Lymphomatoid granulomatosis (CNS manifestations)
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Lymphomatoid granulomatosis of the central nervous system is uncommon, but represents the second most common site of involvement in patients with systemic lymphomatoid granulomatosis, after the lungs, which are most commonly involved. In the 2021 (5th edition) WHO classification of CNS tumors it is considered one of the CNS lymphomas 3.
For a general discussion of the underlying condition, including epidemiology and pathology, please refer to the article lymphomatoid granulomatosis.
Lymphomatoid granulomatosis of the CNS most commonly affects adults (40-60 years of age) and is more common in immunocompromised individuals 4.
Neurologic symptoms are fairly common, seen in around 30% of cases, usually in the setting of systemic or cutaneous symptoms 1. The specifics of the presentation will depend on the location of disease within the central nervous system.
Lymphomatoid granulomatosis is frequently EBV-associated 4. The lesions are composed of large EBV-positive tumor B cells admixed with infiltrating plasma cells and lymphocytes (CD4+ and CD8+ T-lymphocytes). They are typically angiocentric or angiodestructive 4.
MRI is the modality of choice for assessing patients with suspected lymphomatoid granulomatosis.
Appearances are very variable with lesions seen in all compartments (supra- and infratentorial) and demonstrating a wide range of morphology.
Generally, lesions are located in the periventricular white matter but can extend to involve cortex.
Punctate or linear T2 hyperintensities within perivascular spaces are also characteristic and show contrast enhancement 1,2. Larger lesions may be solid or demonstrate ring enhancement. They have variable surrounding edema and may be associated with leptomeningeal enhancement 1.
Treatment and prognosis
Treatment is generally with chemotherapy (including corticosteroids) and radiation. Prognosis is variable and depends on tumor grade.
The appearance of lymphomatoid granulomatosis is variable and thus may mimic many other conditions including:
- 1. Tateishi U, Terae S, Ogata A et-al. MR imaging of the brain in lymphomatoid granulomatosis. AJNR Am J Neuroradiol. 2001;22 (7): 1283-90. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Degnan AJ, Levy LM. Neuroimaging of rapidly progressive dementias, part 2: prion, inflammatory, neoplastic, and other etiologies. AJNR Am J Neuroradiol. 2014;35 (3): 424-31. doi:10.3174/ajnr.A3455 - Pubmed citation
- 3. Louis D, Perry A, Wesseling P et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro-Oncology. 2021;23(8):1231-51. doi:10.1093/neuonc/noab106 - Pubmed
- 4. Reifenberger A, Soffietti M, Deckert M, Ferry JA, Paulus W, Weller M, Batchelor T, Xuan K, Nagane M, Lymphomatoid granulomatosis. In: WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6). https://publications.iarc.fr/601