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Chen F, Li WW, Mo JF, Chen MJ, Wang SH, Yang SY, Song ZW. Neuroendocrine carcinoma of the common hepatic duct coexisting with distal cholangiocarcinoma: A case report and review of literature. World J Gastrointest Surg 2024; 16:1449-1460. [PMID: 38817298 PMCID: PMC11135321 DOI: 10.4240/wjgs.v16.i5.1449] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2024] [Revised: 03/13/2024] [Accepted: 04/15/2024] [Indexed: 05/23/2024] Open
Abstract
BACKGROUND Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is very rare, and the treatment and prognosis are unclear. Herein, we report the case of a middle-aged female with primary large cell NEC (LCNEC) of the common hepatic duct combined with distal cholangiocarcinoma (dCCA). Additionally, after a review of the relevant literature, we summarize and compare mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease. CASE SUMMARY A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months. Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign. Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels. Imaging examination revealed a 1-cm tumour in the middle and lower segments of the common bile duct. Pancreaticoduodenectomy + lymph node dissection was performed, and hepatic duct tumours were unexpectedly found during surgery. Pathology suggested poorly differentiated LCNEC (approximately 0.5 cm × 0.5 cm × 0.4 cm), Ki-67 (50%), synaptophysin+, and chromogranin A+. dCCA pathology suggested moderately differentiated adenocarcinoma. The patient eventually developed lymph node metastasis in the liver, bone, peritoneum, and abdominal cavity and died 24 months after surgery. Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours. CONCLUSION The prognosis of MiNEN and pure NEC alone is different, and the selection of treatment options needs to be differentiated.
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Affiliation(s)
- Fei Chen
- Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Wei-Wei Li
- Graduate School, Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Juan-Fen Mo
- The Key Laboratory, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Min-Jie Chen
- Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Su-Hang Wang
- Department of Pathology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Shu-Ying Yang
- Department of Intensive Medicine, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
| | - Zheng-Wei Song
- Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
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Zhang L, Wan D, Bao L, Chen Q, Xie H, Xu S, Lin S. Neuroendocrine carcinoma in the extrahepatic biliary tract: A case report and literature review. Medicine (Baltimore) 2018; 97:e11487. [PMID: 30024526 PMCID: PMC6086531 DOI: 10.1097/md.0000000000011487] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
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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Affiliation(s)
- Liang Zhang
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
| | - DaLong Wan
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
| | - Li Bao
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
| | - Qing Chen
- Department of Pathology, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - HaiYang Xie
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
| | - ShiGuo Xu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
| | - ShengZhang Lin
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University
- NHFPC Key Laboratory of Combined Multi-Organ Transplantation
- Key Laboratory of the Diagnosis and Treatment of Organ Transplantation, CAMS
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Murakami M, Katayama K, Kato S, Fujimoto D, Morikawa M, Koneri K, Hirono Y, Goi T. Large-cell neuroendocrine carcinoma of the common bile duct: a case report and a review of literature. Surg Case Rep 2016; 2:141. [PMID: 27888490 PMCID: PMC5124016 DOI: 10.1186/s40792-016-0269-8] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2016] [Accepted: 11/22/2016] [Indexed: 02/08/2023] Open
Abstract
Background Large-cell neuroendocrine carcinoma (LCNEC) of the bile duct is extremely rare and is a high-grade type of neuroendocrine tumor with an aggressive clinical course. Here, we report a case of LCNEC of the extrahepatic bile duct. Case presentation An 80-year-old man presented with severe jaundice. Endoscopic retrograde cholangiography and enhanced computed tomography revealed complete obstruction of the common bile duct (CBD) by a dense tumor measuring 1.5 cm in diameter. Although there were no malignant cells in the biliary brush cytology, we suspected a cholangiocarcinoma and performed extrahepatic bile duct resection. Histologically, the LCNEC occupied most of the places deeper than the stratum submucosum and an adenocarcinoma component, approximately 15%, was present in the mucosa. There were no transitional areas between the two components. Immunohistochemically, the LCNEC cells were reactive for CD56 and synaptophysin and had a high MIB-1 index (72%). The patient died of multiple liver, lung, and peritoneal metastases 3 months after surgery. Conclusions LCNEC of the CBD is particularly rare and has a very poor prognosis. Only five cases have been reported in the literature; therefore, there is no established effective therapy, including surgery, for LCNEC of the CBD at present. An accumulation of additional cases and further studies of multimodal treatment are required in the future to improve the prognosis.
