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1.
Br J Surg ; 106(11): 1504-1511, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31386198

RESUMO

BACKGROUND: Differentiation between perihilar cholangiocarcinoma (PHCC) and benign strictures is frequently difficult. The aim of this study was to investigate the incidence and long-term outcome of patients with tumours resected because of suspicion of PHCC, which ultimately turned out to be benign (malignancy masquerade). METHODS: Patients who underwent surgical resection with a diagnosis of PHCC between 2001 and 2016 were reviewed retrospectively. RESULTS: Among 707 consecutive patients, 685 had PHCC and the remaining 22 (3·1 per cent) had benign biliary stricture. All patients with benign disease underwent major hepatectomy, with no deaths. Preoperative histological assessment using bile duct biopsy or aspiration cytology had a high specificity (90 per cent), low sensitivity (62 per cent) and unsatisfactory accuracy (63 per cent). Despite the increasing use of histological assessment, the incidence of benign strictures resected did not decrease over time, being 0·9 per cent in 2001-2004, 4·0 per cent in 2005-2008, 3·8 per cent in 2009-2012 and 2·9 per cent in 2013-2016. The final pathology of benign strictures included IgG4-related sclerosing cholangitis (9 patients), hepatolithiasis (4), granulomatous cholangitis (3), non-specific chronic cholangitis (3), benign strictures after cholecystectomy (2), and a benign stricture possibly caused by parasitic infection (1). The 10-year overall survival rate for the 22 patients with benign stricture was 87 per cent, without recurrence of biliary stricture. CONCLUSION: The incidence of benign strictures resected as PHCC as a proportion of all resections was relatively low, at 3·1 per cent. Currently, unnecessary surgery for suspected PHCC is unavoidable.


Assuntos
Doenças dos Ductos Biliares/diagnóstico , Tumor de Klatskin/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças dos Ductos Biliares/cirurgia , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/cirurgia , Constrição Patológica/diagnóstico , Constrição Patológica/cirurgia , Humanos , Tumor de Klatskin/cirurgia , Pessoa de Meia-Idade , Cuidados Pré-Operatórios/métodos , Estudos Retrospectivos , Resultado do Tratamento
2.
J Hepatobiliary Pancreat Sci ; 26(8): 341-347, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31155841

RESUMO

BACKGROUND: Although there are many studies on technical outcomes of endoscopic nasobiliary drainage (ENBD), no authors reported on preoperative course of patients undergoing ENBD. The aim of this study was to investigate the course of patients with ENBD during the waiting period. METHODS: Patients who underwent resection of perihilar cholangiocarcinoma (PHCC) between January 2013 and September 2017 were retrospectively reviewed. RESULTS: During the study period, 191 consecutive patients underwent surgical resection of PHCC after ENBD. Of the study patients, 154 (80.6%) patients were discharged, returned to their home, then re-admitted for surgery. The remaining 37 patients were continuously hospitalized. The number of cholangitis events during the waiting period was 0 in 120 patients, 1 in 59 patients, 2 ≤ in 12 patients. Endoscopic re-intervention was needed in 52 patients. The median length between the first admission and surgery was 37 days (range 12-197 days) in the entire cohort; it was longer in patients with portal vein embolization than in those without (43 vs. 27 days, P < 0.001). CONCLUSIONS: In patients undergoing resection of PHCC, ENBD is widely tolerable with relatively low incidence of cholangitis and thus recommended for preoperative biliary drainage.


Assuntos
Neoplasias dos Ductos Biliares/cirurgia , Drenagem/métodos , Hepatectomia/métodos , Tumor de Klatskin/cirurgia , Cuidados Pré-Operatórios/métodos , Listas de Espera/mortalidade , Adulto , Idoso , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/mortalidade , Estudos de Coortes , Bases de Dados Factuais , Intervalo Livre de Doença , Endoscopia/métodos , Feminino , Hepatectomia/mortalidade , Mortalidade Hospitalar , Humanos , Japão , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/mortalidade , Masculino , Pessoa de Meia-Idade , Cavidade Nasal , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento
3.
Zhonghua Wai Ke Za Zhi ; 57(1): 6-9, 2019 Jan 01.
Artigo em Chinês | MEDLINE | ID: mdl-30612386

