Baylor University Medical Center Proceedings October 2017 - 456

CLL are heterogeneous and include
headache, cranial nerve palsies, cerebellar signs, visual problems, and
motor and/or sensory deﬁcits. Imaging studies are neither speciﬁc nor
sensitive in the detection of CNS
involvement; the diagnosis is usually conﬁrmed by lumbar puncture.
At present, there are no established
guidelines for treatment of CLL
patients with CNS involvement.
Most patients have been treated
with intrathecal chemotherapy
with or without radiation therapy
or systemic chemotherapy (1). Intrathecal rituximab has been found
Figure. MRI of the brain with contrast, (a) axial view and (b) coronal view, showing asymmetric enhancing thickening to be eﬀective in aggressive B-cell
lymphomas; however, its eﬃcacy in
in the left cavernous sinus (arrow).
CLL has not been assessed (9). For
CLL patients with leptomeningeal disease, ﬂudarabine-based
developed sepsis during the neutropenic period. He died on
therapy has been found to be eﬀective and may be a favorable
postchemotherapy day 12.
therapeutic option (10). In our patient, a combination of ﬂudarabine, cyclophosphamide, and rituximab (FCR regimen) was
DISCUSSION
very toxic, and he succumbed to sepsis during the neutropenic
Cavernous sinus syndrome is characterized by ophthalperiod.
moplegia and sensory deﬁcits over the head due to combined
deﬁcits of the three cranial nerves (third, fourth, and sixth)
1. Lopes da Silva R. Spectrum of neurologic complications in chronic lymresponsible for eye movements and pupil function, and at least
phocytic leukemia. Clin Lymphoma Myeloma Leuk 2012;12(3):164-179.
one branch of the trigeminal nerve. The wide-ranging types of
2. Nakatomi H, Sasaki T, Kawamoto S, Fujimaki T, Furuya K, Kirino T.
pathologies that involve the cavernous sinus can be classiﬁed as
Primary cavernous sinus malignant lymphoma treated by gamma knife
tumoral, congenital, infectious, inﬂammatory, granulomatous,
radiosurgery: case report and review of the literature. Surg Neurol
and vascular.
1996;46(3):272-278.
3. Arimoto H, Shirotani T, Nakau H, Hashizume K, Sakai Y, Matsukuma S.
Among the tumors involving the cavernous sinus, head and
Primary malignant lymphoma of the cavernous sinus-case report. Neurol
neck tumors are the most likely to metastasize to the cavernous
Med Chir (Tokyo) 2000;40(5):275-279.
sinus. The other common primary sites in patients with cavern4. Sadruddin S, Medeiros LJ, DeMonte F. Primary T-cell lymphoblastic
ous sinus metastases are breast, lung, and prostate. Lymphomas
lymphoma of the cavernous sinus. J Neurosurg Pediatr 2010;5(1):94-97.
have been reported to involve the cavernous sinus, as primary
5. Ceyhan M, Erdem G, Kanra G, Kaya S, Onerci M. Lymphoma with
bilateral cavernous sinus involvement in early childhood. Pediatr Neurol
lymphomas (2-4) or as secondary lesions, and may occur as
1994;10(1):67-69.
unilateral or bilateral lesions (5, 6). Lymphomas may involve
6. Huisman TA, Tschirch F, Schneider JF, Niggli F, Martin-Fiori E, Willi
the cavernous sinus as a result of invasion or metastasis origiUV. Burkitt's lymphoma with bilateral cavernous sinus and mediastinal
nating in the head and neck region, or metastasis of systemic
involvement in a child. Pediatr Radiol 2003;33(10):719-721.
origin. Burkitt lymphoma (6), diﬀuse large B-cell lymphoma
7. Hirano H, Tashiro Y, Fujio S, Goto M, Arita K. Diﬀuse large B-cell
lymphoma within a cavernous hemangioma of the cavernous sinus. Brain
(7), T-cell lymphoblastic lymphoma (4), and diﬀuse small B-cell
Tumor Pathol 2011;28(4):353-358.
lymphoma (8) have all been reported as primary lymphoma and
8. Delpassand ES, Kirkpatrick JB. Cavernous sinus syndrome as the presenas metastases in the cavernous sinus, but to our knowledge CLL
tation of malignant lymphoma: case report and review of the literature.
involvement has never been reported in the literature. Infectious
Neurosurgery 1988;23(4):501-504.
causes such as fungus and tuberculosis were considered because
9. Rubenstein JL, Fridlyand J, Abrey L, Shen A, Karch J, Wang E, Issa S,
of the immunocompromised status of our patient. However,
Damon L, Prados M, McDermott M, O'Brien J, Haqq C, Shuman M.
Phase I study of intraventricular administration of rituximab in paan infectious cause was less likely in our patient, in view of the
tients with recurrent CNS and intraocular lymphoma. J Clin Oncol
presence of CLL cells in the CSF. Moreover, the CSF culture
2007;25(11):1350-1356.
was negative.
10. Knop S, Herrlinger U, Ernemann U, Kanz L, Hebart H. Fludarabine may
CNS involvement of CLL remains a poorly studied pheinduce durable remission in patients with leptomeningeal involvement of
nomenon. The clinical manifestations of CNS involvement in
chronic lymphocytic leukemia. Leuk Lymphoma 2005;46(11):1593-1598.
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