The inflammatory pseudotumor (IPT) is a non-neoplastic entity of unknown origin, and is characterised by a proliferation of connective tissue and a polyclonal inflammatory infiltrate. Central nervous system involvement is uncommon, and usually represents a diagnostic and therapeutic challenge even for the experienced clinician.
Intracranial inflammatory pseudotumors classically involve the cavernous sinus but can also occur in the supratentorial or infratentorial compartments and spinal canal. Symptoms are dependent on location, and, when present in the cavernous sinus, typically include cranial nerve palsies of those nerves in the cavernous sinus. These lesions are rapidly responsive to steroid therapy. Surgery is typically indicated for biopsy only, but complete resection may be justified for lesions outside the cavernous sinus 1).
A 56year-old woman diagnosed with a giant, infiltrating mass centred in the left cavernous sinus, who had a rapid clinical and radiological response to steroid therapy. Biopsy specimens were diagnostic for IPT. The progression of a small orbital residual lesion was detected after steroid withdrawal. Treatment with cyclophosphamide induced a complete response that remains stable after six years of follow-up 2).
A 50-year-old man with progressive left-eye visual disturbance and mass lesion was admitted in a hospital. A left orbital mass biopsy revealed what was highly suspected as an inflammatory pseudotumor. Steroid pulse therapy with dexamethasone, radiation therapy, and chemotherapy with amphotericin B were performed, but they were not effective in improving the condition of the patient. Revision open surgery was then performed. A follow-up brain enhancement computerized tomography showed an enlarged mass volume and hydrocephalus with periventricular enhancement. As an additional procedure, ventriculoperitoneal shunt and tuberculosis medication were administered. About 2 weeks later, clinical symptoms and radiologic findings improved. We present a case of intra-cranial IPT and discuss further treatment methods 3).