Calprotectin as Diagnostic Marker for Hepatic Encephalopathy and Spontaneous Bacterial Peritonitis in Cirrhosis.

BACKGROUND: Calprotectin is a well-established marker for intestinal inflammation, mainly in inflammatory bowel disease, and represents one of the most studied biomarkers in stool samples. METHODS: Apart from its important diagnostic role in inflammatory bowel disease, there are few studies showing that calprotectin can also be used as a diagnostic tool in patients suffering from hepatic encephalopathy (HE) and spontaneous bacterial peritonitis (SBP) in cirrhosis. RESULTS: Since calprotectin concentration in the human stool or in ascites is elevated at an early stage of inflammation, it might serve as an early screening tool for patients suffering from cirrhosis who are at risk to develop these conditions. As detection and monitoring of HE and SBP may be unclear and resource-intensive, identification of valid new markers of disease activity is necessary. In this review, we summarize the current knowledge of calprotectin as a diagnostic biomarker in cirrhosis, indicating that it is a highly promising diagnostic surrogate marker to screen for the presence of HE and SBP. CONCLUSIONS: To screen cirrhotic patients for SBP, calprotectin should be assessed in ascitic fluid while it should be measured in feces when screening for HE. However, the value of calprotectin in managing individual patients must be considered in the specific clinical context.

[Endoscopic mucosal resection (EMR) followed by radiofrequency ablation (RFA) in neoplastic Barrett's esophagus or Barrett early cancer is also economically superior to sole radical endoscopic resection]. / Endoskopische Mukosaresektion (EMR) mit nachfolgender Radiofrequenzablation (RFA) bei neoplastischem Barrett-Ösophagus bzw. Barrett-Frühkarzinom ist der alleinigen radikalen endoskopischen Resektion auch ökonomisch überlegen.

INTRODUCTION: Neoplastic changes (mild or high grade intraepithelial neoplasia (L- or HGIEN) or early cancer) in Barrett esophagus are treated with various methods. This study compares clinical-economical aspects of sole stepwise radical endoscopic resection (SRER) against combination treatment with EMR (Endoscopic mucosal resection) and RFA (radiofrequency ablation). MATERIAL AND METHODS: Based on clinical data from a randomized controlled trial 1 we developed an economic model for costs of treatment according to the German Hospital Remuneration System (G-DRG). Our calculating incorporated initial treatment costs and the cost of treating complications (both paid via G-DRG). RESULTS: Medical and economically, the treatment with EMR + RFA advantages over sole SRER treatment 1. The successful complete resection or destruction of neoplastic intestinal metaplastic tissue is similar in both procedures. Acute complications (24 vs. 13 % in SRER EMR + RFA) and late complications (88 vs. 13 % in SRER EMR + RFA) are significantly more likely in sole SRER than in the EMR + RFA. DISCUSSION: While SRER initially appears more cost-effective as a sole therapy, cost levels move significantly above EMR+RFA due to higher complication rates and following procedures costs. Overall, the costs of treatment was €â€Š13 272.11 in the SRER group and €â€Š11 389.33 in the EMR + RFA group. The EMR + RFA group thus achieved a cost advantage of €â€Š1882.78. The study shows that the treatment of neoplastic Barrett esophagus with EMR + RFA is also appropriate in economic terms.

[Secondary retroperitoneal fibrosis in a 39-year-old man after rectal cancer]. / Sekundäre retroperitoneale Fibrose nach Rektumkarzinom bei einem 39-jährigen Mann.

A 39-year-old man had been treated for rectal cancer 6 years ago by lower anterior resection of the rectum and perioperative radiochemotherapy. Since then follow-up had been unremarkable but now the patient presented with unspecific lower abdominal pain. The cause of the pain was identified as paraneoplastic retroperitoneal fibrosis secondary to metachronous pulmonary metastases of the rectal cancer.

[Economic burden of Clostridium difficile enterocolitis in German hospitals based on routine DRG data]. / Ökonomische Auswirkungen einer Clostridium-difficile-Enterokolitis in deutschen Krankenhäusern auf der Basis von DRG-Kostendaten.