RESUMO

Hilar cholangiocarcinoma is one of the most difficult malignant tumors to treat in the biliary system. In Japan, 5-year survival rate of the disease has increased from 32.5% to 67.1% during the past 30 years. The impressive progress reflects the solid efforts in preoperative endoscopic diagnosis, innovation in surgery such as PTPE as well as hepato-pancreatoduodenectomy and perioperative treatment including replacement of the bile and synbiotic treatment, which have finally formed a set of standardized diagnosis and treatment systems. The present review intends to report the history, current status and remaining bottlenecks of the diagnosis and treatment system of hilar cholangiocarcinoma in Japan as follows.


Assuntos
Neoplasias dos Ductos Biliares , Tumor de Klatskin , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/terapia , Ductos Biliares Intra-Hepáticos , Hepatectomia , Humanos , Japão , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/terapia , Resultado do Tratamento
5.
Gastroenterol Hepatol ; 42(4): 271-279, 2019 Apr.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30583874

RESUMO

Cholangiocarcinomas are heterogeneous biliary tract tumors that cause devastating disease. Perihilar cholangiocarcinoma (PHC) is the most common type of biliary tract cancer and are associated with a high mortality. Diagnoses of PHC depend on the results of its clinical presentation, serum biomarkers and imaging techniques. Pre-operative managements including pre-operative biliary drainage (PBD) and portal vein embolization (PVE) could reduce mortality. The best chance of long-term survival and potential cure is surgical resection with negative surgical margin. Lymph node metastasis over N2 nodes precludes long-term survival. The benefit of concomitant vascular resection remains uncertain. Liver transplantation combined with neoadjuvant chemotherapy with radiotherapy is a promising option in highly selected patients with unresectable tumors. Herein, an overview is provided of developments in diagnosis, peri-operative management and surgical treatment among patients with PHCs.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/cirurgia , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/cirurgia , Procedimentos Cirúrgicos do Sistema Biliar/métodos , Humanos , Cuidados Pré-Operatórios
6.
Anticancer Res ; 38(12): 6737-6744, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30504384

RESUMO

BACKGROUND/AIM: Platforms using valid molecular targets can provide concurrent diagnostic and treatment (theragnostic) options in perihilar cholangiocarcinoma (PHC). Neutrophil gelatinase-associated lipocalin (NGAL) is a biomarker in the biliary secretome of PHC. Its potential as a theragnostic target and its prognostic significance in this cancer was, therefore, explored. MATERIALS AND METHODS: In-vitro studies were used to determine NGAL localization in several cholangiocarcinoma cell lines. Tissue expression of NGAL was quantified in PHC resection cases from 2000-2010 by immunohistochemistry. RESULTS: NGAL was expressed in the majority of tested cell lines and localized to their membranes. Tissues from 54 patients underwent NGAL immunohistochemistry. Median tumoral NGAL expression was significantly higher than that in matched liver controls (p<0.001). Higher NGAL tumor expression was associated with nodal metastasis (p=0.021), although no significant association with survival was observed. CONCLUSION: The expression and localization of NGAL in PHC make it a valid candidate biomarker for exploitation in theragnostic platforms.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Biomarcadores Tumorais , Tumor de Klatskin/diagnóstico , Lipocalina-2/fisiologia , Recidiva Local de Neoplasia/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias dos Ductos Biliares/metabolismo , Neoplasias dos Ductos Biliares/patologia , Ductos Biliares Intra-Hepáticos/metabolismo , Ductos Biliares Intra-Hepáticos/patologia , Biomarcadores Tumorais/metabolismo , Linhagem Celular Tumoral , Feminino , Humanos , Imuno-Histoquímica , Tumor de Klatskin/metabolismo , Tumor de Klatskin/patologia , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Recidiva Local de Neoplasia/metabolismo , Recidiva Local de Neoplasia/patologia , Prognóstico
7.
Medicine (Baltimore) ; 97(41): e12830, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30313119

