Mantle Cell Lymphoma Presenting with Primary Neurolymphomatosis and Cutaneous Involvement: A Case Report

Abstract

Abstract Introduction: Neurolymphomatosis (NL) is an extremely rare manifestation of lymphoproliferative and hematologic malignancies characterized by direct neoplastic infiltration of the peripheral nervous system, and ubiquitously portends poor outcomes with dismal survival. This challenging and infrequent clinical entity is most commonly associated with B-cell non-Hodgkin lymphomas (NHLs), with only a few cases of NL as a presentation of mantle cell lymphoma (MCL) ever reported. The heterogenous clinical features of NL overlap with several more common neurologic sequalae of NHL, a challenge which often leads to delays, and even misdiagnosis, of this aggressive condition. As a diagnostically challenging, rapidly progressive rare disease, there is a paucity of evidence describing the natural clinical course of NL, diagnostic algorithms, prognosis, patient outcomes data, and most crucially, optimal therapeutic management strategies. Aggressive lymphomas may also infiltrate the skin causing secondary cutaneous B-cell lymphoma, an exceptionally rare manifestation of MCL. To the authors’ knowledge, this is the first reported case in the literature describing a patient with MCL presenting with primary neurolymphomatosis and secondary cutaneous MCL. Case presentation: A 73-year-old man recently diagnosed with MCL developed painless, ascending weakness, which progressed over weeks to near-complete paralysis. MRI of the lumbar spine was consistent with neurolymphomatosis. Concurrently, he developed erythematous papules and plaques on the face, chest, and proximal upper extremities. Skin biopsy was consistent with cutaneous infiltration of MCL. Treatment with HD-MTX plus R-CHOP therapy produced minimal improvement, and he died three weeks after initiation of therapy. Conclusions Neurolymphomatosis and secondary cutaneous MCL are each very rare manifestations of MCL. Rapid, aggressive infiltration of both the nerve roots and skin by MCL represents unusually high-grade disease. This case underscores the importance of high clinical suspicion for neurolymphomatosis in any subtype of lymphoma showing aggressive features. Prognosis is poor, and early intervention is crucial to minimize progression.