CNS Lymphoma

The patient is a 70 year old Italian man with atrial fibrillation on long-term coumadin therapy. In October 1995, he developed generalized headache, horizontal double vision and his left eye deviated inwards (esotropia). A diagnosis of left sixth nerve palsy was made and attributed to microvascular disease. In November 1995, a brain MRI showed "multiple lacunes". In December 1995, in addition to the left esotropia, the right eye deviated in and his double vision was worse. MRI Brain reviewed by his PCP showed no intracranial mass lesion(s). He was referred for a neuro-ophthalmic opinion. Ocular motility examination: Bilateral esotropia OS > OD With the right eye fixing alone, partial right sixth nerve palsy. With the left eye fixing alone, complete left sixth nerve palsy. Right 3rd and 4th cranial nerve intact Left 3rd and 4th cranial nerve intact Corneal reflexes normal Mild bilateral age related ptosis The remainder of the cranial nerves were intact Brain CT with contrast: 1.Fullness of the cavernous sinus bilaterally, especially on the right 2.Fullness of the sella tursica and abnormal soft-tissue along the dorsum of the upper clivus 3.Opacification of the right sphenoid sinus and thinning and irregularity of its posterior-superior wall Impression: In the setting of bilateral sixth nerve palsies these findings are consistent with invasive aspergillosis or metastatic disease. Chest CT: 1 cm. paratracheal, precarinal and AP window lymph nodes Small (5 mm) right retrocrural and left hilar nodes also present. Abdominal CT: 4 cm. heterogeneous left adrenal mass suspicious for neoplasm Biopsy of soft tissue in the sella and parasellar region was performed by a right ethmoid approach. Pathology: Non-Hodgkin's lymphoma Additional studies: Left adrenal enlargement secondary to hemorrhage Oncology therapy: Intensive CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy for six cycles. Radiation therapy: Radiation therapy to the skull base. April 1996 four months post biopsy: Complete recovery of the right sixth nerve palsy Mild esotropia OS with a partial left sixth nerve palsy and diplopia at distance. No headache By August 1996: Symptom free with no diplopia Complete recovery of the left sixth nerve palsy In September 1998 he was in good health and had a normal examination. Comment: Aggressive non-Hodgkin's lymphomas (NHLs) are characterized by effacement of lymph node architecture with a diffuse infiltration of large lymphocytes and include the diffuse large B-cell lymphoma (31% of NHL), and anaplastic large cell lymphoma (2% of NHL). The outcome and likelihood of cure in patients with diffuse aggressive NHL is directly related to the total number of adverse prognostic features that are present at presentation. Adverse prognostic features include: 1.Age older than 60 2.Advance stage (III or IV), 3.Elevated LDH levels and 4.The presence of 2 or more extranodal sites of disease. The likelihood of cure and long-term disease-free survival ranges from more than 75% in patients with one or fewer adverse factors (as in this patient) to less than 30% in patients with 4 or more adverse factors. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/20