BACKGROUND: Clostridium difficile associated diarrhea (CDAD) is not only a increasing medical but also economical problem. METHODS: Data from the DRG project group of the German society for digestive and metabolic diseases (DGVS) were analyzed for CDAD. Out of 430,875 cases from 37 German hospitals 2,767 cases were grouped by having CDAD either as primary (PD) or secondary diagnosis (SD; likely to be from a hospital source) in an initial or recurring hospital stay (RD). For comparison non-CDAD cases from the same hospitals from that year where matched using propensity score matching. As endpoints we defined LOS (length of stay), difference of LOS to national average LOS, total costs per case and difference between costs and revenue for all three groups. RESULTS: Patients from the PD group (n = 817) showed a mean LOS of 11.2 days compared to 8.5 days for the control group, 4,132 € mean cost per case (536 € more than control) and a mean loss of -1,064 € per case compared to -636 €. In the SD group (n = 1,840) patients stayed in the hospital for 28.8 days (control: 18.1 days), had costs of 19,381 € (control: 13,082 €) and a loss of -3,442 € compared to -849 € in the control group. Recurring cases (RD; n = 110) showed a LOS of 37.3 days (control: 21.3 days), had even higher costs (20.755 € vs. 13,101 €) and higher losses (-4,196 € vs. -1,109 €). CONCLUSION: By extrapolating these findings CDAD not only harms patients but generates a yearly cost burden of 464 million € for the German healthcare system including a loss of 197 million € for German hospitals. To the authors' opinion sufficient measures against CDAD should include pre hospital risk reduction programs, introduction of effective therapeutic and hygienic strategies in hospitals as well as improvements in documentation for these cases to support further developments of the German DRG system.

[Improvement of cost allocation in gastroenterology by introduction of a novel service catalogue covering the complete spectrum of endoscopic procedures]. / Verbesserung der Kostenkalkulation in der Gastroenterologie durch Einführung eines neuen Leistungskatalogs für alle endoskopischen Prozeduren.

BACKGROUND: The German hospital reimbursement system (G-DRG) is incomplete for endoscopic interventions and fails to differentiate between complex and simple procedures. This is caused by outdated methods of personnel-cost allocation. METHODS: To establish an up-to-date service catalogue 50 hospitals made their anonymized expense-budget data available to the German-Society-of-Gastroenterology (DGVS). 2.499.900 patient-datasets (2011-2013) were used to classify operation-and-procedure codes (OPS) into procedure-tiers (e.g. colonoscopy with biopsy/colonoscopy with stent-insertion). An expert panel ranked these tiers according to complexity and assigned estimates of physician time. From June to November 2014 exact time tracking data for a total 38.288 individual procedures were collected in 119 hospitals to validate this service catalogue. RESULTS: In this three-step process a catalogue of 97 procedure-tiers was established that covers 99% of endoscopic interventions performed in German hospitals and assigned validated mean personnel-costs using gastroscopy as standard. Previously, diagnostic colonoscopy had a relative personnel-cost value of 1.13 (compared to gastroscopy 1.0) and rose to 2.16, whereas diagnostic ERCP increased from 1.7 to 3.62, more appropriately reflecting complexity. Complex procedures previously not catalogued were now included (e.g. gastric endoscopic submucosal dissection: 16.74). DISCUSSION: This novel service catalogue for GI-endoscopy almost completely covers all endoscopic procedures performed in German hospitals and assigns relative personnel-cost values based on actual physician time logs. It is to be included in the national coding recommendation and should replace all prior inventories for cost distribution. The catalogue will contribute to a more objective cost allocation and hospital reimbursement - at least until time tracking for endoscopy becomes mandatory.

Clinical manifestations and treatment options in patients with cirrhosis and diabetes mellitus.

BACKGROUND: Diabetes is frequently diagnosed in patients with cirrhosis and represents an important risk factor for morbidity and mortality. Pharmacological therapy is limited due to hepatotoxicity and the risk of hypoglycemia. Investigations on medical practice in this patient population, frequency of diabetes-associated complications and the impact of quality of metabolic control are rare. AIMS AND METHODS: A retrospective analysis was performed to compare the effects of hypoglycemic treatment, the achieved glycemic control under therapy, the prevalence of typical cirrhosis-related or microangiopathic complications, and cardiovascular comorbidities between a group of diabetic patients with cirrhosis (n = 87) and a nondiabetic cirrhotic population (n = 198). RESULTS: The prevalence of diabetes in our cohort was 30.5%. Of all diabetic patients, 39.1% received therapy which might potentially result in serious side effects in patients with end-stage liver disease. The rate of ongoing alcohol abuse (28.7%) and noncompliance under medication (41.4%) was high. Only 28.7% of all diabetic subjects showed satisfactory (as defined by HbA1c ≤ 6.5%) glycemic control under therapy. Patients achieving satisfactory control experienced a lower rate of certain cirrhosis-related complications such as hepatic encephalopathy (HE) and hepatocellular carcinoma (HCC), arterial hypertension, and hypercholesterolemia. HE was significantly more frequent in diabetic than nondiabetic cirrhotic patients.

