Abstract
Representing only 1-4% of all perinatal brain tumors, Choroid plexus carcinoma (CPC), a WHO grade 3 neuroectoderm-derived lesion, typically presents with symptoms of hydrocephalus as it is often within the lateral ventricles. Surgical resection of these tumors represents the most favorable outcomes. Neuroblastoma originates from neural crest cells and is the most common extracranial solid tumor in children. Often discovered incidentally during abdominal palpation, neuroblastoma is commonly asymptomatic. As both choroid plexus carcinoma and neuroblastoma arise from different embryologic origins, the simultaneous occurrence of both is exceptionally rare and has not been previously reported in published literature. This report presents a case of a 12-month-old male with concurrent choroid plexus carcinoma and adrenal neuroblastoma. The patient presented to the emergency department (ED) after a fall from a crib and had no focal neurological deficits. However, upon arrival, the patient experienced several episodes of emesis. Head computed tomography (CT) revealed a mass in the right lateral ventricle. After transcortical resection of the lesion, postoperative magnetic resonance imaging (MRI) of the neuroaxis unexpectedly demonstrated a left adrenal mass. Further workup revealed urine biomarkers, including vanillylmandelic acid (VMA) and homovanillic acid (HVA), were within normal limits, suggesting a non-aggressive neuroblastoma. Subsequently, a left radical adrenalectomy with lymph node dissection was performed to remove the adrenal mass. After an uneventful, short-term inpatient recovery, patient was discharged and began adjuvant chemotherapy postoperatively. The CPC tumor immunohistochemistry (IHC) was positive for AE1/AE3, CK7, synaptophysin, and CD99. The neuroblastoma showed mutant p53 expression. A high index of suspicion, comprehensive full-neuroaxis diagnostic workup, and coordinated multidisciplinary care are essential to optimal outcomes in cases involving rare tumor combinations. The successful management of both central nervous system and neuroendocrine tumors emphasizes the importance of early detection and personalized therapeutic strategies in pediatric oncology, neuro-oncology, and neurosurgery.