Background
Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years.
Material and methods
Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences.
Results
Fifty‐one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7–17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0–163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow‐up of 65 months [0.3–221], the overall and event‐free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33–94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03).
Conclusions
SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.
This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.
Background: Unusual clinical presentation of acute appendicitis in preschool children leads to misdiagnosis and complications. We aimed to analyze the influence of age on clinical presentation, laboratory findings and complications in preschool children with acute appendicitis. Methods: From January 2012 until December 2017, 29 children younger than 6 years of age (median 50 months) with acute appendicitis were enrolled in this retrospective study. Patients were grouped according to their age: group 1: < 48 months (n = 13); group 2: > 48 months (n = 16), their clinical data, laboratory results and complications were compared. Results: In group 1, duration of nausea and vomiting was longer, alteration of general state was more frequent and pain in the right fossa iliaca less frequent than in group 2 (p = 0.026, p = 0.000 and p = 0.029, respectively). Heart rate was higher in group 1 than in group 2 (p = 0.012). Leucocyte and polynuclear neutrophil counts were lower in group 1 than in group 2 (p = 0.028 and = 0.004, respectively) but C-reactive protein levels were not different between groups. In the whole cohort however, C-reactive protein at admission value correlated negatively with age (p = 0.025). Abdominal ultrasound allowed diagnosis in 19/29 patients (65.5%), without any difference between groups. Appendicular perforation was more frequent in group 1 than in group 2 (p = 0.003). Perforation was also related to longer hospital stay (p = 0.018). Peritonitis occurred in 21/29 (72%), post-operative ileus in 5/29 (17%) and sepsis in 4/29 (14%) patients without any difference between groups. In the whole cohort, hospital stay correlated negatively with age (p = 0.000). There was no mortality. Conclusions: Among preschool children, those younger than 48 months present with longer duration of preadmission symptoms indicating longer infection course than in older children. Altered general state and higher degree of tachycardia in the younger reflect higher systemic repercussions of the illness. Less specific abdominal pain and dissociation of the inflammatory markers with lower leucocyte-and neutrophil counts and higher Creactive protein levels in the younger may contribute to further diagnosis delay and higher rate of perforation in these patients.
The laparoscopic approach of high imperforate anus allowed an easy control of the fistula and helped to determine the accurate passage through the levator ani. Nevertheless, technical points have to be improved,such as coprostasis before fistula section to avoid stool leakage, rectum tacking to prevent prolapse,and calibration of muscle channel to avoid stenosis.
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