Treatment of metastatic colorectal cancer with cetuximab: influence on the quality of life.

BACKGROUND: Epidermal Growth Factor Receptor (EGFR) antibodies are innovative anti-cancer drugs prolonging survival in metastatic colocrectal cancer. However, due to adverse drug reactions, patients develop acneform skin toxicities. We hypothesized that the skin reaction leads to a decline in general (QOL) and dermatological health related quality of life (HQOL). Furthermore, we aimed at evaluating predictors for QOL and HQOL to improve individual adjustment of therapy. METHODS: 40 outpatients with metastatic colocrectal cancer were involved in this study. According to their KRAS status, patients were allocated to 2 groups: The CTCX group (n = 20; KRAS wild-type) was treated with the EGFR-antibody Cetuximab plus chemotherapy, the CT group (n = 20; KRAS mutation) was receiving chemotherapy only. Psychological assessment consisted of questionaires to evaluate QOL and HQOL, depression, coping-styles, health beliefs and the patient´s personality. RESULTS: Between the two groups, no significanct difference in QOL was found, QOL remained stable over the course of treatment. Yet, the severity of the skin reactions had a significant influence on HQOL. Internal health beliefs and high compliance were found to be protective factors, while passive coping strategies, depression and the personality trait neuroticism were identified as risk factors. DISCUSSION: Interdisciplinary cooperation between medical professionals and psycho-oncologists is strongly recommended to encourage patients to embark on and to retain EGFR-antibody therapy. If risk factors are present, psycho-oncological therapy should focus on the minimization of depression and on the development of active coping strategies.

[How safe is sedation in gastrointestinal endoscopy? A multicentre analysis of 388,404 endoscopies and analysis of data from prospective registries of complications managed by members of the Working Group of Leading Hospital Gastroenterologists (ALGK)]. / Wie sicher ist die Sedierung in der gastrointestinalen Endoskopie? Eine multizentrische Auswertung von 388 404 Endoskopien und Auswertung der Daten aus prospektiv geführten Komplikationsregistern von Mitgliedern der Arbeitsgemeinschaft leitender Gastroenterologen im Krankenhaus (ALGK).

BACKGROUND: Gastrointestinal endoscopies are increasingly being carried out with sedation. All of the drugs used for sedation are associated with a certain risk of complications. Data currently available on sedation-associated morbidity and mortality rates are limited and in most cases have substantial methodological limitations. The aim of this study was to record severe sedation-associated complications in a large number of gastrointestinal endoscopies. METHODS: Data on severe sedation-associated complications were collected on a multicentre basis from prospectively recorded registries of complications in the participating hospitals (median documentation period 27 months, range 9 - 129 months). RESULTS: Data for 388,404 endoscopies from 15 departments were included in the study. Severe sedation-associated complications occurred in 57 patients (0.01 %). Forty-one percent of the complications and 50 % of all complications with a fatal outcome (10/20 patients) occurred during emergency endoscopies. In addition, it was found that 95 % of the complications and 100 % of all fatal complications affected patients in ASA class ≥ 3. CONCLUSIONS: Including nearly 400,000 endoscopies, this study represents the largest prospective, multicenter record of the complications of sedation worldwide. The analysis shows that sedation is carried out safely in gastrointestinal endoscopy. The morbidity and mortality rates are much lower than previously reported in the literature in similar groups of patients. Risk factors for the occurrence of serious complications include emergency examinations and patients in ASA class ≥ 3.

Effects of polypharmacy on outcome in patients with schizophrenia in routine psychiatric treatment.

