Primary granulocytic sarcoma in the sphenoidal bone and orbit

We report a case of a primary cranial chloroma in boy aged 2 years and 8 months. The symptoms were progressive bilateral exophthalmos, right abducens palsy, and bilateral papilledema. The tumor was partially calcified and was a round mass located in the bilateral sphenoidal bone extending into the orbit. Laboratory study did not show hematological abnormality. The tumor was partially removed by bilateral frontotemporal craniotomy and a diagnosis of primary granulocytic sarcoma was made from the surgical specimen. Progressive deterioration of visual acuity was seen and chemotherapy started on the 11th postoperative day followed by local cranium irradiation (24 Gy). The patient has been in complete remission for 37 months. The visual acuity recovered partially and follow-up magnetic resonance imaging showed a significant decrease in the size of the tumor.

Radiological diagnosis of primary intracranial granulocytic sarcoma is difficult. Surgical removal may be an option for progressive neurological deterioration but chemotherapy is more important for both neurological stabilization and induction of remission.