RESUMO

RATIONALE: Primary mucosa-associated lymphoid tissue (MALT) lymphomas rarely originate in the hilar bile duct. Preoperative diagnosis of a primary MALT lymphoma of the hilar bile duct is difficult owing to the rarity of this disease. Differentiating between obstructive jaundice caused by MALT lymphoma of the hilar bile duct and hilar cholangiocarcinoma (the most common form of bile duct cancer) is challenging. PATIENT CONCERNS: A 57-year-old man presented to our hospital in August 2012 with fluctuant obstructive jaundice. DIAGNOSES: Contrast-enhanced abdominal computed tomography and magnetic resonance cholangiopancreatography showed a hilar liver mass measuring 23 × 28 mm along with intrahepatic biliary dilatation indicating hilar bile duct obstruction with a high index of suspicion for hilar cholangiocarcinoma. INTERVENTIONS AND OUTCOMES: Based on frozen section examination, he was intraoperatively diagnosed with chronic nonspecific inflammation. Histopathological and immunohistochemical examinations confirmed a diagnosis of malignant lymphoma, specifically classified as an extranodal marginal zone B-cell lymphoma of MALT type. LESSONS: A primary MALT lymphoma of the bile duct should be considered among the differential diagnosis in patients with a hilar tumor who present with fluctuating jaundice and are preoperatively diagnosed with suspected hilar cholangiocarcinoma, and/or an intraoperative diagnosis of chronic nonspecific inflammation (based on frozen section examination) assessed for stenosis or obstruction of the bile duct.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Neoplasias dos Ductos Biliares/patologia , Diagnóstico Diferencial , Humanos , Tumor de Klatskin/diagnóstico , Linfoma de Zona Marginal Tipo Células B/diagnóstico por imagem , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Pessoa de Meia-Idade
8.
Presse Med ; 47(11-12 Pt 1): 950-960, 2018.
Artigo em Francês | MEDLINE | ID: mdl-30224216

RESUMO

Magnetic resonance cholangiopancreatography is a non-invasive imaging, highly performant in detecting and locating biliary stenosis and in predicting its malignancy. The combination of two and three-dimensional MRI sequences is recommended to assess a biliary obstacle. Cholangiopathies are the main differential diagnosis of cholangiocarcinomas. In addition to bile duct abnormalities beyond the stenosis, the predictive signs of malignancy are: asymmetric irregular luminal narrowing (longer than 3mm and thicker than 13mm), abrupt discontinuation of stenosis, and enhancement superior to that of the adjacent hepatic parenchyma during arterial and portal acquisitions. Diffusion sequences improve the sensitivity of biliary stenosis detection. Moreover, restriction of diffusion is useful for distinguishing malignant from benign stenosis. Combining Magnetic resonance cholangiopancreatography, late LAVA and diffusion sequences improves the sensitivity of detection of a possible biliary extension without significant specificity. MRI with gadolinium chelate injection is efficient in assessing vascular extension of cholangiocarcinomas. However its accuracy remains lower than that of Ct-scan. To assess hepatic, lymphatic and peritoneal extensions of cholangiocarcinomas, it is fundamental to combine diffusion and dynamic MRI sequences.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/cirurgia , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/cirurgia , Imagem por Ressonância Magnética , Diagnóstico Diferencial , Humanos , Período Pré-Operatório
9.
Cell Physiol Biochem ; 49(5): 1694-1702, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30231247

RESUMO

BACKGROUND & AIMS: Current studies have indicated that long non-coding RNAs (lncRNAs) could act as tumor biomarkers for disease diagnosis and prognosis prediction. In this study, we mainly focused on determining the expression of circulating lncRNAs in patients suffering for hilar cholangiocarcinoma (HC), aiming to reveal the potential lncRNA as a fingerprint. METHODS: A total 12 lncRNAs were previously proven to be aberrantly expressed in HC tumor tissues. All of the 12 lncRNAs were selected as candidate targets for subsequent circulating lncRNA assay. The candidate lncRNAs were validated by qRT-PCR arranged in training and validation sets. The risk score analysis was employed. Data was presented with receiver operating characteristic curve (ROC). RESULTS: Circulating PCAT1, MALAT1, and CPS1-IT1 were significantly increased in plasma samples of HC patients in both the training set and validation set. Through ROC analysis, we found that the three plasmatic lncRNAs presented the area under ROC curve value (AUC) as 0.784, 0.860, and 0.677. Further combination with the three factors indicated a higher power (AUC, 0.893; sensitivity, 85.5%; specificity, 93.2%). CONCLUSION: This was the first time to reveal the potential circulating fingerprints for predicting HC. PCAT1, MALAT1, and CPS1-IT1 may act as novel early diagnosis biomarkers for predicting HC.


