Tumors Presenting as Multiple Cranial Nerve Palsies

Abstract

Cranial nerve palsy could be one of the presenting features of underlying benign or malignant tumors of the head and neck. The tumor can involve the cranial nerves by local compression, direct infiltration or by paraneoplastic process. Cranial nerve involvement depends on the anatomical course of the cranial nerve and the site of the tumor. Patients may present with single or multiple cranial nerve palsies. Multiple cranial nerve involvement could be sequential or discrete, unilateral or bilateral, painless or painful. The presentation could be acute, subacute or recurrent. Anatomic localization is the first step in the evaluation of these patients. The lesion could be in the brain stem, meninges, base of skull, extracranial or systemic disease itself. We present 3 cases of underlying neoplasms presenting as cranial nerve palsies: a case of glomus tumor presenting as cochlear, glossopharyngeal, vagus and hypoglossal nerve palsies, clivus tumor presenting as abducens nerve palsy, and diffuse large B-cell lymphoma presenting as oculomotor, trochlear, trigeminal and abducens nerve palsies due to paraneoplastic involvement. History and physical examination, imaging, autoantibodies and biopsy if feasible are useful for the diagnosis. Management outcomes depend on the treatment of the underlying tumor.

Peripheral nervous system involvement in lymphomas

“…Lymphomas may also present with multiple cranial nerve palsies, whose underlying mechanisms consist in malignancy spreading within contiguous nerves or meningeal lymphomatosis . Multiple cranial nerves palsies have also been reported in primary skull base lymphoma, which poses differential diagnosis with other pathological processes involving the skull base, such as meningioma, schwannoma, nasopharyngeal carcinoma, sarcoidosis, granulomatosis with polyangiitis, and carcinomatosis meningitis .…”

Isolated oculomotor nerve palsy as a manifestation of diffuse large B cell lymphoma: A case report

“…A tumor can associate with the cranial nerves locally, via direct infiltration or by a paraneoplastic process ( 6 ). Cranial nerve involvement depends on its anatomical course and the site of the tumor ( 1 ).…”

“…As the patient had pain in the same eye before starting steroids, it would have been highly probable that signs of infiltration would have been identified with a cranial MRI, as this would have justified the pain and may contributed to an earlier diagnosis of the lymphoma. Notably, cranial nerve palsies have been reported even in the presence of normal MRIs ( 5 , 6 ). Intravascular lymphoma, one of the DLBCL subtypes, has a high frequency of nerve infiltration including the CNS ( 4 ), but unfortunately no biopsy was performed in the present case to confirm this possibility.…”

“…This process may precede the actual lymphocytic infiltration by weeks to months ( 1 ). Paraneoplastic syndromes are a class of heterogeneous disorders with diverse presentations caused by an immune response to an underlying malignancy ( 6 ). While rarely associated with Hodgkin's and non-Hodgkin's lymphoma, these often develop into lymphoma during the end stages of the disease ( 6 ).…”

Multiple Cranial Neuropathies in a Patient With Diffuse Large B-cell Lymphoma: Case Report and Review of Literature

“…Cranial nerve palsies occur infrequently in patients with diffuse large B-cell lymphoma (DLBCL) and are seen in only 5% of the DLBCL cases [ 1 ]. DLBCL can affect any part of neuraxis from the brain, leptomeninges, spinal cord to peripheral nerves [ 2 ]. The possible pathogenic mechanism of cranial nerve involvement in DLBCL includes hematogenous spread, direct infiltration, or paraneoplastic phenomenon.…”