S3-Guideline “Exocrine Pancreatic Carcinoma” 20071 ... - DGVS

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456 Leitlinie Comments Neither the supplementation with antioxidants [74, 75] nor the intake of non-steroidal antirheumatics [76–78] leads to a reduction of pancreatic carcinoma risk. Screening of asymptomatic population Recommendation A screening of asymptomatic persons with CA19–9 for early diagnosis should not be done. Recommendation grade: B, evidence level 3, strong consensus Comment Two studies with large study populations show that screening of asymptomatic persons is not sensible due to the low positive predictive value [79, 80]. Recommendation Molecular biologic screening methods such as mutation analysis to screen the asymptomatic normal population are currently not recommended. Recommendation grade: A, evidence level 5, strong consensus Comment There is currently no scientific evidence which warrants the screening of the asymptomatic normal population with molecular biologic methods. Recommendation Imaging techniques to screen the asymptomatic normal population can currently not be recommended. Recommendation grade: C, evidence level 3b, strong consensus Comment Currently, there is no scientific evidence that mandates the screening of the asymptomatic normal population with imaging techniques [79]. Risk group – identification and monitoring Sporadic pancreatic carcinoma in the family (sporadic pancreatic cancer kindred: SPC) Recommendation Compared to the normal population, first degree relatives of patients with pancreatic carcinoma have an increased risk of also developing pancreatic cancer. Evidence level 2b, consensus Comments The risk is twice as high for first degree relatives of patients with pancreatic carcinoma. If the patient is under 60 years of age, the risk is 3 times as high [81, 82]. Second and third degree relatives are not suitable as index patients to define an individually increased pancreatic carcinoma risk. If pancreatic carcinoma occurs in two or more first degree relatives in one family, this family fulfills the currently valid criteria of “familial pancreatic carcinoma”. Thus, it does not belong to the group of sporadic pancreatic carcinoma. Recommendation A recommendation for primary prevention for relatives of pancreatic carcinoma patients which differs from that for the normal population cannot be given. Recommendation grade: D, evidence level 5, strong consensus Adler G et al. S3-Guideline “Exocrine Pancreatic… Z Gastroenterol 2008; 46: 449–482 Comment In general, the recommendation for the normal population can also be applied to relatives of pancreatic carcinoma patients. There is currently no scientific evidence that supports a benefit of deviating procedures. Familial pancreatic carcinoma (familial pancreatic cancer kindred: FPC) An increased pancreatic carcinoma risk was observed in several hereditary syndromes. In these cases, pancreatic carcinoma is not considered a leading clinical/phenotypic form (see below). Today, familial pancreatic carcinoma (FPC) is differentiated from these syndromes. FPC is always assumed if at least two first degree relatives (independent of age) in one family have pancreatic carcinoma and the family does not fulfill the clinical or familial anamnestic criteria of other hereditary syndromes (see below). FPC-tumors cannot be histologically differentiated from sporadic tumors. A prospective study from Germany showed that between 1 – 3% of all pancreatic carcinoma patients fulfill the FPC-criteria. Thus, FPC has a similar frequency to other hereditary tumor diseases [83]. The mean age of onset of FPC is not significantly different from sporadic cases (€ 62 years). However, preliminary data seem to indicate that offspring of FPC-patients may develop pancreatic carcinoma up to 10 years earlier (anticipation) [84]. As for all hereditary diseases, relatives from FPC-families should be referred to genetic consultation. A specific gene defect for FPC was found for only a small subgroup (about 10%). This is why predictive gene diagnostics outside of clinical studies cannot be recommended at this time. Compared to the normal population, family members with at least two first degree relatives who have pancreatic carcinoma (independent of the patient’s age) have a highly increased risk of also developing pancreatic carcinoma. Evidence level 2b, consensus Comments The risk of a first degree relative to develop pancreatic carcinoma is 18 times as high if two family members have this disease. It can increase up to 57 times if three or more persons are affected. However, these numbers are based only on one prospective study [85]. Therefore, they must be interpreted with caution. Recommendation A recommendation for primary prevention for relatives of FPC-families which differs from that for the normal population cannot be given. Recommendation grade: C, evidence level 3, consensus Comments In general, the recommendations for the normal population can also be applied to relatives of pancreatic carcinoma patients. There is currently no scientific evidence that supports a benefit of deviating procedures. Recently, data on preliminary screening tests using imaging techniques for families at risk were published [86 – 88]. However, they have not been confirmed by independent studies. Therefore, based on the current data a general recommendation of screening tests such as endosonography, ERCP, or MRCP for high risk groups are not recommended outside of controlled studies because of the high risk of false positive results. Downloaded by: Thieme Verlagsgruppe. Copyrighted material.
