Understanding the Pathognomonic Tumefactive Appearance of Neurolymphomatosis: A Unifying Theory of Neurolymphoma

Abstract

The diagnosis of peripheral neurolymphomatosis (NL) is difficult and often delayed, because patients can have isolated, nonspecific nerve symptoms. Magnetic resonance imaging will usually show nonspecific findings of enlarged, contrast-enhancing nerves. We aimed to elucidate the mechanism behind an imaging finding we believe is pathognomonic of NL and likely of other hematologic diseases with peripheral nerve involvement. We reviewed the imaging studies of a previously reported cohort of patients, in addition to those from more recent patients, all with tumefactive NL, in which enlarged nerve bundles were surrounded by tumor. We reviewed the demographic data, clinical data (e.g., primary or secondary disease, biopsy-proven diagnosis), and imaging findings (e.g., tumefactive appearance, primary involved nerve, location of epicenter of tumefactive appearance, vascular involvement). All cases showed a maximum tumefactive appearance at branch or junction points, with a gradual decrease of this appearance moving proximally and distally from the epicenter in a "crescendo-decrescendo" pattern. We have described this as a phasic mechanism with 3 phases: malignant cells fill the intraneural space; extrude at a weak spot of the nerve, which often occurs at a branch or junction point; and then expand and fill the subparaneurial space, creating the grossly tumefactive appearance with proximal and distal spread. We have presented a novel, unifying theory explaining the pathognomonic tumefactive appearance of NL. Our theory offers the first rational explanation for the radiological appearance of NL with peripheral nerve involvement. We believe that with earlier recognition of NL on imaging studies, patients will be able to receive an earlier diagnosis and undergo earlier treatment.