OBJECTIVE: Evaluating the effects of different types of psychotropic polypharmacy on clinical outcomes and quality of life (QOL) in 374 patients with schizophrenia and schizoaffective disorder in routine care. METHOD: Psychotropic regimen, clinical outcomes, and QOL were assessed before discharge and after 6, 12, 18, and 24 months. Data were analyzed by mixed-effects regression models for longitudinal data controlling for selection bias by means of propensity scores. RESULTS: At baseline 22% of participants received antipsychotic monotherapy (APM) (quetiapine, olanzapine, or risperidone), 20% more than one antipsychotic drug, 16% received antipsychotics combined with antidepressants, 16% antipsychotics plus benzodiazepines, 11.5% had antipsychotics and mood stabilizers, and 16% psychotropic drugs from three or more subclasses. Patients receiving APM had better clinical characteristics and QOL at baseline. Patients receiving i) antipsychotics plus benzodiazepines or ii) antipsychotics plus drugs from at least two additional psychotropic drug categories improved less than patients with APM. CONCLUSION: Combinations of antipsychotics with other psychotropic drugs seem to be effective in special indications. Nevertheless, combinations with benzodiazepines and with compounds from multiple drug classes should be critically reviewed. It is unclear whether poorer outcomes in patients with such treatment are its result or its cause.

Accuracy of anorectal manometry in patients with fecal incontinence.

BACKGROUND/AIM: Patients with fecal incontinence (FI) have lower anal resting (MRP) and squeeze (MSP) pressure and an impaired sensitivity compared to healthy people. However, whether anorectal manometry (ARM) can separate precisely between health and disease is discussed controversially. The aim was to evaluate the accuracy of ARM in a huge cohort of patients and controls. METHODS: ARM was obtained in 144 controls and in 559 FI patients. MRP, MSP, and balloon volume at first perception (BVP) and urge sensation (BVU) were determined. Receiver operating curve analysis was used to determine optimal cut-offs and sensitivity, specificity and accuracy calculated. RESULTS: FI patients showed lower MRP, MSP, BVU (p < 0.001) and a higher BVP (p = 0.007). Deterioration of the ARM parameter increased with FI severity. ARM demonstrated an excellent sensitivity (91.4%) and accuracy (85.8%), but only a moderate specificity (62.5%). The sensitivity of ARM rose with FI severity. The pressure data showed higher sensitivity and accuracy than the sensory data despite comparable specificity. CONCLUSIONS: Sensitivity and accuracy of single ARM parameters is only moderate for the pressure data and poor for the sensory data. In contrast, ARM demonstrated an excellent sensitivity, a moderate specificity, and a convincing accuracy justifying its use in clinical routine.

[Management of impacted foreign bodies in the upper gastrointestinal tract in adult patients - results of a retrospective case series]. / Management von impaktierten Fremdkörpern im oberen Gastrointestinaltrakt bei erwachsenen Patienten - Ergebnisse einer retrospektiven Fallserie.

INTRODUCTION: Impaction of foreign bodies in the upper gastrointestinal tract represents a rare endoscopic emergency regarding the danger of organic lesions. Therefore, the therapeutic strategy, rational postinterventional monitoring and potential consequences for the course in the future are often not clear. METHODS: Out of all oesophagogastroduodenoscopies performed between 2008 and 2010 (n = 10,830), the endoscopies due to bolus impaction were analysed retrospectively concerning endoscopic findings, course of intervention and causative predisposing factors using descriptive statistics. RESULTS: In the study period, endoscopy was performed in 45 different patients 49 times when foreign body impaction was suspected. In 38 cases (77.6%) a foreign body could be detected by endoscopy, most frequently a meat bolus (65.8%), followed by dental prostheses and tablets. Endoscopic removal could be successfully performed in 97.4%. An operation was necessary in only one patient. A macroscopically unremarkable upper gastrointestinal tract without any predisposing conditions for foreign body impaction could be detected in 33.3%. In one case eosinophilic oesophagitis could be diagnosed histologically. The foreign body was pushed into the stomach by the "push technique" in 54.1%. The foreign body removal was performed as an ambulant intervention in 63.2%. DISCUSSION: In the majority of cases, impacted foreign bodies of the upper gastrointestinal tract could be removed safely by endoscopy, e.g., using the "push technique". However, the therapeutic strategy should be individually adjusted based on the consistancy of the foreign body. If no predisposing conditions can be found, histological diagnosis should be performed to detect potential eosinophilic oesophagitis early enough.