Assuntos
Biomarcadores Tumorais/sangue , Tumor de Klatskin/diagnóstico , RNA Longo não Codificante/sangue , Área Sob a Curva , Biomarcadores Tumorais/genética , Estudos de Casos e Controles , Feminino , Humanos , Tumor de Klatskin/genética , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Risco
10.
Can J Gastroenterol Hepatol ; 2018: 6962090, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30159303

RESUMO

Background: Mirizzi syndrome is a condition difficult to diagnose and treat, representing a particular "challenge" for the biliary surgeon. The disease can mimic cancer of the gallbladder, causing considerable diagnostic difficulties. Furthermore, it increases the risk of intraoperative biliary injury during cholecystectomy. The aim of this study is to point out some particular aspects of diagnosis and treatment of this condition. Methods: The clinical records of patients with Mirizzi syndrome, treated in the last five years, were reviewed. Clinical data, cholangiograms, preoperative diagnosis, operative procedures, and early and late results were examined. Results: Eighteen consecutive patients were treated in the last five years. Presenting symptoms were jaundice, pain, and cholangitis. Preoperative diagnosis of Mirizzi syndrome was achieved in 11 patients, while 6 had a diagnosis of gallbladder cancer and 1 of Klatskin tumor. Seventeen patients underwent surgery, including cholecystectomy in 8 cases, bile duct repair over T-tube in 3 cases, and hepaticojejunostomy in 4 cases. Two cases (11.1%) of gallbladder cancer associated with the Mirizzi syndrome were incidentally found: a patient underwent right hepatectomy and another patient was unresectable. The overall morbidity rate was 16.6%. There was no postoperative mortality. An ERCP with stent insertion was required in three cases after surgery. Sixteen patients were asymptomatic at a mean distance of 24 months (range: 6-48) after surgery. Conclusions: Mirizzi syndrome requires being treated by an experienced biliary surgeon after a careful assessment of the local situation and anatomy. The preoperative placement of a stent via ERCP can simplify the surgical procedure.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias da Vesícula Biliar/diagnóstico , Tumor de Klatskin/diagnóstico , Síndrome de Mirizzi/diagnóstico por imagem , Síndrome de Mirizzi/cirurgia , Dor Abdominal/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Colangite/etiologia , Diagnóstico Diferencial , Erros de Diagnóstico , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Feminino , Humanos , Icterícia Obstrutiva/etiologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Síndrome de Mirizzi/complicações , Complicações Pós-Operatórias , Tomografia Computadorizada por Raios X , Ultrassonografia
11.
HPB (Oxford) ; 20(11): 1092-1097, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30057125

RESUMO

BACKGROUND: Irreversible electroporation (IRE) has successfully been used for palliation of pancreatic and liver cancers due to its ability to ablate tumors without destroying nearby vital structures. To date, it has not been evaluated in patients with advanced hilar cholangiocarcinoma (AHC). This study presents a single-institution experience with IRE for management of obstructive jaundice in AHC. METHODS: A single-institution database was queried for patients undergoing IRE for AHC after PTBD placement for relief of obstructive jaundice from 2010 to 2017 and compared to a control group treated with standard of care only (No IRE). RESULTS: Twenty-six patients underwent IRE for AHC after PTBD replacement. Three patients experienced complications, with two experiencing severe (≥ grade 3) complications. After IRE, median time to PTBD removal was 122 days (range 0-305 days) and median catheter-free time before requiring PTBD replacement was 305 days (range 92-458 days). In comparison, the 137 control patients had an admission rate of 59% (N = 80 patients) for PTBD infection, occlusion, or catheter related problem. CONCLUSION: IRE safely achieves biliary decompression via tumor electroporation and allows PTBD removal for an extended period of time. In appropriately selected patients with obstructive jaundice in the setting of AHC, IRE can be used to increase catheter-free days and optimize overall quality of life.


