A Systematic Review of Spinal Lymphomatoid Granulomatosis Cases

ABSTRACT Objective Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated systemic angiocentric and angiodestructive lymphoproliferative disorder. It commonly involves the lungs and can also affect the skin, liver, kidney, and central nervous system. It can rarely occur in the spine, although the details are unclear. Here, we performed a systematic review of published cases (including our one case) of spinal LYG. Methods We performed a systematic search of studies in English on spinal LYG, focusing on its clinical features, imaging, and treatments, according to the PRISMA guidelines on the PubMed database. We identified 14 patients from the literature. We also found one case of isolated cervical LYG (grade 3) who was treated with steroid and radiation therapy for the spinal lesion after pathological diagnosis. We performed a pooled analysis of these 15 cases. Results The mean age was 43.4 years, and 13 patients were male out of the 15 cases. Brain lesions were present in 11 out of 12 intramedullary spinal lesions, and only 1 was an isolated spinal LYG case. Regarding the diagnostic methods, one case was not described. Of the 14 cases described, 12 cases were performed with biopsy (7 brain, 4 lung, and 1 spinal cord lesion biopsies), and 2 underwent surgical removal for an extramedullary lesion. In the overall prognosis from a mean follow-up period of 21.8 months, 4 cases died despite several treatments. Conclusions Spinal LYG is rare, particularly isolated spinal LYG. Thus, further accumulation of cases may be necessary to better understand its characteristics.