[Adenocarcinoma of the stomach and neuroendocrine carcinoma of the colon in a 45-year-old male patient suffering from common variable immunodeficiency (CVID) and ulcerative colitis]. / Adenokarzinom des Magens und neuroendokrines Kolonkarzinom bei einem 45-jährigen Patienten mit Common Variable Immunodeficiency (CVID) und Colitis ulcerosa.

Common variable immunodeficiency (CVID) is the most common primary antibody deficient syndrome in adults. Among the broad spectrum of clinical manifestations are recurrent infections, allergies, autoimmune, tumour, pulmonary, liver and gastrointestinal diseases. Here we report the case of a 45-year-old male patient, who has been suffering from ulcerative colitis - likewise recognised as a CVID-associated disease - for many years. He was admitted to our clinic with a rapid progressive reduction of his general condition and a loss of weight. Diagnostic work-up revealed adenocarcinoma of the stomach as well as an undifferentiated neuroendocrine carinoma of the colorectum at the rectosigmoidal junction. Curative resection of the distal stomach and proctolcolectomy were performed. To date, the pathogenesis of the association of many diseases with CVID is still ambiguous. Yet, there is no doubt about the significantly higher incidence of e.g., inflammatory bowel disease or gastric cancer in patients with CVID. Our case highlights that in patients with CVID and obscure deterioration of their general health condition a careful search for especially malignant complications is mandatory although to date there are no precise recommendations for screening.

[Frequency of cardiac arrhythmia in patients with liver cirrhoses and evaluation of associated factors]. / Häufigkeit von Herzrhythmusstörungen bei Patienten mit Leberzirrhose sowie Evaluierung assoziierter Faktoren.

INTRODUCTION: Studies analysing the frequency of rhythm disorders in patients with cirrhosis are rare. Nevertheless, factors triggering rhythm disorders occur frequently in cirrhosis. Therefore, a retrospective case control study was performed investigating the frequency of cardiac arrhythmia in a population of patients with cirrhosis while evaluating several associated factors. METHODS: The files of patients with cirrhosis (n  =  293) in the period 2004 - 2008 were analysed retrospectively regarding cardiac arrhythmia. The frequency of cardiac arrhythmia in the presence of relevant risk factors was analysed using χ ²tests and logistic regression models. RESULTS: 61.1  % of all patients were male (mean age 61.7 years) and 38.9  % female (mean age 62.8 years). The severity of cirrhosis according to the Child-Pugh score (CP) was as follows: CP A 43.3  %, CP B 32.8  % and CP C 23.9  %. Altogether, rhythm disorders were diagnosed in 16.4  % (48/293) of the study population, most frequently atrial fibrillation (68.8  %) and atrial flutter (6.7  %). An advanced age and comorbidities such as arteriosclerotic diseases, hypercholesterinemia (p  <  0.001, each) and diabetes mellitus (p  =  0.013) correlated significantly with the frequency of rhythm disorders which occurred more often in males than in females (p  = 0.066). Ongoing alcohol abuse, the severity of cirrhosis and arterial hypertension were not associated significantly with the onset of rhythm disorders. 84.4  % of all patients with cardiac arrhythmia were treated by diuretics. Decreased (<  3.5 mmol/L) and elevated (>  5 mmol/L) potassium values were observed in 60.6  % of the study collective. Rhythm disorders were more often observed in patients with hyperkalemia (especially atrioventricular block, p  < 0.01). CONCLUSION: Compared to the average population, the prevalence of atrial fibrillation was increased in our cirrhotic cohort. The occurrence of rhythm disorders was significantly associated with arteriosclerotic diseases, hypercholesterinaemia and diabetes mellitus. Additionally, cardiac arrhythmia must be considered under diuretic therapy and in the presence of electrolyte disturbances.

[Current clinical practice among German Internists regarding the prophylaxis of gastroduodenal ulcers associated with NSAIDs, aspirin and Helicobacter pylori]. / Klinische Praxis der Prophylaxe gastroduodenaler Ulzera.

