Intravascular lymphoma can present with neuro-ophthalmic findings

This is the first reported case of intravascular lymphoma (IVL), a rare subtype of large-cell non-Hodgkin lymphoma involving the pituitary gland and presenting with neuro-ophthalmic findings.

A 68-year-old female presented with headache, right third nerve palsy and Horner syndrome. MRI showed a 1.4-cm sellar mass consistent with a pituitary macroadenoma. Two weeks later, despite treatment with dexamethasone, the patient developed complete bilateral ophthalmoplegia and ptosis. After repeat MRI showing invasion of the clivus and cavernous sinuses, the patient underwent trans-sphenoidal pituitary biopsy.

The preliminary histopathology was consistent with bland pituitary apoplexy, but subsequent examination of an incidentally biopsied nasal polyp revealed endovascular malignant lymphoid cells that, on further scrutiny, were also present in the pituitary tissue. The diagnosis of IVL was confirmed, and the patient had an excellent clinical and radiological response to cyclophosphamide, doxorubicin, vincristine, prednisolone and rituximab (CHOP-R) chemotherapy.