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Journal of Clinical Oncology, Vol 26, No 4 (February 1), 2008: pp. 678-679 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.14.4964
Primary CNS Lymphoma Presenting With Acute Blindness and EncephaloceleDepartment of Neurosurgery, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea
Department of Pathology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea A 25-year-old man was transferred to the Department of Neurosurgery, Seoul National University Hospital (Seoul, Korea) with suspected idiopathic intracranial hypertension. He presented with rapidly progressing bilateral visual impairment, without headache. He had suffered an episode of febrile illness 1 month before his visual dysfunction. Physical and neurologic examinations revealed no abnormality, except his blindness. An ophthalmologic examination showed a macular dot hemorrhage with suspected optic atrophy. However, there was no evidence of overt papilledema or other abnormalities on either the retina or optic vasculature. There was no evidence of immunosuppression, HIV, or Epstein-Barr virus infections on serology tests. Brain magnetic resonance images (MRI) revealed herniation of the left frontal lobe into the frontal sinus, with erosion of the posterior wall of the frontal sinus (Figs 1A, 1B, and 1C; yellow arrows) and a slight high signal intensity lesion in the left frontal base on T2-weighted images without enhancement (Figs 1A and 1B; blue arrow). Only subtle enhancement could be found in the herniated area of the brain and the adjacent meninges (Fig 1B; white arrow). Because there had been no such lesion on brain MRI of the patient that had been taken 7 years earlier, or any subsequent history of trauma, this lesion was believed to be a recently developed spontaneous lesion. A repeated lumbar puncture was performed, and the opening pressure was consistently as high as 50 cm H2O. The CSF profile disclosed 33/µL mononuclear elements. However, no malignant cells were observed cytologically in the CSF. No possible factors contributing to the increase in CSF pressure were found in clinical and radiologic reviews. Suspecting a variant of idiopathic intracranial hypertension manifesting only as a visual disturbance, we performed repeated CSF drainage, administered acetazolamide medication, and performed optic nerve sheath fenestration, which resulted in no improvement in the patient's vision. Subsequently, a bifrontal craniotomy with cranialization of the frontal sinus was performed as a prophylactic measure against cerebral infection from the frontal sinus. Herniated brain into frontal sinus was excised (Fig 2A) and dural repair was done. Contrary to our expectations, the pathology report of the brain tissue revealed a diffuse large B-cell lymphoma (Fig 2B, hematoxylin and eosin stain; original magnification x200). On immunohistochemical staining, the phenotype of the tumor cells was CD5– and CD20+ (Fig 2C). Seven days after surgery, the patient showed sudden aphasia, with confusion. Brain MRI revealed aggravation of the high-intensity lesion in the left frontal lobe on T2-weighted images, still without enhancement (Fig 3A; blue arrow), and a small amount of subdural fluid had collected (Figs 3A and B; yellow arrows). After two cycles of high-dose methotrexate therapy followed by 50.4 Gy of radiation therapy, the patient recovered fully from his neurologic deterioration, except for his vision, and radiologic remission was also achieved.
Presentation of a primary CNS lymphoma (PCNSL) with acute visual loss, encephalocele, and a nonenhanced lesion is extremely rare. On MRI of the PCNSL, contrast enhancement is strong in 85% of patients, moderate in 10% of patients, and absent in 1% of patients.1 Neuroophthalmologic abnormalities in these lymphomas are usually attributed to their direct invasion of orbital structures.2 However, bilateral visual loss with no evidence of involvement of the optic apparatus in the lymphoma must be explained by another mechanism. A possible mechanism involves reactive optic arachnoiditis, which can develop adjacent to a skull-base lymphoma or a leptomeningeal lymphoma.2,3 A leptomeningeal lymphoma can develop in a localized form, with negative CSF cytologic results and no clinical signs.4 In the present case, it is postulated that leptomeningeal involvement of the PCNSL resulted in the erosion of the posterior wall of the frontal sinus and an encephalocele developed through the bony defect. Atypical clinical, radiologic, and laboratory presentations of a lymphoma can delay its accurate diagnosis, as in this case. Although it is rare, a disease process like this case may occur in other locations in the skull in PCNSL. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES
1. Batchelor T, Loeffler JS: Primary CNS lymphoma. J Clin Oncol 24:1281-1288, 2006 2. Güler E, Kutluk T, Akalan N, et al: Acute blindness as a presenting sign in childhood non-Hodgkin lymphoma. J Pediatr Hematol Oncol 25:69-72, 2003[CrossRef][Medline] 3. Sakai C, Takagi T, Wakatsuki S: Primary meningeal lymphoma presenting solely with blindness: A report of an autopsy case. Int J Hematol 63:325-329, 1996[CrossRef][Medline] 4. Balmaceda C, Gaynor JJ, Sum M, et al: Leptomeningeal tumor in primary central nervous system lymphoma: Recognition, significance, and implications. Ann Neurol 38:202-209, 1995[CrossRef][Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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