Other diseases/syndromes which are associated with an increased pancreatic carcinoma risk Patients with Peutz-Jeghers-Syndrome have a 36 to 42% lifetime risk of developing pancreatic carcinoma. Evidence level 2a, strong consensus Comments Two studies prove the much higher risk of developing pancreatic carcinoma for patients with Peutz-Jeghers-Syndrome [89, 90]. Patients with B-K mole-syndrome (including pancreatic-carcinoma-melanoma-syndrome) have an up to 17% lifetime risk of developing pancreatic carcinoma. Evidence level 2b, strong consensus Comments Several studies in FAMMM or families with pancreatic-carcinoma-melanoma-syndrome prove a much higher risk of pancreatic carcinoma [83, 91–93]. Patients and relatives of patients with hereditary breast or ovarian cancer have a higher risk of contracting pancreatic carcinoma. Evidence level 2a, consensus Comments There are only few systematic studies on the pancreatic carcinoma risk in families with hereditary breast or ovarian cancer. These are usually based on families with known germ line mutations in the BRCA 1 and 2 genes. The pancreatic carcinoma risk seems to be 2 to 6 times as high in these families [94 – 96]. Patients and relatives of patients with HNPCC may have a higher risk of developing pancreatic carcinoma. Evidence level 3, consensus Comment The data available are very sparse and point towards a possible risk increase [97, 98]. Patients and their relatives with ataxia teleangiectasia do not have an increased pancreatic carcinoma risk. Evidence level 3, consensus Comment It is difficult to evaluate the data on pancreatic carcinoma risk of ataxia teleangiectasia patients, because of the few numbers of cases. A higher risk of pancreatic carcinoma does not seem to be evident [99]. Patients with FAP and their relatives have an increased risk of pancreatic carcinoma. Evidence level 3, consensus Comments One study reports an increased relative risk of 4.5 for pancreatic carcinoma [100]. Patients with cystic fibrosis probably have no increased risk for pancreatic carcinoma. Evidence level 3, consensus Comment There are no definite statements in the literature on this subject. Patients with Li-Fraumeni-syndrome and their relatives may also have an increased risk of developing pancreatic carcinoma. Evidence level 4, consensus Leitlinie 457 Comment Data are sparse on this subject and further studies are necessary [97]. Other diseases that may be associated with a higher risk of pancreatic carcinoma Patients with von Hippel’s disease and Fanconi’s anemia may have an increased risk of pancreatic carcinoma while patients with neurofibromatosis do not. Evidence level 4 Comments One study reported an association of pancreatic carcinoma with von Hippel’s disease [97]. Furthermore, gene mutations in the FANCC and FANCG genes were determined in patients with pancreatic carcinoma. A loss of heterozygosity was found in resected tumors. These data suggest that patients with mutations in the FANCC gene as is the case in Fanconi’s anemia may have a predisposition for pancreatic carcinoma. The relative and life time risks are not known [101]. Recommendation In general, the recommendation for the normal population to reduce the risk of pancreatic carcinoma also applies to relatives of the abovementioned pancreatic carcinoma patients with hereditary disease. There is currently no scientific evidence that supports a benefit of deviating procedures (see also FPC). This recommendation applies only to the pancreatic carcinoma risk and not to deviating recommendations on screening/monitoring of the corresponding hereditary disease. Recommendation grade: D, evidence level 4, consensus Patients with hereditary pancreatitis Patients with hereditary pancreatitis have a highly increased risk of pancreatic carcinoma. Evidence level 2b, strong consensus Comment The cumulative risk of developing pancreatic carcinoma by the age of 70 is between 40 and 44% in this group [102, 103]. Recommendation Imaging techniques can currently not be recommended to monitor patients with hereditary pancreatitis. Recommendation grade: C, evidence level 3b, consensus Comments Prospective screening of families at risk was performed at two institutions (Johns Hopkins University and University of Seattle). Johns Hopkins recommends an annual screening and the Seattle-program a screening every two to three years. Lesions that are typical for chronic pancreatitis and unexpectedly frequent IPMNs were found using endosonography and ERCP. The value Adler G et al. S3-Guideline “Exocrine Pancreatic… Z Gastroenterol 2008; 46: 449–482 Downloaded by: Thieme Verlagsgruppe. Copyrighted material.
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S3-Guideline “Exocrine Pancreatic Carcinoma” 20071 ... - DGVS

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