Primary Lymphoma of Peripheral Nerve

Abstract

Lymphoma presenting as a solitary tumor of peripheral nerve is exceedingly rare, with only six previously reported cases. The authors describe an additional four cases of primary lymphoma of peripheral nerve involving the sciatic nerve (two cases), the radial nerve, and the sympathetic chain and spinal nerve. The patients were two men and two women with an average age of 55.5 years. All tumors were high-grade B-cell lymphomas. Two patients experienced relapse of disease with involvement of other nervous system sites and died of lymphoma. One patient is alive with stable local disease at 57 months. The fourth patient is alive with no evidence of disease at 54 months. Expression of neural cell adhesion molecule (CD56) has been reported to correlate with an increased incidence of central nervous system involvement in peripheral T-cell lymphoma; all their cases were CD56 negative. Recent reports indicate a high proportion of primary brain lymphomas show loss of CDKN2A/p16 gene expression. Therefore, CDKN2A/p16 was evaluated in their patients both by polymerase chain reaction and by immunohistochemistry for the p16 protein. The authors found homozygous deletion of the CDKN2A/p16 gene in one of three patients studied, confirmed immunohistochemically by absent staining for p16. The fourth patient showed absent staining for p16, suggesting inactivation of the gene in this case as well. The two patients with p16 loss both died of lymphoma, whereas the two patients with normal p16 expression are alive. Primary lymphoma of peripheral nerve is a rare neoplasm, usually of large B-cell type, has a variable prognosis, and appears to have less consistent loss of p16 expression than primary central nervous system lymphoma.

MRI and pet-CT in the diagnosis and follow-up of a lymphoma case with multifocal peripheral nerve involvement

“…Most of the reported cases of primary peripheral nerve lymphomas have heralded the onset of systemic lymphoma, whereas there have been few cases found in patients with known systemic disease. In either case, it may be a harbinger of a more disseminated disease involving the peripheral and central nervous systems (1,4,8,9). The B-cell type of non-Hodgkin lymphoma is the predominant type involving the peripheral nerves, with the NK/T-cell subtype being extremely rare (2,4,8).…”

“…Various treatment approaches have been applied, including chemotherapy, radiotherapy, and surgery, alone and in various combinations (11). Prophylactic and therapeutic intrathecal methotrexate therapy is also commonly used because these patients have an increased risk of central nervous system relapse (1,4,8,9).…”

High‐dose intravenous methotrexate followed by autologous stem cell transplantation as a potentially effective therapy for neurolymphomatosis

“…To our knowledge, few cases to date have shown successful response to combination chemotherapies. 6,23 In the report by Misdraji et al, 14 four patients received a combination of prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide, ara-C, bleomycin, etoposide, methotrexate, CHOP, and a combination of cyclophosphamide, mitoxantrone, etoposide, and prednisone. Two patients achieved complete remission of disease and two achieved a partial response; 2 patients had disease recurrence after 4 months and 19 months, respectively, and subsequently died 6 months and 43 months, respectively, from diagnosis.…”

“…11,12 Patients with NL usually present in the context of widespread systemic multiorgan involvement by non-Hodgkin lymphoma (NHL). 13,14 The involvement of a single peripheral nerve or plexus as the only site of active lymphoma rarely has been reported. 14 -16 Many of these cases had coexistent leptomeningeal involvement.…”

“…14 -16 Many of these cases had coexistent leptomeningeal involvement. 14,15 A recent report by Baehring et al 13 identified four clinical presentations, which included painful involvement of nerves or roots, cranial neuropathy with or without pain, painless involvement of peripheral nerves, and painful or painless involvement of a single peripheral nerve. The treatment of NL represents a challenge because the diagnosis in many cases is delayed and adequate penetration of chemotherapy into the nervous system is difficult.…”