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Radiologic morphology of low-grade primary central nervous system lymphoma in immunocompetent patients
Kristoph Jahnke1, Andreas Schilling, Jens Heidenreich
1Department of Hematology, Oncology and Transfusion Medicine, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin, Germany.
Insights
Low-grade primary central nervous system lymphomas (PCNSLs) present with atypical MR imaging features, differing from high-grade PCNSLs. This can lead to delayed or incorrect diagnoses in patients with these rare non-Hodgkin lymphomas (NHLs).
Area of Science:
- Neuroradiology
- Oncology
- Neuropathology
Background:
- Primary central nervous system lymphomas (PCNSLs) are typically high-grade non-Hodgkin lymphomas (NHLs).
- Typical MR imaging findings include contrast-enhancing lesions near the subarachnoid space without necrosis.
- Low-grade PCNSLs are rare and their imaging characteristics are less understood.
Purpose of the Study:
- To evaluate the radiologic morphology and clinical characteristics of low-grade PCNSLs.
- To determine if low-grade PCNSLs exhibit distinct imaging features compared to high-grade PCNSLs.
Main Methods:
- Retrospective review of 332 patients screened for multicenter prospective trials.
- Central review of MR imaging scans by two consultant neuroradiologists.
- Identification of 10 patients with low-grade PCNSLs.
Main Results:
- Low-grade PCNSLs showed atypical features: deep or spinal location (6/10), non-periventricular (5/10), T2 hyperintensity (10/10), moderate/absent enhancement (6/10), and heterogeneous enhancement (5/10).
- Eight patients had >2 atypical features, suggesting a morphology distinct from high-grade PCNSLs.
- Atypical imaging combined with mild symptoms led to false or delayed diagnoses.
Conclusions:
- Low-grade PCNSLs can present with variable and atypical radiologic morphology.
- The distinct imaging patterns of low-grade PCNSLs pose a risk for delayed or missed diagnoses.
- Awareness of these atypical features is crucial for accurate and timely diagnosis.
Background And Purpose:
Primary central nervous system lymphomas (PCNSLs) are usually high-grade and are rarely low-grade non-Hodgkin lymphomas (NHLs). On MR imaging, PCNSLs typically present as contrast-enhancing lesions in contact with the subarachnoid space without evidence of necrosis. We evaluated the radiologic morphology and clinical characteristics of low-grade PCNSLs, hypothesizing that they may differ from high-grade PCNSLs.
Methods:
Records were reviewed from 332 patients screened for inclusion in 3 multicenter prospective trials. MR imaging scans were obtained from all patients and were centrally reviewed by 2 consultant neuroradiologists.
Results:
Ten patients (3%) with low-grade PCNSLs (7 men and 3 women; median age, 59 years; age range, 19-61 years) were identified. Four patients had one lesion, 2 patients 2 lesions, and 4 patients had multiple lesions. The following radiologic features infrequently seen in high-grade PCNSLs were found in a substantial proportion of patients: location in deep structures or spine (n = 6); lack of periventricular location (n = 5); hyperintensity on T2-weighted images (n = 10); moderate or absent contrast enhancement (n = 6); and heterogeneous contrast enhancement (n = 5). In 8 patients, >2 of these features were present in at least one lesion, and, thus, the radiologic appearance was assessed atypical of high-grade PCNSLs. The atypical radiologic appearance in combination with atypical or mild symptoms resulted in a false or delayed diagnosis.
Conclusion:
Low-grade PCNSLs may have a variable and atypical radiologic morphology compared with high-grade PCNSLs with the risk of false or delayed diagnosis.