OBJECTIVE: Rapidly growing information on adverse gastrointestinal effects of non-steroidal anti-inflammatory drugs (NSAIDs) and Helicobacter pylori (Hp) causes continuous changes in clinical patient management. Decisions on the prevention of gastroduodenal ulcers in individual patients often do not follow guidelines. We aimed to assess the current management of gastroduodenal ulcers by internists in southern Germany. METHODS: All 965 members of the Association of Bavarian Internists, a German province with about 8000 internists, were invited to participate in answering a 12-item questionnaire. The questions addressed different clinical scenarios regarding ulcer disease associated with traditional NSAIDs (tNSAIDs), aspirin and Hp. Particularly, we asked for the clinical approach to patients with NSAID-/aspirin-associated ulcers and prophylactic measures before the beginning of a potentially ulcerogenic medication. RESULTS: N = 225 (23.3 %) physicians returned completed questionnaires. In patients with Hp-negative, NSAID-/aspirin-associated ulcers, > 80 % of respondents would initiate long-term proton pump inhibitor (PPI) therapy, whereas 20 % and 17.8 % would prescribe COX-2 selective inhibitors or opiates instead of non-selective NSAIDs and clopidogrel instead of aspirin. The management of Hp-positive ulcers, especially in cases with additional use of aspirin or NSAIDs, was very heterogenous, including Hp-eradication only, eradication +  long-term PPI, eradication +  clopidogrel or COX-2-inhibitors/opiates. CONCLUSION: This survey depicts individual discrepancies in the clinical management of patients receiving NSAIDs and/or aspirin, regarding the prophylaxis of gastroduodenal ulcer disease.

Endoscopic injection of autologous blood for bleeding gastroduodenal ulcers.

Endoscopic injection of diluted epinephrine and the application of endoclips or thermal probes are established endoscopic therapies for peptic ulcer bleeding (PUB). However, autologous blood may offer advantages at least over diluted epinephrine due to its specific properties: corpuscular components may provide prolonged persistence and procoagulatory constituents may additionally prevent recurrent bleeding. We performed successful endoscopic injection of autologous blood for acute spurting (Forrest Ia) or oozing (Ib) bleeding from duodenal or gastric ulcers. The injection of autologous blood was simple to apply and achieved hemostasis in bleeding Forrest I peptic ulcers. This technique can be considered as an adjunct or an alternative to other methods for endoscopic control of PUB.

Influence of gender and age on anorectal function: normal values from anorectal manometry in a large caucasian population.

INTRODUCTION: In the literature, data on the effects of gender and age on the pressure data of anorectal manometry differ. Possible reasons are investigation of only small numbers of healthy people and comparison of only 2 groups with large age differences. In addition, data about the influence of gender or age on anorectal sensation are sparse. Therefore, the aim of the present study was to determine the influence of gender and age on anorectal manometry in a large healthy female and male cohort spanning a great age range. METHODS: Anorectal manometry was performed in 72 women and 74 men with a median age of 64 years in both groups (ranges: women 22-90 years; men 23-88 years). We determined mean anal resting and squeeze pressure as well as minimal rectal balloon volume for perception and for urge/desire to defecate. The Mann-Whitney U test was used to analyze for gender differences, regression analysis to search for age influences. RESULTS: Squeeze pressure (p = 0.007) and perception threshold (p < 0.001) are significantly lower in females, while the mean resting pressure and urge threshold are similar in females and males. Mean resting pressure (women p < 0.0001; men p = 0.03) and mean squeeze pressure decrease (women p < 0.0001; men p = 0.004) with age. An age-related increase in sensory thresholds (= decreased rectal sensitivity) is only seen in females (perception threshold p = 0.01; urge threshold p = 0.04). CONCLUSION: Most of the parameters measured by anorectal manometry (anal canal pressure, sensory thresholds) are influenced by gender and age. Therefore, the results of anorectal manometry must be interpreted in relation to sex- and age-adapted normal values.

[Mesenteric inflammatory veno-occlusive disease (MIVOD)--an underdiagnosed disease?]. / Mesenterische inflammatorische veno-okklusive Erkrankung (MIVOD) - eine unterdiagnostizierte Erkrankung?

Mesenteric, inflammatory veno-occlusive disease is an entity of unknown incidence and aetiology. Inflammation and necrosis of mesenteric veins leads to severe ischaemic damage of an intestinal segment. The clinical and endoscopic presentation can be ambiguous. However, modern multiphasic contrast-enhanced computed tomography allows early diagnosis, when characteristic features are considered. As local resection is the only known cure with minimal risk of recurrent disease, knowledge of this rare disease entity can spare our patients a risky delay of resection and erroneous therapeutic approaches.