Assuntos
Técnicas de Ablação , Neoplasias dos Ductos Biliares/complicações , Eletroporação , Icterícia Obstrutiva/cirurgia , Tumor de Klatskin/complicações , Cuidados Paliativos/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias dos Ductos Biliares/diagnóstico , Bases de Dados Factuais , Feminino , Humanos , Icterícia Obstrutiva/diagnóstico , Icterícia Obstrutiva/etiologia , Tumor de Klatskin/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento
12.
Semin Liver Dis ; 38(2): 160-169, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29871021

RESUMO

The diagnosis of malignant biliary strictures remains problematic, especially in the perihilar region and in primary sclerosing cholangitis (PSC). Conventional cytology obtained during endoscopic retrograde cholangiography (ERC)-guided brushings of biliary strictures is suboptimal due to limited sensitivity, albeit it remains the gold standard with a high specificity. Emerging technologies are being developed and validated to address this pressing unmet patient need. Such technologies include enhanced visualization of the biliary tree by cholangioscopy, intraductal ultrasound, and confocal laser endomicroscopy. Conventional cytology can be aided by employing complementary and advanced cytologic techniques such as fluorescent in situ hybridization (FISH), and this technique should be widely adapted. Interrogation of bile and serum by examining extracellular vesicle number and cargo, and exploiting next-generation sequencing and proteomic technologies, is also being explored. Examination of circulating cell-free deoxyribonucleic acid (cfDNA) for differentially methylated regions is a promising test which is being rigorously validated. The special expertise required for these analyses has to date hampered their validation and adaptation. Herein, we will review these emerging technologies to inform the reader of the progress made and encourage further studies, as well as adaptation of validated approaches.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Biomarcadores Tumorais/genética , DNA Tumoral Circulante/genética , Diagnóstico por Imagem/tendências , Detecção Precoce de Câncer/tendências , Endoscopia do Sistema Digestório/tendências , Tumor de Klatskin/diagnóstico , Técnicas de Diagnóstico Molecular/tendências , Neoplasias dos Ductos Biliares/genética , Neoplasias dos Ductos Biliares/patologia , Difusão de Inovações , Humanos , Tumor de Klatskin/genética , Tumor de Klatskin/patologia , Valor Preditivo dos Testes , Prognóstico
13.
Surgery ; 164(2): 244-250, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29801730

RESUMO

BACKGROUND: The performances of the American Joint Committee on Cancer staging systems of the 7th and 8th edition were compared using a cohort of patients undergoing surgery for perihilar cholangiocarcinoma at 2 tertiary referral Italian hepatobiliary centers. METHODS: The American Joint Committee on Cancer 7th and 8th edition staging systems were used to classify 214 patients who underwent surgery for perihilar cholangiocarcinoma. The performances of the 2 staging systems were compared using the concordance index. RESULTS: Using the American Joint Committee on Cancer 7th edition staging system, we found that the 5-year overall survival for stages I, II, and IVa was 71%, 34%, and 34%, while no patients in stages IIIa, IIIb, and IVb survived 5 years. In comparison, when the American Joint Committee on Cancer 8th edition staging system was used, the 5-year overall survival was 71% and 35% in stages I and II, resulting in 23%, 19%, and 22% in stages IIIa, IIIb, and IIIc, respectively. Of note, no patients in stages IVa and IVb survived 5 years. The American Joint Committee on Cancer 8th edition staging system had a slightly better discriminatory ability with a concordance index of 0.624 compared with 0.619 for the American Joint Committee on Cancer 7th edition. CONCLUSION: The newly released classification American Joint Committee on Cancer 8th edition staging system demonstrated a poor to moderate ability to predict prognosis of patients undergoing liver resection for perihilar cholangiocarcinoma, which was only slightly better than the previous edition. Further refinements are needed to improve the prognostic ability of the American Joint Committee on Cancer staging system for perihilar cholangiocarcinoma.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Tumor de Klatskin/diagnóstico , Idoso , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/cirurgia , Feminino , Humanos , Itália/epidemiologia , Tumor de Klatskin/mortalidade , Tumor de Klatskin/cirurgia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos
15.
Am J Gastroenterol ; 113(5): 765-772, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29549357

