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Inflammatory myofibroblastic tumours (IMTs) are rare soft tissue tumours. Reports of gastrointestinal tract, liver and pancreas tumours are limited. The objective of this study is to identify presenting features, contributing prognostic / etiological factors and any variability in outcomes in the context of different historical treatments. We retrospectively reviewed the records of seven children treated at our hospital between 2006 and 2019 and assessed the demographic, presentation, treatment, immunohistochemistry, and outcomes of their tumours. Age range at presentation was 4 months-15 years with a male predominance. Presentations were typically due to local mass effect or incidental discovery. Systemic symptoms were rare. Outcomes were good with six out of seven stable or in remission irrespective of treatment. Surgical resection where possible is the treatment of choice. Medical therapy had good outcomes with chemotherapy acting as first line treatment when required. The only negative prognostic factor identified was local spread at the time of presentation.

Background and Aims:Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder that requires repeat endoscopic evaluation(s) to assess response to treatment. This results in high health care costs and a procedural burden in affected children. Noninvasive alternate modalities to reassess disease activity have not been established. Low baseline impedance measured by multichannel pH impedance (pH-MII) is seen in adults with EoE, in keeping with poor mucosal integrity. We aimed to investigate the relationship between esophageal eosinophilia (or severity of eosinophilic infiltration) and baseline impedance in children with EoE.Methods:We retrospectively identified 15 children diagnosed with EoE at our institution who had undergone pH-MII within 30 days of 3-level esophageal biopsy. This group were not concurrently prescribed proton pump inhibitors and had negligible reflux parameters on pH-MII. Average impedance baseline was calculated upper, mid, and lower esophageal segments via baseline impedance automated analysis (RIAA) and mean nocturnal baseline impedance (MNBI) methods. Eosinophil count data for upper, mid, and lower esophageal segments in the EoE group was collated.Results:A significantly lower baseline impedance was seen across the esophageal length in children with EoE, compared with 30 controls who had no differences in age or reflux burden on nonparametric testing. A relationship between baseline impedance and eosinophil number at corresponding esophageal segments was not established.Conclusions:Baseline impedance may be an important, less invasive adjunct in clinical practice to monitor treatment response in children with EoE. Larger prospective cohort studies should delineate optimally predictive baseline impedance thresholds for active and inactive disease.

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This two-day-old term, female infant was born via uncomplicated vaginal delivery following an uneventful pregnancy. On the first day of life, the infant was noted to have pink, raised, perianal softtissue protuberances, larger while crying but spontaneously reducing when calm (Fig. 1).Anorectal disorders in children include neonatal etiologies, mostly anorectal malformations and infantile/childhood causes such as anal fissures and hemorrhoids (1). Our team suspected neonatal hemorrhoids, which can be manifested as an isolated clinical problem or can coexist with more complex anorectal anomalies (2,3).Neonatal hemorrhoids can be caused by compression of the abdominal vasculature from anatomic obstructions as can be seen with malignancy or embryologic abnormality. Similarly, impaired venous return from prolonged pressure on the newborn's abdominal cavity during delivery or portal hypertension from portal vein thrombosis following umbilical vein catheterization can result in neonatal hemorrhoids (4). A rectal polyp can masquerade as a hemorrhoid (5). Chronic liver disease is associated with hemorrhoids in up to 35% of children with portal hypertension; however, there are no reported cases of this etiology in neonates ( 6).An ultrasound of the abdomen and pelvis revealed normal hepatic and genitourinary anatomy (7). Doppler waveforms demonstrated hepatopedal flow in the portal vein. A newborn screen was normal.The protuberances self-resolved by 2 weeks of life. Since the infant was thriving, had normal imaging, and had hemorrhoid resolution, no serious underlying pathology was suspected. Neonatal hemorrhoids are a rare and often benign finding that warrants imaging to exclude underlying anatomic anomalies or portal hypertension and close follow-up.