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Affiliation(s)
- Makoto Murakami
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan.
| | - Kanji Katayama
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Shigeru Kato
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Daisuke Fujimoto
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Mitsuhiro Morikawa
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Kenji Koneri
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Yasuo Hirono
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
| | - Takanori Goi
- First Department of Surgery, School of Medicine, University of Fukui, 23 Shimoaiduki, Matsuoka, Eiheiji-cho, Fukui, 910-1193, Japan
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Kihara Y, Yokomizo H, Urata T, Nagamine M, Hirata T. A case report of primary neuroendocrine carcinoma of the perihilar bile duct. BMC Surg 2015; 15:125. [PMID: 26652845 PMCID: PMC4676104 DOI: 10.1186/s12893-015-0116-z] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2015] [Accepted: 12/07/2015] [Indexed: 12/15/2022] Open
Abstract
Background Although neuroendocrine tumors are most commonly found in the digestive system, neuroendocrine tumors originating from the bile duct are rare, and neuroendocrine carcinomas derived from the perihilar bile duct are extremely rare. This report presents the clinical course and clinicopathological features of neuroendocrine carcinomas arising from the extrahepatic bile duct. Case presentation A 70-year-old Japanese woman was preoperatively diagnosed with perihilar cholangiocarcinoma, and a radical resection with an extended left hepatic lobectomy and a choledochojejunostomy was performed. From the histopathological findings, we diagnosed the tumor as a neuroendocrine carcinoma of the bile duct (small cell type) with lymph node metastasis. The patient was treated with the same adjuvant chemotherapy as that used for small cell carcinoma of the lung. At 10 months after surgery, there was no recurrence of the disease. Conclusion Neuroendocrine carcinoma of the extrahepatic biliary tracts is a very rare and highly malignant disease with a poor prognosis. A multidisciplinary approach could improve the prognosis for this neoplasm.
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Affiliation(s)
- Yasuhiro Kihara
- Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan.
| | - Hiroshi Yokomizo
- Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan.
| | - Takahiro Urata
- Division of Gastroenterology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan.
| | - Michiko Nagamine
- Division of Diagnostic Pathology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan.
| | - Toshihiko Hirata
- Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan.
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5
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Nakamaru K, Ikeura T, Miyoshi H, Takaoka M, Danbara N, Horitani S, Masuda M, Yamada Y, Uemura Y, Toyokawa H, Satoi S, Kwon AH, Okazaki K. Successful Multidisciplinary Therapy for Small Cell Carcinomas Arising from the Extrahepatic Bile Duct. Intern Med 2015; 54:2711-5. [PMID: 26521898 DOI: 10.2169/internalmedicine.54.4603] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 74-year-old man was diagnosed with small cell carcinoma arising from the extrahepatic bile duct according to a histological examination of the biopsy specimen obtained during endoscopic retrograde cholangiopancreatography. Additionally, bulky hilar lymphadenopathy was observed, and the patient was treated with the combination of radiation and chemotherapy (cisplatin and irinotecan). Post-therapy, he underwent pancreaticoduodenectomy. The histological examination of the resected specimen revealed no residual cancer cells in the bile duct wall and a small amount of cancer cells in only a single lymph node. Due to this multidisciplinary therapy, the patient showed no signs of recurrence 12 months postoperatively.
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Affiliation(s)
- Koh Nakamaru
- The Third Department of Internal Medicine, Kansai Medical University, Japan
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Sasatomi E, Nalesnik MA, Marsh JW. Neuroendocrine carcinoma of the extrahepatic bile duct: Case report and literature review. World J Gastroenterol 2013; 19:4616-4623. [PMID: 23901240 PMCID: PMC3725389 DOI: 10.3748/wjg.v19.i28.4616] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2012] [Revised: 02/12/2013] [Accepted: 04/19/2013] [Indexed: 02/06/2023] Open
Abstract
Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.
