Trigeminal ganglion dysfunction secondary to lymphoma

Ear, Nose & Throat Journal, Dec, 2007 by Juan Gomez, Jagan Gupta, Enrique Palacios

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Lymphoma can involve the brain parenchyma and skull base, the orbital contents, and the paranasal sinuses; it may also extend into the cavernous sinus toward the trigeminal ganglion. (1,2) In lymphoma of the central nervous system (CNS), cranial nerve involvement most often affects the trigeminal nerve and trigeminal (gasserian) ganglion. (2,3) The incidence of trigeminal nerve involvement has increased as a result of an increase in the number of immunocompromised patients--primarily, those with acquired immunodeficiency syndrome and those who have undergone organ or bone marrow transplantation. (1,2)

An 18-year-old man with a history of stage IVB T-cell lymphoma presented with fight ear pain and facial paresthesia. His initial cancer diagnosis had included pericardial and bone marrow involvement. Computed tomography (CT) (figure 1) and magnetic resonance imaging (MRI) (figure 2) demonstrated the presence of a mass that involved the right trigeminal ganglion.

Clinically, a patient with lymphomatous involvement of the trigeminal ganglion may present with headaches, trigeminal paresthesias, lacrimation, conjunctival injection, rhinorrhea, ptosis, and diplopia. (4) Cluster headaches may develop secondary to autonomic activation of the trigeminal vascular and cranial parasympathetic pathways. (4) Trigeminal ganglion involvement may affect the ophthalmic and maxillary division of the trigeminal nerve and lead to motor and sensory impairment of the mandibular division, suggesting peripheral extension from the ganglion. (3)

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Other conditions that might exhibit imaging and clinical manifestations similar to those of CNS lymphoma include nasopharyngeal carcinoma (either as a direct extension or as a metastasis), paraganglioma, granulomatous disease, histiocytosis, schwannoma, and vascular malformation. (3) The proximity of the trigeminal ganglion to the cavernous sinus and venous plexus in the intradural space supports hematogenous and lymphatic spread when there is lymphomatous involvement of the trigeminal ganglion. (2,3)

The optimal imaging modalities for visualizing the trigeminal ganglion and perineural spread of lymphoma are contrast-enhanced CT and MRI. These studies will demonstrate any bony involvement of the skull base, as occurred in the case described here. (3,5) Histologic confirmation of the diagnosis can be obtained with a CT-guided biopsy. (5) Recently, fusion imaging with positron-emission tomography and CT has been used to demonstrate neoplastic processes, including lymphomatous processes of the base of the skull, and to enhance post-treatment follow-up assessments. (6)

References

(1.) Arimoto H, Shirotani T, Nakau H, et al. Primary malignantlymphoma of the cavernous sinus--case report. Neurol Med Chir (Tokyo) 2000;40(5):275-9.

(2.) Hevner R, Vilela M, Rostomily R, et al. An unusual cause of trigeminal-distribution pain and turnout. Lancet Neurol 2003;2(9): 567-71.

(3.) Williams LS, Schmalfuss IM, Sistrom CL, et al. MR imaging of the trigeminal ganglion, nerve, and the perineural vascular plexus: Normal appearance and variants with correlation to cadaver specimens. AJNR Am J Neuroradiol 2003;24(7): 1317-23.

(4.) Palmieri A. Mainardi E Maggioni F, et al. Cluster-like headache secondary to cavernous sinus metastasis. Cephalalgia 2005;25(9): 743-5.

(5.) Donnet A, Moulin G, Tubiana N, et al. Lymphomatous meningitis: Neuroradiological appearances. Neuroradiology 1992;34(5): 411-12.

(6.) Rodriguez MA, Bseiso AW. Lymphomas of the head and neck. In: Harrison LB, Sessions RB, Hong WK, eds. Head and Neck Cancer: A Multidisciplinary Approach. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2004:899-918.

Juan Gomez, MD; Jagan Gupta, MD; Enrique Palacios, MD, FACR

From the Department of Radiology, Tulane University Medical Center, New Orleans.