Use of preoperative endoscopic therapy remains common and has resulted in more intraoperative complications, primarily perforation, more postoperative complications, and a higher rate of failure than when no preoperative therapy was used. Endoscopic therapy for achalasia should not be used unless patients are not candidates for surgery.

Adolescent-aged patients are less likely to be referred to a COG institution, potentially exposing them to worse outcomes in some cancer subtypes. Reassuringly, minority populations are receiving adequate access to pediatric cancer care; unfortunately their survival rates are lower.

Background-Wallstents (Schneider Stent, Inc., USA) used for the palliation of malignant biliary strictures, although associated with prolonged patency, can occlude. There is no consensus regarding the optimal management of Wallstent occlusion. Aims-To evaluate the eYcacy of different endoscopic methods for managing biliary Wallstent occlusion. Methods-A multicentre retrospective study of patients managed for a biliary Wallstent occlusion. Results-Data were available for 38 patients with 44 Wallstent occlusions, all of which had initial endoscopic management. Twenty four patients had died and 14 were alive after a median follow up of 231 (30-1095) days following Wallstent occlusion. Occlusions were managed by insertion of another Wallstent in 19, insertion of a plastic stent in 20, and mechanical cleaning in five. Endoscopic management was successful in 43 (98%). Following management of the occlusion, bilirubin decreased from 6.0 (0.5-34.3) to 2.1 (0.2-27.7) mg/100 ml (p<0.05). No complications occurred. The median duration of second stent patency was 75 days (95% confidence interval 43 to 107) after insertion of another Wallstent, 90 days (71 to 109) after insertion of a plastic stent, and 34 days (30 to 38) after mechanical cleaning (NS). The respective median survivals were 70 days (22-118), 98 days (54-142), and 34 days (30-380) (NS). Incremental cost eVective analysis showed that plastic stent insertion is the most cost eVective option. Conclusion-Although all three methods are equally eVective in managing an occluded Wallstent, the most cost eVective method appears to be plastic stent insertion.

Neuroendocrine tumors, often referred to as carcinoid tumors, are relatively rare within the pediatric and young adult populations. However, when they do occur, the more aggressive tumors can be associated with significant morbidity and even mortality in this younger age group. This article reviews the history of pediatric neuroendocrine tumors, typical clinical presentation, appropriate diagnostic studies, staging, and treatment of this unusual cancer.

Epstein–Barr virus–associated smooth‐muscle tumors (EBV‐SMTs) are unique and rare neoplasms described in immunocompromised patients. The case describes a nine‐year‐old female with a history of acute lymphoblastic leukemia with relapse and subsequent allogeneic bone marrow transplantation who presented with multiple EBV‐SMTs of the liver. EBV utilizes the mammalian target of rapamycin (mTOR) pathway for tumor growth, and sirolimus, a mTOR inhibitor, has shown to result in a short‐term response. We now report an extended treatment response with sirolimus in a pediatric patient with an EBV‐SMT.

Prolactinomas are rare in children and adolescents but well studied in adults. Dopamine agonists are the treatment of choice for all ages. Bromocriptine is the only agonist approved for use in pediatric patients by the FDA. Cabergoline, a second-generation ergot derivative with a longer half-life, has been used in resistant prolactinomas and as first-line treatment in adults. The authors describe an adolescent boy with a pituitary macroadenoma with an initial prolactin level of 73,777 ng/mL. After failing to respond to bromocriptine and standard-dose cabergoline, he responded well to very high daily doses of cabergoline (1.5 mg daily), with a current prolactin level of 726 ng/mL and notable reduction in tumor size. Escalating doses of cabergoline should be considered in pediatric patients with dopamine-resistant prolactinomas.

No abstract

Hodgkin's disease, one of the more common tumors in pediatric oncology, has been shown to be highly responsive to steroids in conjunction with other chemotherapy agents. The authors describe a patient with Cushing's syndrome that suppressed his Hodgkin's disease. He developed constitutional symptoms, and his Hodgkin's disease progressed after the removal of his pituitary tumor. The patient is disease-free following ABVD/MOPP chemotherapy. A high level of steroids before treatment, albeit endogenous, did not preclude successful treatment of Hodgkin's disease. The effect of Cushing's syndrome on Hodgkin's disease has not been previously reported.