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MESH Headings
- Aged
- Bile Duct Neoplasms/chemistry
- Bile Duct Neoplasms/pathology
- Bile Duct Neoplasms/surgery
- Bile Ducts, Extrahepatic/chemistry
- Bile Ducts, Extrahepatic/pathology
- Bile Ducts, Extrahepatic/surgery
- Biomarkers, Tumor/analysis
- Biopsy
- Carcinoma, Large Cell/chemistry
- Carcinoma, Large Cell/secondary
- Carcinoma, Large Cell/surgery
- Carcinoma, Neuroendocrine/chemistry
- Carcinoma, Neuroendocrine/secondary
- Carcinoma, Neuroendocrine/surgery
- Cholecystectomy
- Hepatectomy
- Humans
- Immunohistochemistry
- Lymphatic Metastasis
- Male
- Treatment Outcome
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7
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Ikegami T, Kayashima H, Sadanaga N, Morizono S, Nakashima A, Matsuura H, Shirabe K, Maehara Y. Composite small cell and mucinous carcinoma originating from the intrahepatic bile duct: report of a case. Surg Today 2012; 43:194-8. [PMID: 23001532 DOI: 10.1007/s00595-012-0331-5] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2011] [Accepted: 11/07/2011] [Indexed: 12/19/2022]
Abstract
The biliary tract is a very rare site for the occurrence of extrapulmonary small cell carcinoma. A 68-year-old Japanese female was being followed up for autoimmune hepatitis, and was referred to our hospital because segmental intrahepatic bile duct dilation was found on routine imaging studies, suggesting intrahepatic cholangiocarcinoma. She underwent left lobectomy of the liver and concomitant resection of the caudate lobe. Microscopic examination of the explanted liver showed a primary composite tumor comprising small cell and mucinous carcinomas that originated in the intrahepatic bile duct. Further immunohistochemical studies, including cytokeratin-19 and chromogranin-A staining, showed the two cellular components of the tumor to have similar characteristics. The amphicrine properties indicated that the tumor had a monoclonal origin but with biphenotypic differentiation, which was responsible for the histogenesis of this tumor.
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Affiliation(s)
- Toru Ikegami
- Department of Surgery, Saiseikai Fukuoka General Hospital, 1-3-46 Tenjin, Chuou-ku, Fukuoka 810-0001, Japan.
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8
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Manxhuka-Kerliu S, Petrusevska G, Maloku H, Sahatciu-Meka V, Loxha S, Loxha N, Shahini L. Hepatobiliary neuroendocrine carcinoma: a case report. J Med Case Rep 2010; 4:53. [PMID: 20370888 PMCID: PMC2836365 DOI: 10.1186/1752-1947-4-53] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2009] [Accepted: 02/18/2010] [Indexed: 11/12/2022] Open
Abstract
INTRODUCTION Neuroendocrine carcinoma of the gallbladder is a rather uncommon disease. We report a case of a neuroendocrine tumor that was located in the wall of the gallbladder and that extended into the liver. CASE PRESENTATION A 52-year-old Caucasian woman presented with right-sided abdominal pain, ascites and jaundice. An MRI scan revealed a tumor mass located in the gallbladder wall and involving the liver. A partial hepatectomy and cholecystectomy were performed. Histology revealed a neuroendocrine tumor, which showed scattered Grimelius positive cells and immuno-expressed epithelial and endocrine markers. Our patient is undergoing chemotherapy treatment. CONCLUSION Gastroenteropancreatic neuroendocrine tumors need a multidisciplinary approach, involving immunohistochemistry and molecular-genetic techniques.
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Affiliation(s)
- Suzana Manxhuka-Kerliu
- Faculty of Medicine, Institute of Pathology, University of Prishtina, Mother Theresa St, NN, 10 000 Prishtina, Kosovo
| | - Gordana Petrusevska
- Faculty of Medicine, Institute of Pathology, St Ciril & Methodius University, Vodnjanska NN, 1000, Skopje, Former Yugoslav Republic of Macedonia
| | - Halit Maloku
- Surgery Clinic, University Clinical Center of Kosovo, Mother Theresa St, NN, 10 000, Prishtina, Kosovo
| | - Vjollca Sahatciu-Meka
- Faculty of Medicine, University Clinical Center of Kosovo, Mother Theresa St, NN, 10 000, Prishtina, Kosovo
| | - Sadushe Loxha
- Faculty of Medicine, Institute of Pathology, University Clinical Center of Kosovo, Mother Theresa St. NN, 10 000, Prishtina, Kosovo
| | - Naim Loxha
- Surgery Clinic, Regional Hospital of Peja, Kosovo
| | - Labinot Shahini
- Faculty of Medicine, Institute of Pathology, University Clinical Center of Kosovo, Mother Theresa St, NN, 10 000, Prishtina, Kosovo
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Cho SB, Park SY, Joo YE. [Small cell carcinoma of extrahepatic bile duct presenting with hemobilia]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2009; 54:186-90. [PMID: 19844156 DOI: 10.4166/kjg.2009.54.3.186] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
We report a case of small cell carcinoma of extrahepatic bile duct presenting with jaundice and hemobilia. A 59-year-old woman was admitted due to right upper quadrant pain and jaundice. An abdominal computed tomography revealed a 2 cm sized mass in the extrahepatic bile duct. Endoscopic retrograde cholangiopancreatography revealed bloody discharge coming out of the papillary orifice in endoscopic view and a dilated extrahepatic bile duct with multiple irregular filling defects in cholangiogram. A coronal T2-weighted image revealed a hyperintense mass at extrahepatic bile duct. Laparotomy was performed, and pathologic examination of resected specimen showed tumor cells having round to oval nuclei with coarsely granular chromatin and scanty cytoplasm, which were immunoreactive for synaptophysin and chromogranin A, compatible with the diagnosis of small cell carcinoma. The small cell carcinoma of bile duct, despite its rarity, should be considered in differential diagnosis of the causes for obstructive jaundice and hemobilia.
