The Diagnostic Dilemma of Neurolymphomatosis

Abstract

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkin's lymphoma (NHL) and NL, all of whom were men aged 47–69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

Feline lymphoma in the nervous system: pathological, immunohistochemical, and etiological aspects in 16 cats

“…The gross findings often diverge from the common lymphoma presentation in other organs (Linzmann et al 2009). Lymphoma is the main secondary tumor in the feline CNS and neurolymphomatosis is a rare condition in cats, other animals, and humans (Allen & Amis 1975, Hankenson et al 1998, Kuntzer et al 2000, Mellanby et al 2003, Higgins et al 2008, Choi et al 2013, Shree et al 2016. The neoplastic infiltration in leptomeninges (leptomeningeal lymphomatosis) and perivascular space from medullar parenchyma, as observed in the present study, is a common consequence relative to spinal nerve neurolymphomatosis (Lane et al 1994, Schaffer et al 2012, Mandrioli et al 2012, Rupp et al 2014, Sakurai et al 2016.…”

Painful right elbow mass

“…Neurolymphoma (NL) is a rare entity representing peripheral nervous system infiltration by malignant lymphocytes [1]. It is most commonly seen in the context of aggressive sub-types of lymphoma, such as diffuse large B cell non-Hodgkin's lymphoma [2].…”

Sciatic pain by neurolymphomatosis as initial presentation of disseminated diffuse large B cell lymphoma involving the testis and the CNS

“…NL is a very rare manifestation of non‐Hodgkin lymphoma (NHL) occurring in only 0.85% to 2.9% of newly diagnosed cases . It may present with a pattern of mononeuropathy or symmetrical neuropathy as well as pure demyelinating or mixed axonal and demyelinating neuropathy …”

“…2 It may present with a pattern of mononeuropathy or symmetrical neuropathy as well as pure demyelinating or mixed axonal and demyelinating neuropathy. 3 Here, we describe the case of a testicular lymphoma with first manifestation of visual disturbances and sciatic pain. F I G U R E 1 PET-CT scan images.…”