Progressive neurolymphomatosis with cutaneous disease: Response in a patient with mycosis fungoides

Abstract

Peripheral neurolymphomatosis is a rare manifestation of advanced lymphoproliferative disorders. It is often associated with B cell lymphomas and rarely with cutaneous T cell lymphomas, such as mycosis fungoides and Sézary syndrome. In this case report, we present a 78-year-old male with a long-standing history of mycosis fungoides. The patient initially presented with chronic peripheral neuropathy in an ulnar nerve distribution. After an unsuccessful ulnar nerve transposition, the ulnar nerve was re-explored and a mass consistent with diffuse lymphomatous infiltration was diagnosed. Magnetic resonance (MR) imaging of the left brachial plexus and later of the sacral plexus demonstrated diffuse thickening and peripheral nodularity in keeping with neurolymphomatosis. The patient's clinical course rapidly deteriorated thereafter and the patient succumbed to his disease. Although uncommon, neurolymphomatosis may be considered in patients with chronic peripheral neuropathy and an underlying history of a lymphoproliferative disorder. US and MR may serve as helpful non-invasive adjuncts in making the diagnosis and identifying sites for biopsy.

Putative mechanisms for spread and transformation of cutaneous T‐cell lymphoma to neurolymphomatosis

“…Several cases of NL arising in patients with T‐cell lymphoma have been reported, but none have demonstrated direct epineural invasion by cells in the subcutaneous tissues, despite disease present in subcutaneous tissues overlying a relatively superficial nerve . In a case reported by Hanna et al, the patient described developed high ulnar neuropathy, as well as median neuropathies localized to the wrists, in the setting of multiple skin plaques of the upper extremities. It is plausible that their patient developed upper extremity NL via direct spread from involved subcutaneous tissues.…”

“…Mycosis fungoides is an erythrodermic variant of T‐cell lymphoma, progressing over years with cutaneous plaques, patches, and nodules caused by clonal T cells invading the skin . Spread to the nervous system is rare (2%‐14% of cases), but most commonly manifests with spread to the leptomeninges, and less often as neurolymphomatous involvement of the peripheral nerves …”

Neuropathies associated with lymphoma†

“…In light of some promising case reports, larger clinical studies with this technique should be initiated Figure 3. 33,34…”