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Affiliation(s)
- Sung Bum Cho
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
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10
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Okamura Y, Maeda A, Matsunaga K, Kanemoto H, Boku N, Furukawa H, Sasaki K, Uesaka K. Small-cell carcinoma in the common bile duct treated with multidisciplinary management. ACTA ACUST UNITED AC 2009; 16:575-8. [PMID: 19288048 DOI: 10.1007/s00534-009-0051-4] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2008] [Accepted: 03/24/2008] [Indexed: 12/18/2022]
Abstract
Small-cell carcinoma occasionally occurs in the gastrointestinal tract, with rare reports of it in the biliary system. When it does occur in the biliary system, it is mainly within the gallbladder, and those arising in the extrahepatic bile duct are extremely rare with only seven reported cases to date. A 62-year-old man with this unusual condition is documented. The patient was preoperatively diagnosed as having small-cell carcinoma on the basis of a biopsy specimen and was treated with multidisciplinary management, which consisted of preoperative chemotherapy, a curative resection, adjuvant chemotherapy, and radiation therapy. The patient died 23 months after the initial diagnosis (20 months after surgery). To the best of our knowledge, the survival time of this case was the longest among reported cases, and multidisciplinary management might contribute to a prolonged survival.
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Affiliation(s)
- Yukiyasu Okamura
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center Hospital, 1007 Shimo-Nagakubo, Sunto-Nagaizumi, Shizuoka, 411-8777, Japan.
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11
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Hosonuma K, Sato K, Honma M, Kashiwabara K, Takahashi H, Takagi H, Mori M. Small-cell carcinoma of the extrahepatic bile duct: a case report and review of the literature. Hepatol Int 2008; 2:129-132. [PMID: 19669289 PMCID: PMC2716877 DOI: 10.1007/s12072-007-9027-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/27/2007] [Revised: 06/19/2007] [Indexed: 01/27/2023]
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.
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Affiliation(s)
- Kenichi Hosonuma
- Department of Gastroenterology, Tomioka General Hospital, 2073-1 Tomioka, Tomioka, Gunma 370-2316 Japan
| | - Ken Sato
- Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, 3-39-15 Showa, Maebashi, Gunma 371-8511 Japan
| | - Manabu Honma
- Department of Pathology, Tomioka General Hospital, 2073-1 Tomioka, Tomioka, Gunma 370-2316 Japan
| | - Kenji Kashiwabara
- Department of Clinical Pathology, Gunma University Graduate School of Medicine, Maebashi, Gunma 371-8511 Japan
| | - Hitomi Takahashi
- Department of Gastroenterology, Tomioka General Hospital, 2073-1 Tomioka, Tomioka, Gunma 370-2316 Japan
| | - Hitoshi Takagi
- Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, 3-39-15 Showa, Maebashi, Gunma 371-8511 Japan
| | - Masatomo Mori
- Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, 3-39-15 Showa, Maebashi, Gunma 371-8511 Japan
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12
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Arakura N, Muraki T, Komatsu K, Ozaki Y, Hamano H, Tanaka E, Kawa S. Small cell carcinoma of the extrahepatic bile duct diagnosed with EUS-FNA and effectively treated with chemoradiation. Intern Med 2008; 47:621-5. [PMID: 18379148 DOI: 10.2169/internalmedicine.47.0663] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/29/2023] Open
Abstract
Small cell carcinoma of the bile duct system is extremely rare, and surgical procedures have been complicated by early hematogenous dissemination. In this study, we report a patient with small cell carcinoma of the bile duct system presenting with jaundice. The diagnosis was made early by endoscopic ultrasonography-guided fine needle aspiration biopsy (EUS-FNA). We performed radiation therapy of a total of 30 Gy, resulting in a marked decrease of serum neuron specific enolase levels and amelioration of jaundice, which had been resistant to drainage procedures. The patient was then treated with combined chemotherapy of cisplatin and CPT-11, which resulted in the disappearance of the tumor mass by image tests.
