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Isolated primary neurolymphomatosis is a rare manifestation of lymphoma, which is challenging to diagnose as there is only involvement of the nervous system, and nerve biopsy is not frequently pursued due to the high risk of irreversible complications.We present a case of isolated primary neurolymphomatosis of diffuse large B-cell lymphoma restricted to only the right brachial plexus and right axillary nerve. The clinical course has been indolent for several years. The initial examination, including MRI and the cerebrospinal fluid study, did not yield any evidence of malignancy. Eventually, due to the patient's symptom progression and the follow-up imaging findings, we conducted a partial nerve biopsy of the brachial plexus to confirm the malignancy. His neurological symptoms did not further deteriorate post-biopsy.Isolated primary neurolymphomatosis with an indolent course is rare and challenging to diagnose. Serial MRI and fluorodeoxyglucose-positron emission tomography reveal clues for tumor involvement. Partial nerve biopsy or targeted fascicular nerve biopsy could be an alternative for achieving a pathologic diagnosis.

Intralymphatic spread is common in solid cancers, but has been rarely studied in lymphomas. Review of 635 extranodal specimens from 475 diffuse large B-cell lymphoma (DLBCL) patients revealed intralymphatic spread in 10 surgical resection specimens from 10 patients including 9 de novo DLBCLs and 1 Richter transformation. The prevalence in de novo DLBCL with extranodal involvements was 1.65%. The most common involved site of intralymphatic spread was the gastrointestinal tract, followed by the female genital tract and breasts. Lymphatic vessels, lined by D2-40-positive endothelial cells, were expanded by lymphoma cells, reminiscent of intravascular lymphoma or tumor emboli. None of the involved lymphatic vessels were located in the mucosa. Patients with intralymphatic spread had a trend of lower overall response rate and a trend of higher progressive disease than those without intralymphatic spread. Compared with patients without intralymphatic spread, those patients with intralymphatic spread had a shorter median overall survival (14.3 vs. 96.2 mo; P=0.004) and a shorter median progression-free survival (11.2 vs. 64.2 mo; P=0.01), respectively. Multivariate analyses showed that intralymphatic spread was an independent poor prognostic factor for overall survival (hazard ratio, 3.029; 95% confidence interval, 1.315-6.978; P=0.009), irrespective of the National Comprehensive Cancer Network-International Prognostic Index, B symptoms, and serum albumin levels. Among patients who underwent surgical resection, intralymphatic spread was still an independent prognostic factor. In conclusion, our study demonstrated extranodal intralymphatic spread in DLBCL. Inspiringly, this rare morphologic finding may serve as a new negative prognostic indicator in DLBCL with extranodal involvements.