Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor of perivascular differentiation, characterized by prominent vascularity and heterogeneous clinical behavior in neonates. Prenatal diagnosis, particularly of intracranial lesions, is exceptional and poses significant diagnostic and management challenges. This study offers insights into diagnosing and managing this condition in a young patient, enriched by a systematic review of the literature over the past 15 years. We report a fetus diagnosed prenatally with a 5 cm highly vascular intracranial mass detected on routine third‐trimester ultrasound (US) and also characterized by fetal magnetic resonance imaging (MRI). Further prenatal assessment showed cardiomegaly. The pregnancy was managed by a multidisciplinary team with a planned cesarean section performed at 35 weeks of gestation. The neonate was born in good general condition. Postnatal biopsy confirmed the diagnosis of HPC, and a ventriculoperitoneal shunt was placed to manage hydrocephalus. The infant subsequently received neoadjuvant chemotherapy (CHx) to reduce tumor size and vascularity. Follow‐up imaging showed a reduction in the tumor size. At 4 months of age, the neonate was readmitted due to newly identified osteolytic lesions along the spine. In the context of suspected multifocal disease and partial response to CHx, a neurosurgical excision of the intracranial mass was performed. At 1‐year follow‐up, no evidence of tumor progression or new lesion development was observed, and neurological development was normal. In conclusion, this case highlights the importance of early prenatal diagnosis and multidisciplinary management in congenital intracranial HPC, supporting the feasibility of a combined CHx and surgical approach. Further research is needed to refine management strategies and establish standardized follow‐up protocols for this neonatal tumor.
Lettieri et al. (2026) studied this question.