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Affiliation(s)
- Norikazu Arakura
- Department of Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan.
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13
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Abstract
Colonic composite tumors are uncommon masses composed of endo- and exocrine cells. Treatment is similar to that of adenocarcinomas. We report the case of a 44-year-old woman who consulted for abdominal pain. A computed tomography (CT) scan showed a tumor at the splenic flexure bowel and 2 hepatic nodules, suggesting metastases. Preoperative biopsies confirmed an adenocarcinoma. The patient underwent surgery, which confirmed the CT findings. Extended right colectomy was performed, followed by an ileal-sigmoid anastomosis, resection of a diaphragm segment, and resection of both hepatic metastases. The patient made a good postoperative recovery. Histological analysis showed moderately differentiated tubular adenocarcinoma combined with a poorly differentiated neuroendocrine carcinoma and metastases in 25 of 28 lymph nodes. The 2 hepatic metastases showed areas of poorly differentiated neuroendocrine carcinoma. The patient is currently asymptomatic and is undergoing chemotherapy.
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Affiliation(s)
- Jean M Butte
- Departamento de Cirugía Digestiva, División de Cirugía, Pontificia Universidad Católica de Chile, Marcoleta 367, Santiago, Chile
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Sato K, Waseda R, Tatsuzawa Y, Fujinaga H, Wakabayashi T, Ueda Y, Katsuda S. Composite large cell neuroendocrine carcinoma and adenocarcinoma of the common bile duct. J Clin Pathol 2006; 59:105-7. [PMID: 16394290 PMCID: PMC1860263 DOI: 10.1136/jcp.2005.028019] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Large cell neuroendocrine carcinoma (LCNEC) is a high grade type of neuroendocrine tumour with an aggressive clinical course. This report describes the first case of LCNEC combined with an adenocarcinoma component in the common bile duct. A 68 year old man presented with jaundice. Severe stenosis of the bile duct was revealed by endoscopic retrograde cholangiography, and adenocarcinoma cells were detected by brush cytology. Pancreaticoduodenectomy was performed, and the patient died of disease three months after surgery. A tumour measuring 2.0 cm in diameter was located in the intrapancreatic portion of the bile duct. Histologically, the tumour consisted of a LCNEC component and a well differentiated adenocarcinoma component. There were transitional areas between the two components. Immunohistochemically, LCNEC cells were reactive for neuroendocrine markers, but no specific hormonal expression was found. Chromogranin A positive cells were found in some areas of the adenocarcinoma component. These findings are consistent with the theory that both of the carcinoma components originated from a common pluripotent stem cell.
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Affiliation(s)
- K Sato
- Department of Pathophysiological and Experimental Pathology, Kanazawa Medical University, Uchinada, Kahoku, Ishikawa 920-0293, Japan.
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Patnaik AK, Lieberman PH, Erlandson RA, Antonescu C. Hepatobiliary neuroendocrine carcinoma in cats: a clinicopathologic, immunohistochemical, and ultrastructural study of 17 cases. Vet Pathol 2005; 42:331-7. [PMID: 15872379 DOI: 10.1354/vp.42-3-331] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
Hepatobiliary neuroendocrine carcinoma was diagnosed in 17 cats in a period of 10 years. Seven tumors were of intrahepatic origin, one of which was a composite containing components of epithelial and neuroendocrine carcinoma. Nine tumors were of extrahepatic origin, and one tumor was located in the gall-bladder. The cats were adult and geriatric, and the male : female ratio varied according to tumor group. Hepatomegaly, anorexia, weight loss, and vomiting were the most common clinical signs observed in the cats with hepatic neuroendocrine carcinoma. The cats with extrahepatic neuroendocrine carcinoma showed these signs plus icterus (5/9) and high concentrations of hepatic enzymes. Histologically, the hepatic neuroendocrine carcinomas had two patterns, one with acinar structures separated by vascular stroma lined by cuboidal or columnar cells and the other solid with groups of anaplastic cells separated by vascular stroma. The composite tumor consisted of both bile duct carcinoma and neuroendocrine carcinoma. The extrahepatic neuroendocrine carcinomas and the gallbladder neuroendocrine carcinoma were characterized by solid sheets or groups of round to oval cells with vascular or fibrovascular stroma. Immunohistochemical examination of 10 of the neuroendocrine carcinomas revealed that all 10 stained with neuron-specific enolase; one bile duct carcinoma and the gallbladder carcinoma stained with chromogranin; four of five bile duct carcinomas and the gall bladder carcinoma stained with synaptophysin; and one bile duct carcinoma stained with gastrin. One cat with hepatic carcinoma had duodenal ulcer; in this cat, ultrastructural studies showed neurosecretory granules leading to the diagnosis of Zollinger-Ellison syndrome. In four cats in which necropsy was permitted, carcinomatosis (4/4), lymph nodes (4/4), lungs (2/4), and intestines (1/4) were the metastatic sites. Fourteen of the 17 cats were euthanatized during or immediately after surgery.