RESUMO

BACKGROUND: Distinguishing perihilar cholangiocarcinoma (PHC) from benign forms of sclerosing cholangitis affecting the hilar bile ducts is challenging, since histological confirmation of PHC is difficult to obtain and accurate non-invasive diagnostic tests are not available. IgG4-associated cholangitis (IAC), an imitator of PHC, may present with clinical and radiographical signs of PHC. IAC can be accurately diagnosed with a novel qPCR test. The aim of this study was to investigate the incidence and long-term activity of IAC in patients resected for PHC in a single tertiary center over a period of 30 years. METHODS: All patients with benign disease who underwent surgery for presumed PHC in our institute between 1984 and 2015 were identified. Benign liver and bile duct specimens were re-evaluated by a pathologist and scored according to international consensus pathology criteria for IgG4-related disease (IgG4-RD). Patients with benign disease still alive were followed-up and a clinical diagnosis of IAC was made using a combination of the HISORt group C (response to steroids) criteria and elevated serum IgG4 levels and/or the novel IgG4/IgG RNA ratio. Also, recurrent symptomatic disease at any time after surgery requiring immunosuppression was assessed. RESULTS: Out of 323 patients who underwent surgery for presumed PHC, 50 patients (15%) had benign disease. In 42% (n = 21/50) of these patients a histological (n = 17) or clinical (n = 4) diagnosis of IAC was established. The remaining patients were diagnosed with unclassified sclerosing inflammation, cystadenoma, or sclerosing hemangioma. Nine out of 12 IAC patients who were followed-up showed episodes of recurrent disease requiring immunosuppressive treatment. CONCLUSIONS: Liver and bile duct resections for PHC during three decades disclosed in 15% benign biliary disorders mimicking PHC of which 42% were definitely diagnosed as IAC. IgG4-RD remains active in the majority of patients with IAC years after surgery. Novel diagnostic tests for IAC might reduce misdiagnosis, unnecessary surgery, and life-threatening complications.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Colangite Esclerosante/diagnóstico , Tumor de Klatskin/diagnóstico , Atenção Terciária à Saúde/estatística & dados numéricos , Adulto , Idoso , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares/patologia , Ductos Biliares/cirurgia , Colangite Esclerosante/epidemiologia , Colangite Esclerosante/imunologia , Colangite Esclerosante/terapia , Diagnóstico Diferencial , Erros de Diagnóstico/estatística & dados numéricos , Feminino , Seguimentos , Humanos , Imunoglobulina G/imunologia , Imunossupressores/uso terapêutico , Incidência , Tumor de Klatskin/mortalidade , Tumor de Klatskin/patologia , Tumor de Klatskin/cirurgia , Fígado/patologia , Fígado/cirurgia , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Recidiva , Estudos Retrospectivos , Taxa de Sobrevida
17.
Am J Case Rep ; 19: 76-81, 2018 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-29353872

RESUMO

BACKGROUND A variety of benign etiologies of biliary stricture may initially be mistaken for hilar cholangiocarcinoma. Consequently, many patients undergo surgery for a benign disease that could have been treated medically. Eosinophilic cholangitis (EC) is an uncommon, benign, self-limiting disease that should be considered when approaching a case of obstructive jaundice since it causes biliary stricture formation. Transmural eosinophilic infiltration of the biliary tree is characteristic of EC. It may initially be indistinguishable from hilar cholangiocarcinoma. CASE REPORT We present a rare case of an 84-year-old male who was referred to our hospital for abdominal mass investigation with the provisional diagnosis of cholangiocarcinoma. During the workup, the index of suspicion for malignancy remained high as the typical laboratory and radiological findings for benign causes of biliary stricture were not present. Hence, the patient underwent left hepatectomy with caudate lobe resection and received a retrograde diagnosis of EC. CONCLUSIONS This case demonstrates that EC could present in the elderly with cardinal signs of cancer and absence of the typical findings of EC which was not previously reported. Since only 70% of patients present with peripheral eosinophilia, we stress on the importance of implementing diagnostic criteria for EC in the setting where peripheral eosinophilia is absent. Furthermore, this disorder has been reported to respond well to steroid therapy, hence, diagnostic criteria for EC would provide another treatment option for elderly and/or those who are not fit for surgery.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Colangite/diagnóstico , Eosinofilia/diagnóstico , Tumor de Klatskin/diagnóstico , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Hepatectomia , Humanos , Masculino , Doenças Raras
18.
Ann Surg ; 267(5): 797-805, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29064885

