Pediatric cerebellar pilocytic astrocytoma presenting with spontaneous intratumoral hemorrhage.

ABSTRACT

Pilocytic astrocytomas (PAs) are benign glial tumors and one of the most common childhood posterior fossa tumors. Spontaneous intratumoral hemorrhage in PAs occurs occasionally, in about 8-20% of cases. Cerebellar hemorrhages in pediatric population are rare and mainly due to head injuries, rupture of vascular malformations, infections, or hematological diseases. We have investigated the still controversial and unclear pathophysiology underlying intratumoral hemorrhage in PAs. Bleeding in low-grade tumors might be related to structural abnormalities and specific angio-architecture of tumor vessels, such as degenerative mural hyalinization, "glomeruloid" endothelial proliferation, presence of encased micro-aneurysms, and glioma-induced neoangiogenesis. The acute hemorrhagic presentation of cerebellar PA in childhood although extremely uncommon is of critical clinical importance and necessitates promptly treatment. We described a case of hemorrhagic cerebellar PA in a 9-year-old child and reviewed the English-language literature that reported spontaneous hemorrhagic histologically proven cerebellar PA in pediatric patients (0-18 years). According to our analysis, the mortality was not related to symptom onset, tumor location, hemorrhage distribution, presence of acute hydrocephalous, and timing of surgery, while the GCS at hospital admission resulted to be the only statistically significant prognostic factor affecting survival outcome. The abrupt onset of signs and symptoms of acute hydrocephalous and consequent raised intracranial pressure are life-threatening conditions, which need emergent medical and neurosurgical treatments. At a later time, the identification of posterior fossa hemorrhage etiology is crucial to select the appropriate treatment and address the surgical strategy, optimizing the postoperative results.