Essentials for Early Diagnosis of Primary Intramedullary Spinal Cord Lymphoma. How to Suspect Primary Intramedullary Spinal Cord Lymphoma Early and Proceed to Invasive Biopsy? A Case Report and Literature Review

Overview

Journal Surg Neurol Int

Specialty Neurology

Date 2024 Mar 12

PMID 38468650

Authors

Seiji Shigekawa

Akihiro Inoue

Yukihiro Miyazaki

Mashio Taniwaki

Kota Kanehisa

Sayaka Matsumoto

Yoko Okada

Riko Kitazawa

Takeharu Kunieda

Affiliations

Soon will be listed here.

Abstract

Background: Primary intramedullary spinal cord lymphoma (PISCL) is an extremely rare condition. Early diagnosis is very difficult due to the nonspecific clinical and imaging findings. A biopsy is essential for a definitive diagnosis, but courage is required to perform the surgery. Here, we present a case of PISCL and suggest useful indicators for accurate diagnosis of this pathological entity.

Case Description: A 70-year-old woman presented with subacute bilateral lower-limb paralysis, disturbance of warm and pain sensations, and vesicorectal disturbance. Magnetic resonance imaging showed a contrast-enhanced mass from C7 to Th2 and large, edematous lesions from the upper cervical to lower thoracic spinal cord. Elevated uptake of F-fluoro-2-deoxy-D-glucose (FDG) was identified in the enhanced regions on FDG-positron emission tomography (PET). Cerebrospinal fluid (CSF) analysis revealed highly elevated levels of β2-microglobulin (β2-MG). Steroid pulse therapy and therapeutic plasma exchange were performed for suspected myelitis, but symptoms did not improve. Spinal cord biopsy was, therefore, performed for treatment-resistant myelopathy. Histopathological examination revealed diffuse large B-cell lymphoma, which was diagnosed as PISCL because systemic examination showed no other findings suggestive of malignant lymphoma.

Conclusion: In cases with poor response to treatment and a progressive course, PISCL should be considered, and spinal cord biopsy should be performed if PET shows increased F-FDG uptake and β2-MG is elevated in CSF.

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