RESUMO

OBJECTIVE: To investigate the influence of type of surgery (transplant vs resection) on overall survival (OS) in patients with hilar cholangiocarcinoma (H-CCA). BACKGROUND: Outcomes after resection for H-CCA are poor, yet transplantation is currently only reserved for well-selected patients with unresectable disease. METHODS: All patients with H-CCA who underwent resection from 2000 to 2015 at 10 institutions were included. Three institutions additionally had active H-CCA transplant protocols with similar selection criteria over similar time periods. RESULTS: Of 304 patients with suspected H-CCA, 234 underwent attempted resection and 70 were enrolled in a transplant protocol. Excluding incomplete/R2 resections (n = 43), patients who were enrolled, but did not undergo transplant (n = 24), and transplants without confirmed H-CCA diagnoses (n = 5), 191 patients underwent curative-intent resection and 41 curative-intent transplant. Compared with resection, transplant patients were younger (52 vs 65 years; P < 0.001), and more frequently had primary sclerosing cholangitis (PSC; 61% vs 2%; P < 0.001) and received chemotherapy and/or radiation (98% vs 57%; P < 0.001). Groups were otherwise similar in demographics and comorbidities. Patients who underwent transplant for confirmed H-CCA diagnosis had improved OS compared with resection (3-year: 72% vs 33%; 5-year: 64% vs 18%; P < 0.001). Among patients who underwent resection for tumors <3 cm with lymph-node negative disease, and excluding PSC patients, transplant was still associated with improved OS (3-year: 54% vs 44%; 5-year: 54% vs 29%; P = 0.03). Transplant remained associated with improved survival on intention-to-treat analysis, even after accounting for tumor size, lymph node status, and PSC (P = 0.049). CONCLUSIONS: Resection for hilar cholangiocarcinoma that meets criteria for transplantation (<3 cm, lymph-node negative disease) is associated with substantially decreased survival compared to transplant for the same criteria with unresectable disease. Prospective trials are needed and justified.


Assuntos
Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos , Hepatectomia/métodos , Tumor de Klatskin/cirurgia , Transplante de Fígado/métodos , Adulto , Idoso , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/mortalidade , Feminino , Seguimentos , Humanos , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/mortalidade , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia
20.
Chirurgia (Bucur) ; 112(3): 193-207, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28675356

RESUMO

Perihilar cholangiocarcinoma is the most common type of biliary tract cancer and is associated with a high mortality, usually due to late presentation. High-resolution cross-sectional imaging modalities are necessary for diagnosis and preoperative planning. Although surgical resection with negative margins offers the only hope for cure, only a small subset of patients are amenable for surgery at the time of diagnosis. Portal vein embolization and biliary tract decompression are important in some patients prior to surgical resection. Liver transplantation in combination with neoadjuvant therapy has resulted in excellent 5-year recurrence-free survival rates in highly selected patients with inoperable disease. Gemcitabine plus cisplatin constitute the backbone of chemotherapy in patients with inoperable metastatic perihilar cholangiocarcinoma. Recent advances in understanding the molecular pathogenesis of CCA have created a growing interest in identifying novel therapies targeting key molecular pathways. Herein, we provide an overview of the most current principles of management of patients with perihilar cholangiocarcinoma.


Assuntos
Neoplasias dos Ductos Biliares/terapia , Tumor de Klatskin/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/cirurgia , Cisplatino/administração & dosagem , Desoxicitidina/administração & dosagem , Desoxicitidina/análogos & derivados , Drenagem/métodos , Drenagem/tendências , Embolização Terapêutica/métodos , Embolização Terapêutica/tendências , Humanos , Tumor de Klatskin/diagnóstico , Tumor de Klatskin/mortalidade , Tumor de Klatskin/cirurgia , Assistência Perioperatória/métodos , Assistência Perioperatória/tendências , Veia Porta/cirurgia , Taxa de Sobrevida , Resultado do Tratamento
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