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Affiliation(s)
- A K Patnaik
- Department of Veterinary Pathology, The Animal Medical Center, 510 East 62nd Street, New York, NY 10021, USA.
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Kuraoka K, Taniyama K, Fujitaka T, Nakatsuka H, Nakayama H, Yasui W. Small cell carcinoma of the extrahepatic bile duct: case report and immunohistochemical analysis. Pathol Int 2004; 53:887-91. [PMID: 14629756 DOI: 10.1046/j.1440-1827.2003.01575.x] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
A small cell carcinoma of the extrahepatic bile duct in a 75-year-old Japanese man is reported. The patient suffered from obstructive jaundice, and percutaneous transhepatic cholangiography-drainage (PTCD) revealed a massive lesion in the lower common bile duct. Because it was diagnosed as a malignant tumor, pancreaticoduodenectomy was performed. A nodular infiltrating tumor measuring 4.5 x 3.0 x 2.0 cm was located in the intrapancreatic portion of the extrahepatic bile duct. Histologically, the tumor was composed of a dense proliferation of small atypical cells with a little region of high-grade dysplasia in the adjacent epithelium of the common bile duct. Tumor cells were immunoreactive to neuroendocrine markers such as chromogranin A, synaptophysin, CD56, and Leu7. Although carcinoma cells invaded into pancreas and duodenum, there were no histological findings that indicated the carcinoma arose from the mucosa of either the pancreatic duct or duodenum. These results indicated that the tumor was a small cell carcinoma derived from the epithelium of the extrahepatic bile duct; a rare neoplasm with only a few cases reported. A few neuroendocrine cells were recognized in the adjacent epithelium of the extrahepatic bile duct, suggesting that the tumor cells might be derived from them. Using immunohistochemical examination, no p53 abnormality was found. Tumor cells showed positive nuclear staining for p16, while negative for cyclin D1, suggesting that functional retinoblastoma protein (pRB) might be lost in the p16/pRB pathway, as in small cell lung cancer.
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Affiliation(s)
- Kazuya Kuraoka
- Department of Molecular Pathology, Hiroshima University Graduate School of Biomedical Sciences, Hiroshima, Japan
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Yamamoto J, Abe Y, Nishihara K, Katsumoto F, Takeda S, Abe R, Toyoshima S. Composite glandular-neuroendocrine carcinoma of the hilar bile duct: report of a case. Surg Today 1998; 28:758-62. [PMID: 9697272 DOI: 10.1007/bf02484625] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
We report herein an unusual case of a composite glandular-neuroendocrine carcinoma of the hilar bile duct. A 71-year-old Japanese woman was admitted to our hospital suffering from general fatigue, progressive jaundice, and a high fever. Computed tomography and angiography findings revealed a solid hypervascular mass in the hepatic hilus. Thus, a subsegmentectomy of the liver (S4, S5) and bile duct resection with lymph node dissection were performed. A tumor measuring 6.0 x 3.0 cm was found to be located in the bile duct of the hepatic hilus. Histologically, the tumor was composed of well-differentiated adenocarcinoma and small cell neuroendocrine carcinoma cells, with a histological transition between the two components. Grimelius' method revealed the presence of diffuse positive tumor cells in neuroendocrine carcinoma. The neuroendocrine tumor cells were also diffusely immunoreactive to chromogranin A. To the best of our knowledge, only 22 previous cases of composite glandular-neuroendocrine carcinoma in the biliary tract have been reported; however, this is the first case report of a clearly composite tumor of the hilar bile duct.
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Affiliation(s)
- J Yamamoto
- Department of Surgery, Kitakyushu Municipal Medical Center, Japan
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