In primary sclerosing cholangitis, gallbladder polyps are frequently malignant.
ABSTRACT The management of gallbladder polyps/masses in patients with primary sclerosing cholangitis (PSC) (i.e., cholecystectomy vs observation) remains problematic. Given the risk of biliary tract cancer in PSC in the face of the benign nature of most gallbladder polyps in the general population, our aim was to determine the prevalence of gallbladder cancer in PSC patients with a gallbladder mass who had undergone cholecystectomy.
The case records of all patients with PSC undergoing a cholecystectomy at the Mayo Clinic between 1977-1999 were reviewed.
Of the 102 patients with PSC who underwent a cholecystectomy, 14 of 102 (13.7%) had a gallbladder mass. In the subset of patients with gallbladder masses, eight of 14 (57%) had adenocarcinomas (seven primary adenocarcinomas and one metastatic cholangiocarcinoma); the other six had benign masses (five adenomas and one cholesterol polyp). In those patients with benign masses, 33% had associated epithelial cell dysplasia; in patients with primary gallbladder cancers, 57% had associated dysplasia. The patients with primary gallbladder adenocarcinoma had a favorable outcome after cholecystectomy, with a 36-month survival of 66%.
In conclusion, gallbladder neoplasms in PSC patients are malignant in approximately 40-60% of the cases. The presence of gallbladder epithelial cell dysplasia suggests a dysplasia-carcinoma sequence in PSC similar to that observed in ulcerative colitis. Consideration should be given to performing a cholecystectomy in PSC patients with gallbladder polyps. If a cholecystectomy is not performed, careful interval follow-up is warranted.
Article: Primary sclerosing cholangitis.[Show abstract] [Hide abstract]
ABSTRACT: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that involves progressive destruction of the bile ducts. Its prevalence is 4 to 16 cases per 100,000 persons. Its incidence has risen over the last 20 years, with a more than 35% increase in the last 10 years alone. PSC tends to arise in patients with chronic inflammatory bowel diseases. It is associated with an increased risk of various types of cancer (13%-14%), most prominently cholangiocellular carcinoma (CCC). This review is based on a selective search in PubMed for original articles, meta-analyses, and review articles about PSC that appeared from January 1980 to May 2013. The diagnosis is generally established with a bile duct imaging study-typically, magnetic resonance cholangiopancreaticography (MRCP): this test is more than 80% sensitive and more than 90% specific for the diagnosis of PSC. The time from diagnosis to death or liver transplantation is 12 to 18 years, and the risk that a patient with PSC will die of cancer is 40% to 58%. Options for drug treatment are limited. Randomized, controlled trials have not shown any improvement of outcomes from the administration of ursodeoxycholic acid (UDCA). Interventional endoscopy is used to treat dominant stenoses and cholangitis, even though this method of treatment is supported only by low-level evidence. Liver transplantation results in a 10-year survival rate above 80%. There is no causally directed treatment for PSC. Early diagnosis, complication management, and the evaluation of an optimally timed liver transplantation are the main determinants of outcome.Deutsches Ärzteblatt International 12/2013; 110(51-52):867-74. · 3.61 Impact Factor
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ABSTRACT: Background: Gallbladder cancer (GBC) is a rare disease of the hepatobiliary tract characterized by silent presentation, poor prognosis, and limited therapy. Current imaging modalities, clinical symptoms and laboratory values are of limited value in diagnosis and tumour markers are used as a clinical adjunct. Five year survival is 5% -12% and a majority of patients survive less than 1 year. Early identification, negative nodal status, and extended cholecystectomy improve survival; adjuvant therapy does not appear to play a role. Objective: To evaluate the effects of stage and nodal status of GBC on survival by analyzing clinical and radiological factors leading to preoperative diagnosis and appropriate sub-sequent management. Results: Forty-three patients (31 female, 12 male) had primary GBC. Average age was 69 years. The most common presenting symptom was pain (65%). Fifteen (35%) patients presented with acute cholecystitis; 9 were suspicious for a mass. Ultrasound and computed tomography were the imaging modalities most often used. Pre-operative diagnoses were made in only 7 (16%) patients, whereas 16 (37%) patients were diagnosed intra-operatively and 20 (47%) post-operatively. Adjuvant chemotherapy was offered in 16 (37%) patients. Average survival was 28 months (range 0.5 -238 months), with a 5-year survival of 13% across all stages. Early GBC (stages 1 -2) had a 5-year survival of 43%, and late GBC (stages 3 -4) had no survival at 5 years, with an average survival of 9 months. Positive nodes decreased survival. Conclusion: Our results mirror what has been reported in the literature. Most patients pre-sented with acute cholecystitis, with confounding clinical data, but few were preoperatively diagnosed with GBC. Im-aging was rarely diagnostic. Late stage GBC, as well as node-positive status, had dismal outcomes with low 1-year and 5-year survival rates. Improved preoperative suspicion of early GBC would allow for more intentional curative resec-tions, before nodes become positive.
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ABSTRACT: Extraintestinale Manifestationen von Morbus Crohn und Colitis ulcerosa finden sich bei mehr als der Hälfte aller Patienten und können belastender als die Grunderkrankung selbst sein. Extraintestinale Manifestationen müssen von Sekundärerkrankungen oder Folgen der chronisch-entzündlichen Darmerkrankungen (CED) unterschieden werden, da sie einer anderen Therapie bedürfen. Während Krankheitsfolgen wie Vitaminmangelzustände oder Osteoporose durch Vitaminsubstitution oder Calcium- und Vitamin-D-Gabe spezifisch therapierbar sind, ist die Therapie extraintestinaler Manifestationen von CED wie der primär sklerosierenden Cholangitis, der sog. Typ-II-Arthritis oder granulomatöser Entzündungen der Haut, Leber oder Lunge häufig deutlich schwieriger und komplexer. Extraintestinale Symptome des Morbus Crohn und der Colitis ulcerosa können sich an nahezu jedem Organ manifestieren. Es ist wichtig, diese extraintestinalen Manifestationen der CED zu kennen, um einerseits rechtzeitig und frühzeitig Therapiemaßnahmen einleiten zu können. Andererseits können extraintestinale Symptome die Primärmanifestation eines Morbus Crohn und seltener einer Colitis ulcerosa darstellen. Sie müssen dann als solche erkannt werden, um die Betroffenen frühzeitig adäquat therapieren zu können.Medizinische Klinik 01/2004; 99(3). · 0.27 Impact Factor