Endoscopic resection of ampullary lesions: a single-center 8-year retrospective cohort study of 91 patients with long-term follow-up

Background

Endoscopic ampullectomy is established as a valuable treatment for adenomas of the Vaterian papilla. Few large series are available, however, let alone any with long-term follow-up. Moreover, multiple tangible issues remain. The aim of our study was to evaluate efficacy, safety, and outcome of endoscopic ampullectomy and compare it to existing literature

Methods

This is a single-center, retrospective study with a minimal follow-up of 3 years including 91 patients, including familial adenomatous polyposis (FAP) and non-FAP, who had an endoscopic ampullectomy between 2000 and 2008. Outcome parameters included ampulloma characteristics, biotical accuracy as well as safety, efficacy, recurrence rate, and survival after endoscopic ampullectomy.

Results

Endoscopic resection was successful in 71 patients (78 %). Histological review of the resected specimens revealed nonspecific changes (13.8 %), low or medium grade dysplasia (52.9 %), high grade dysplasia (21.8 %) and carcinoma (18.3 %). Bioptic accuracy was 38.3 %. Overall complications were observed in 23 patients (25.2 %): pancreatitis (15.4 %), hemorrhage (12.1 %) and cholangitis (4.9 %). Recurrence occurred in 18.3 %. Fourteen patients underwent pancreaticoduodenectomy. Survival after complete endoscopic ampullectomy was excellent for patients with low to moderate grade dysplasia and high grade dysplasia. Incomplete endoscopic resection of high grade dysplasia or invasive carcinoma was associated with unfavorable outcome when treated merely endoscopically.

Conclusions

Endoscopic ampullectomy is obligatory for assessment of the true histological nature of an ampulloma. Endoscopic resection is a safe and efficient procedure for adenomas with low to moderate dysplasia but also for high grade dysplastic lesions, provided that a complete endoscopic resection is achieved.     

Action perception predicts action performance

Everyday action impairments often are observed in demented older adults, and they are common potential barriers to functional independence. We evaluated whether the ability to segment and efficiently encode activities is related to the ability to execute activities. Further, we evaluated whether brain regions important for segmentation also were important for action performance. Cognitively healthy older adults and those with very mild or mild dementia of the Alzheimer's type watched and segmented movies of everyday activities and then completed the Naturalistic Action Test. Structural MRI was used to measure volume in the dorsolateral prefrontal cortex (DLPFC), medial temporal lobes (MTL), posterior cortex, and anterior cingulate cortex (ACC). Dementia status and the ability to segment everyday activities strongly predicted naturalistic action performance, and MTL volume largely accounted for this relationship. In addition, the current results supported the Omission-Commission Model: Different cognitive and neurological mechanisms predicted different types of action error. Segmentation, dementia severity, and MTL volume predicted everyday omission errors, DLPFC volume predicted commission errors, and ACC volume predicted action additions. These findings suggest that event segmentation may be critical for effective action production, and that the segmentation and production of activities may recruit the same event representation system.     

Progressive amnestic dementia, hippocampal sclerosis, and mutation in C9ORF72

The most common cause of familial frontotemporal lobar degeneration with TAR DNA-binding protein-43 pathology (FTLD-TDP) has been found to be an expansion of a hexanucleotide repeat (GGGGCC) in a noncoding region of the gene C9ORF72. Hippocampal sclerosis (HpScl) is a common finding in FTLD-TDP. Our objective was to screen for the presence of C9ORF72 hexanucleotide repeat expansions in a pathologically confirmed cohort of “pure” hippocampal sclerosis cases (n = 33), outside the setting of FTLD-TDP and Alzheimer’s disease (AD). Using a recently described repeat-associated non-ATG (RAN) translation (C9RANT) antibody that was found to be highly specific for c9FTD/ALS, we identified a single “pure” HpScl autopsy case with a repeat expansion in C9ORF72 (c9HpScl). Mutation screening was also performed with repeat-primed polymerase chain reaction and further confirmed with Southern blotting. The c9HpScl patient had a 14-year history of a slowly progressive amnestic syndrome and a clinical diagnosis of probable AD. Neuropsychological testing revealed memory impairment, but no deficits in other cognitive domains. Autopsy showed hippocampal sclerosis with TDP-43 immunoreactive neuronal inclusions relatively limited to limbic lobe structures. Neuritic pathology immunoreactive for p62 was more frequent than TDP-43 in amygdala and hippocampus. Frequent p62-positive neuronal inclusions were present in cerebellar granule neurons as is typical of C9ORF72 mutation carriers. There was no significant FTLD or motor neuron disease. C9RANT was found to be sensitive and specific in this autopsy-confirmed series of HpScl cases. The findings in this patient suggest that the clinical and pathologic spectrum of C9ORF72 repeat expansion is wider than frontotemporal dementia and motor neuron disease, including cases of progressive amnestic dementia with restricted TDP-43 pathology associated with HpScl.