Hereditary pancreatitis in childhood: course of disease and complications.

BACKGROUND: Hereditary pancreatitis is rare. Pain therapy for juvenile symptom onset, child development and the risk of pancreatic carcinoma in adulthood must be considered. PATIENTS, MATERIAL AND METHODS: Data from a cohort of 11 patients with disease onset in childhood (< 16 years) were analyzed retrospectively. The gene encoding cationic trypsinogen (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1) and cystic fibrosis transmembrane conductance regulator (CFTR) genes were investigated as genetic factors. Treatment concept and complications were registered. Prognosis, treatment success and quality of life were objectified using the chronic pancreatitis prognosis score and a standardized questionnaire (KIDSCREEN-10 index). RESULTS: The mean age of symptom onset was 7.5 ±4.2 years. The PRSS1 and SPINK1 mutations each occurred with 36.4%, 3 patients had a pancreas divisum and 2 a long common channel. The course of pancreatitis was obstructive in 90.9%. Exocrine pancreatic insufficiency occurred in seven patients so far (mean age 12.5 years). Stenting was performed in 72.7% and 18.2% needed pancreatic surgery. Currently the chronic prognosis score is on average 7.5 points, pain on numerical rating scale 0 (no pain). The mean KIDSCREEN­T score of 66.9 confirms a very good quality of life. CONCLUSION: Patients with genetically caused chronic pancreatitis are rare. Their care ranges from pain therapy in childhood and adolescence to questions concerning family planning and pancreatic cancer prevention from mid-adulthood onward. The disease is challenging for the interdisciplinary cooperation. We found the step-up strategy to be a good option for pain therapy. A national registry monitored by scientific societies with active recruitment for screening examinations will further improve and ensure care in the long term.

Clinical Outcome in Patients with Carcinoma of the Esophagogastric Junction Treated with Neoadjuvant Radiochemotherapy or Perioperative Chemotherapy: A Two-Center Retrospective Analysis.

BACKGROUND: Adenocarcinoma of the esophagogastric junction (AEG) is a rare but rising tumor entity in the Western world. Treatment is complex, as multimodality is key to optimal results. However, trials solely including AEG are rare, and the question if neoadjuvant radiochemotherapy (NRCT) or neoadjuvant/perioperative chemotherapy (NACT) is superior remains unanswered. PATIENTS AND METHODS: Patients with AEG I-III treated between October 2010 and August 2019 at the Ordensklinikum Linz or the Kepler University Hospital were identified either from a monitored tumor registry or by chart review. Time-to-event data were analyzed by Kaplan-Meier product limit estimation. The Kruskal-Wallis test and Fisher's exact test were used for comparing continuous and categorical data, respectively. RESULTS: A total of 85 patients (median age 63 years; median Charlson Comorbidity Index 3; 98.8% ECOG PS 0-1) were analyzed. Of these, 52 patients received NRCT (81% CROSS protocol) and 33 NACT (65% EOX and 35% FLOT protocol). There was a significantly higher pathological complete response rate in the NRCT group (30 vs. 12%; p = 0.010); distant relapse rates were higher in the NRCT group and local relapse rates were higher in the NACT group (both not significant). These differences, however, did not translate into a different disease-free survival (20 months; 95% CI: 13-34) or overall survival (44 months; 95% CI: 33-NA). Patients >65 years old had the same advantage from treatment as patients <65 years of age. CONCLUSIONS: Although treatment of AEG is complex, the progress documented over the last centuries can be reproduced in our real-life setting. Data regarding the superiority of either type of neoadjuvant/perioperative treatment are sparse. We assume no difference between EOX-based NACT and NRCT.