Adrenal Suppression in Children During the Treatment for Acute Lymphoblastic Leukemia Beyond Induction.

BACKGROUND: Adrenal suppression (AS) is an iatrogenic, life-threatening condition that can occur after glucocorticoid exposure. Despite recognition that AS occurs after induction phase treatment in children with acute lymphoblastic leukemia (ALL), the risk of AS in phases beyond induction is unknown. We conducted a pilot study in pediatric patients with ALL to ascertain whether the risk of AS persists in post-induction phases of treatment. PROCEDURE: Patients diagnosed between 12 months to younger than 18 years with B or T-ALL and starting any new phase of treatment were eligible for the study. Relapsed or infant ALL were excluded. Low dose ACTH stimulation testing (LDST), measurement of albumin and cortisol binding globulin were performed in all patients. Screening for symptoms of AS was done. RESULTS: Twenty-four patients enrolled in the study. One was diagnosed with clear AS. Five others had a borderline cortisol peak, representing possible mild AS. Symptoms were nonspecific and did not help distinguish patients with normal LDST from those with borderline or abnormal results. CONCLUSION: Patients on treatment for ALL continue to be at risk of AS beyond induction treatment. Although this risk appears small, physicians must be vigilant as patients may be asymptomatic but could develop adrenal crisis during treatment.

How well do image-defined risk factors (IDRFs) predict surgical outcomes and survival in patients with neuroblastoma? A systematic review and meta-analysis.

INTRODUCTION: Since their introduction to the International Neuroblastoma Risk Group (INRG) staging system in 2009, the role of Image-Defined Risk Factors (IDRFs) in predicting outcomes has been studied in heterogeneous populations with varying results. We conducted a systematic review and meta-analysis in order to determine quantitative measures of precisely how well pre-treatment IDRFs predict surgical outcomes and survival. METHODS: A systematic review was performed for studies between January 1990 and July 2019 that compared surgical outcomes and/or survival in pediatric neuroblastoma patients with one or more IDRFs to patients without IDRFs. Summary risk ratios (RR) and hazard ratios (HR) were calculated using a random-effects model. RESULTS: 19 retrospective cohort studies were included, representing data from 1132 patients. The risk ratio (RR) of incomplete surgical resection in IDRF-positive patients compared to IDRF-negative patients was 2.45 (95% CI 1.51-3.97). The RR of surgical complications was 2.30 (95% CI 1.46-3.61). The hazard ratio (HR) for 5-year EFS was 2.08 (95% CI 2.93-4.13) while the 5-year HR for OS was 2.44 (95% CI 1.46-4.08). CONCLUSION: IDRF-positive neuroblastoma patients have a higher risk of incomplete surgical resection, surgical complications, and 5-year mortality and/or relapse. Our results affirm that IDRFs remain a useful prognostic tool for neuroblastoma patients both for short and long-term outcomes. LEVEL OF EVIDENCE: II.

Prenatally Diagnosed Infant AML.

We report the first case of a fetus with acute myeloid leukemia, without Down syndrome, diagnosed in utero. A cordocentesis sample prepared to investigate hepatomegaly led to further evaluations revealing acute myeloid leukemia, monocytic type, in the fetus. Cytogenetic analysis showed mixed lineage leukemia duplication, no gene disruption or trisomy. Planned treatment included intrauterine exchange transfusion to extend gestation, low-dose chemotherapy at birth, and full chemotherapy once stable. Before any intervention, the child was delivered emergently for maternal condition and died 2 hours later. Although it is now possible to diagnose hematologic malignancy in a fetus, there is little information to direct management.