Introduction

The object of this review is to describe the choroid plexus tumors (CPTs) occurring in the fetus and neonate with regard to clinical presentation, location, pathology, treatment, and outcome.

Materials and methods

Case histories and clinical outcomes were reviewed from 93 cases of fetal and neonatal tumors obtained from the literature and our own institutional experience from 1980 to 2016.

Results

Choroid plexus papilloma (CPP) is the most common tumor followed by choroid plexus carcinoma (CPC) and atypical choroid plexus papilloma (ACPP). Hydrocephalus and macrocephaly are the presenting features for all three tumors. The lateral ventricles are the main site of tumor origin followed by the third and fourth ventricles, respectively. CPTs of the fetus are detected most often near the end of the third trimester of pregnancy by fetal ultrasound. The extent of surgical resection plays an important role in the treatment and outcome. In spite of excellent survival, which is especially true in the case of CPP, surgical resection may carry significant risks in an immature baby. Given the neonatal low blood volume and increased vascularity of the tumors, there is potential risk for hemorrhage. Although advances in neurosurgical techniques have led to a greater degree of complete surgical resections, survival for the perinatal CPC group remains low even with multimodality therapies.

Conclusion

Perinatal CPTs have variable overall survivals depending on degree of surgical resection and tumor biology. An increased understanding of the molecular features of these tumors may lead to improved therapies and ultimately survival.

Abstract Background Hypoxia inducible factor-1 (HIF-1) is a major regulator of the cellular adaption to low oxygen stress and the innate immune function of myeloid cells. Treatment with the novel HIF-1 stabilizing drug AKB-4924 has been shown to enhance the bactericidal activity of keratinocytes as well as phagocytic cells. In this study, we sought to investigate the effect of pharmacological boosting of HIF-1 with AKB-4924 in keratinocytes and their contribution to the innate immune response. Findings Treatment with the novel HIF-1 stabilizing drug AKB-4924 can increase keratinocyte production of pro-inflammatory cytokines in vitro and enhance neutrophil recruitment in vivo. Conclusions HIF plays an important role in cytokine production by keratinocytes and in neutrophil recruitment to the skin. The HIF-boosting drug AKB-4924 has the potential to enhance the immune response even in the complex environment of bacterial skin infections.