immunohistochemical analysis as outlined above. Pre-operative brush biopsies often provide high false-negative results and therefore are of little use [12]. These tumors are highly aggressive and present with, or quickly develop metastasis, most commonly to regional lymph nodes and the liver, which results in an overall poor prognosis [10, 13–15].…”

Section: Discussionmentioning

confidence: 99%

Biliary tract large cell neuroendocrine carcinoma: current evidence

Raiker

Chauhan

Hasanein

et al. 2019

Orphanet J Rare Dis

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BackgroundPrimary neuroendocrine carcinomas of the gallbladder and biliary tract are rare, with pure large cell neuroendocrine carcinomas (LCNEC) being exceedingly rare and with a particularly poor prognosis.MethodsWe performed a review of published data on biliary tract large cell neuroendocrine carcinomas in PubMed.ResultsPreliminary search revealed over 2000 results but we found only 12 cases of pure large cell neuroendocrine carcinomas of biliary tract noted in literature to date. Because it commonly presents with non-specific symptoms of abdominal pain and jaundice, diagnosis is made after resection with histo-pathological and immunohistochemical analysis. These cancers are particularly aggressive with high recurrence rates, most often presenting with metastasis to regional lymph nodes and/or the liver resulting in a poor prognosis. Overall, complete surgical excision with systemic chemotherapy is the treatment mainstay. If the cancer is unresectable due to multiple metastases, medical management with systemic chemotherapy is the primary treatment modality.ConclusionThe prognosis of hepatobiliary LCNEC remains poor with median survival of only 11 months from initial diagnosis. Studies focusing on high grade neuroendocrine carcinoma are needed to enhance our understanding of biology and therapeutics in this rare but aggressive cancer.

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

Cited by 12 publications

References 23 publications

Neuroendocrine carcinoma in the extrahepatic biliary tract

Neuroendocrine carcinoma in the extrahepatic biliary tract

Large Cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct

Biliary tract large cell neuroendocrine carcinoma: current evidence

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