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The Significance of Adjuvant Therapy for Extrahepatic Cholangiocarcinoma After Surgery.

ABSTRACT: Extrahepatic cholangiocarcinoma (EHCC) is a rare malignant tumor, and current treatment methods are also relatively limited. Radical surgery is the only potentially curative method for the long survival time. However, despite undergoing radical resection, prognosis remained poor due to the high recurrence rate and distant metastasis. Therefore, adjuvant chemotherapy and radiotherapy should be offered to patients who have undergone surgery. Unfortunately, the low incidence of this disease has resulted in a lack of high-level evidence to confirm the importance of adjuvant chemotherapy or radiotherapy. At present, it is still controversial whether adjuvant therapy can prolong the survival of patients after operation, especially patients with negative margins or lymph nodes. Furthermore, standard regimens of adjuvant have not been identified. This review summarizes the currently available evidence of the effect of adjuvant therapy in the management of EHCC. Ultimately, we concluded that adjuvant therapy may improve survival in high-risk (positive margin or lymph node or advanced stage) patients and adjuvant concurrent chemoradiotherapy followed by chemotherapy may be the optimum selection for them. This needs to be verified by randomized prospective clinical trials.

SUBMITTER: Wang G

PROVIDER: S-EPMC6941596 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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The Significance of Adjuvant Therapy for Extrahepatic Cholangiocarcinoma After Surgery.

Wang Gaoyuan G Wang Qiang Q Fan Xia X Ding Lijuan L Dong Lihua L

Cancer management and research 20191230

Extrahepatic cholangiocarcinoma (EHCC) is a rare malignant tumor, and current treatment methods are also relatively limited. Radical surgery is the only potentially curative method for the long survival time. However, despite undergoing radical resection, prognosis remained poor due to the high recurrence rate and distant metastasis. Therefore, adjuvant chemotherapy and radiotherapy should be offered to patients who have undergone surgery. Unfortunately, the low incidence of this disease has res ...[more]

PMID: 31920396

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Project description:Presentation of the Case A 37-year-old woman presented at 35 weeks of gestation with her third child with failure to adequately gain weight and was noted by her obstetrician to have delay in the growth of her baby. Ultrasound of the abdomen incidentally revealed the presence of a liver lesion. After additional evaluation, she ultimately delivered her daughter at 36 weeks uneventfully. She subsequently underwent additional evaluation. Liver magnetic resonance imaging (MRI) revealed a 5-cm solitary solid mass in segment 4A of the liver, concerning for malignancy. Serum α-fetoprotein, carcinoembryonic antigen, cancer antigen (CA)19-9, CA15-3, and CA125 were all normal. Liver biopsy was positive for adenocarcinoma. The tumor cells demonstrated a phenotype suggesting a possible breast primary, although the immunohistochemistry did not support that diagnosis and the tumor was negative for mammaglobin, gross cystic disease fluid protein (GCDFP)-15, estrogen receptor (ER), and progesterone receptor (PR) (Table 1). The tumor was also CDX2 and cardiotrophin-1 negative, but cytokeratin (CK) 19 positive. Her endoscopic retrograde cholangiopancreatography, upper endoscopy, colonoscopy, breast mammogram, and breast MRI were completely normal. A positron emission tomography-computed tomography scan showed a fluorodeoxyglucose-avid 5.8-cm × 6.0-cm hypoattenuating lesion with peripheral enhancement involving segment 4 and segment 8 at the dome. In addition, central necrosis within the lesion was noted. The left main portal vein was mildly attenuated by the mass. She eventually underwent a left hepatectomy en bloc with caudate resection, portal lymphadenectomy, cholecystectomy, and omental pedicle flap. On exploration of the abdomen, no additional disease was noted. The final pathology revealed a 9.4-cm moderately to poorly differentiated adenocarcinoma of the intrahepatic bile ducts. Venous invasion was present. Perineural invasion was absent. The margins were negative. Thirteen lymph nodes were obtained, all of which were negative, consistent with a stage T2, N0, MX intrahepatic cholangiocarcinoma. The tumor was positive for CK7, CK19, and CA19-9 and negative for CK20, CDX2, CA125, ER, PR, GCDFP-15, synaptophysin, and chromogranin (Table 1). The uninvolved liver was unremarkable and a trichrome stain showed no fibrosis. Following an uneventful postoperative recovery, she was referred for consideration of adjuvant therapy.

Project description:Background & aimsCholangiocarcinoma (CCA), a deadly malignancy of the bile ducts, can be classified based on its anatomical location into either intrahepatic (iCCA) or extrahepatic (eCCA), each with different pathogenesis and clinical management. There is limited understanding of the molecular landscape of eCCA and no targeted therapy with clinical efficacy has been approved. We aimed to provide a molecular classification of eCCA and identify potential targets for molecular therapies.MethodsAn integrative genomic analysis of an international multicenter cohort of 189 eCCA cases was conducted. Genomic analysis included whole-genome expression, targeted DNA-sequencing and immunohistochemistry. Molecular findings were validated in an external set of 181 biliary tract tumors from the ICGC.ResultsKRAS (36.7%), TP53 (34.7%), ARID1A (14%) and SMAD4 (10.7%) were the most prevalent mutations, with ∼25% of tumors having a putative actionable genomic alteration according to OncoKB. Transcriptome-based unsupervised clustering helped us define 4 molecular classes of eCCA. Tumors classified within the Metabolic class (19%) showed a hepatocyte-like phenotype with activation of the transcription factor HNF4A and enrichment in gene signatures related to bile acid metabolism. The Proliferation class (23%), more common in patients with distal CCA, was characterized by enrichment of MYC targets, ERBB2 mutations/amplifications and activation of mTOR signaling. The Mesenchymal class (47%) was defined by signatures of epithelial-mesenchymal transition, aberrant TGFβ signaling and poor overall survival. Finally, tumors in the Immune class (11%) had a higher lymphocyte infiltration, overexpression of PD-1/PD-L1 and molecular features associated with a better response to immune checkpoint inhibitors.ConclusionAn integrative molecular characterization identified distinct subclasses of eCCA. Genomic traits of each class provide the rationale for exploring patient stratification and novel therapeutic approaches.Lay summaryTargeted therapies have not been approved for the treatment of extrahepatic cholangiocarcinoma. We performed a multi-platform molecular characterization of this tumor in a cohort of 189 patients. These analyses revealed 4 novel transcriptome-based molecular classes of extrahepatic cholangiocarcinoma and identified ∼25% of tumors with actionable genomic alterations, which has potential prognostic and therapeutic implications.

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The Significance of Adjuvant Therapy for Extrahepatic Cholangiocarcinoma After Surgery.

Publications

The Significance of Adjuvant Therapy for Extrahepatic Cholangiocarcinoma After Surgery.

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