Aggressive Mantle Cell Lymphoma with Neurolymphomatosis and Cutaneous Infiltration: First Case Report and Review of Literature

Mantle cell lymphoma accounts for 5-10% of cases of non-Hodgkin's lymphoma. Neurolymphomatosis(NL) and Cutaneous involvement of mantle cell lymphoma has been rarely described. When present, they are indicators of high-grade disease and poor prognosis. We present the first reported case of Neurolymphomatosis and concurrent cutaneous mantle cell lymphoma.

A 73-year-old man with a recent diagnosis of mantle cell lymphoma presented with progressive weakness of the upper and lower extremities that began one month after his first round of chemotherapy. The Dermatologic exam revealed red-violaceous, edematous papules and plaques on the head, trunk, and upper extremities. Prior work-up including MRI brain and cervical spine, lumbar puncture and cytology were unremarkable. He received treatment with dexamethasone and PLEX with no improvement. MRI lumbar spine revealed abnormal linear and nodular contrast enhancement in the dural sac along the lumbosacral nerves with associated soft tissue thickening in epidural spaces, consistent with NL. Skin biopsy showed medium to large cells with atypical nuclei throughout the dermis. Immunohistochemical stains for CD20, CD5, and cyclin D1 were positive, while CD3 and CD23 were negative. These findings were consistent with cutaneous mantle cell lymphoma. MR-CHOP therapy was promptly initiated however the patient developed pancytopenia with worsening swallowing dysfunction and died of aspiration pneumonia and sepsis.

We identified 13 cases of Mantle cell Neurolymphomatosis in the literature, including our case. Mean age on presentation was 57.5 with male predominance. Six cases had neuronal involvement on primary presentation. Most common symptom was painful sensorimotor neuropathy. Cranial nerve involvement was seen in four cases and leptomeningeal involvement was seen in two of the cases. Diagnosis was established with MRI in eight cases with 100% positivity. Seven cases had PET-CT on presentation, of which five were positive. Most cases were treated with systemic chemotherapy with R-CHOP and high dose Methotrexate. One case received stem cell transplant which was followed by relapse. One case reported successful treatment with CAR-T brexu-cel infusion with no evidence of relapse on 2-month follow up. Most patients died of progression of disease although survival data was not available for all cases.

Rapid, aggressive infiltration of both the nerve roots and skin by mantle cell lymphoma represents unusually high-grade disease. This case highlights the importance of clinical suspicion of NL in any subtype of lymphoma showing aggressive features. There is a narrow window for treatment to prevent fatal outcomes. MRI is the diagnostic modality of choice, which was instrumental in establishing diagnosis in our case and further workup was not felt necessary. Novel CART-cell therapy and consolidative therapies such as high dose chemotherapy followed by stem cell transplant (HDC- ASCT) should be considered in appropriate cases, as we intended in our case after disease stabilization.

No relevant conflicts of interest to declare.