Rare Case of Skull Base Infantile Hemangiopericytoma in 4 Year-Old Male

Introduction: The patient is a 4 year old Hispanic male who developed headaches and ataxia while being treated for his pre B- cell acute lymphoblastic leukemia and was diagnosed with a large right cerebellopontine angle skull base mass that extended superiorly to involve Meckel's cave in the middle fossa. He was also found to have elevated parathyroid hormone levels.

Methods: The patient underwent staged resections, first via a right retrosigmoid approach during which cranial nerves III, IV, VI, VII, VIII and lower cranial nerves were all identified and preserved. He then underwent a right frontotemporal craniotomy three weeks later to resect the portion of the mass adjacent to Meckel's cave and near the midbrain. Gross total resection was achieved. The patient's parathyroid hormone levels and hypercalcemia resolved after the first resection although the precise mechanism for this remains unclear. He did develop post operative meningitis which was treated with IV antibiotics. Subsequent follow up MRIs have continued to show no residual or recurrent tumor 22 months out. He completed therapy for his ALL.

Results: Pathology was consistent with infantile hemangiopericytoma with hypercellularity, spindle shaped cells, and characteristic staghorn vasculature and positivity for CD 34. Only a few cases of intracranial infantile hemangiopericytoma (HPC), have been reported in the literature. When occurring in infants (usually under the age of one year of age), infantile HPCs are usually extracranial, most often involving the trunk and extremities and are characterized by benign behavior. There have been reports of congenital intracranial hemangiopericytomas that had significant reductions in size from chemotherapy alone. Some infantile hemangiopericytomas have been reported to involute without treatment. In older children, infantile HPCs can behave in a more aggressive manner and as such gross surgical resection favored. This case, with intracranial infantile HPC in a child being treated for coexisting ALL (now in remission) is extremely rare and may be the first reported in neurosurgical literature.

Conclusion: Intracranial infantile hemangiopericytoma is a very rare tumor type, especially in an older child. A case of infantile HPC with coexisting ALL has not been reported before in the neurosurgical literature to our knowledge.

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