Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 16, Number 7, July 2025, pages 259-266

Secondary Central Nervous System Lymphoma Involving Meninges: A Rare Case Report and a Comprehensive Review of Peripheral T-Cell Lymphoma, Not Otherwise Specified

Figures

Figure 1.
Figure 1. Initial non-contrast computed tomography (CT) scan of the head demonstrating bilateral extra-axial dural-based masses (red arrows).
Figure 2.
Figure 2. MRI of the brain with contrast showing an infiltrative process extending from the extracranial soft tissues into the epidural space bilaterally (red arrows), with associated brain parenchymal lesion, and vasogenic edema in the right frontal lobe causing extrinsic compression (white arrow) (a) and diffuse meningeal enhancement (white arrows) (b). MRI: magnetic resonance imaging.
Figure 3.
Figure 3. PET scan showing FDG-avid lesions in the right supraclavicular and left axillary lymph node regions (arrows). PET: positron emission tomography; FDG: fluorodeoxyglucose.
Figure 4.
Figure 4. Hematoxylin and eosin (H&E) stained section of the lymph node biopsy showing diffuse effacement of nodal architecture by a dense infiltrate of monomorphic lymphoid tumor cells (white arrows) (a) with prominent arborized endothelial venules (red arrow) (b).
Figure 5.
Figure 5. Immunohistochemistry demonstrating strong and diffuse membranous CD4 staining across the neoplastic lymphoid population, consistent with a T-helper cell phenotype.
Figure 6.
Figure 6. Immunohistochemistry demonstrating neoplastic lymphoid population with sparse scattered CD15 staining (blue arrows).
Figure 7.
Figure 7. Repeat MRI of the brain with contrast demonstrating marked improvement in the previously noted irregular dural thickening (red arrows) and significant reduction of the mass-like thickening anterior to the right frontal lobe (white arrow) following initial treatment. MRI: magnetic resonance imaging.

Tables

Table 1. Common Subtypes of Peripheral T-Cell Lymphomas in the Order of Frequency of Occurrence
 
SubtypeMain characteristics
NK: natural killer; ALK: anaplastic lymphoma kinase; HTLV-1: human T-lymphotropic virus-1.
Peripheral T-cell lymphoma, not otherwise specifiedThe most common and heterogeneous subtype. Predominantly nodal.
Anaplastic large cell lymphomaIt is further divided into ALK-positive and ALK-negative subtypes, with ALK-positive cases having a better prognosis. Predominantly nodal.
Angioimmunoblastic T-cell lymphomaCharacterized by systemic symptoms and immune dysregulation. Mostly nodal.
Extranodal NK/T-cell lymphomaAssociated with Epstein-Barr virus and frequently involves the nasal cavity and upper aerodigestive tract.
Adult T-cell leukemia/lymphomaThis subtype varies with region and is common in areas endemic to HTLV-1 infection, such as some parts of Japan and the Caribbean, and frequently affects lymph nodes. CNS involvement is common compared to other subtypes.
Subcutaneous panniculitis-like T-cell lymphomaInvolves the subcutaneous fat tissue.
Enteropathy-associated T-cell lymphomaStrongly associated with celiac disease
Monomorphic epitheliotropic intestinal T-cell lymphomaA rare and aggressive subtype that primarily affects the gastrointestinal tract, especially the small intestine.
Hepatosplenic T-cell lymphomaAnother rare and aggressive lymphoma primarily affecting young males.

 

Table 2. Clinical Manifestations of PTCL-NOS
 
Clinical FeatureDescription/frequency
PTCL-NOS: peripheral T-cell lymphoma, not otherwise specified; CNS: central nervous system.
B symptomsPresent in approximately 35% of cases. Includes fever, night sweats, fatigue, and unexplained weight loss.
LymphadenopathyObserved in 35-40% of cases, often presenting as painless swelling in the neck, armpits, or groin. Multinodal involvement is common. In some instances, massive lymphadenopathy can lead to complications such as limb swelling or compression of adjacent structures.
HepatosplenomegalyOccurs in approximately 20% of patients, potentially leading to abdominal discomfort, pain (rare), fullness, or early satiety.
Skin involvementCommon site of extranodal disease. May present as rashes, nodules, or plaques.
Gastrointestinal involvementCommon site of extranodal disease. Symptoms can include abdominal pain, diarrhea, weight loss, and, in rare cases, gastrointestinal bleeding. In high-burden disease, complications such as bowel obstruction or perforation may occur.
Pulmonary and salivary gland involvementLess commonly affected; when involved, may present with respiratory symptoms or salivary gland enlargement.
CNS involvementRare organ to involve and uncommon at presentation. Solitary to multiple enhancing lesions on imaging. Symptoms include persistent headaches, focal neurological deficits, new-onset seizures, and cognitive or behavioral changes. The brain is the most affected site (> 90% of CNS cases), followed by the spinal cord, meninges, and peripheral neural plexus.
Bone marrow involvementSeen in approximately 20% of cases; may result in cytopenias such as anemia, leukopenia, or thrombocytopenia.
Nodal and extranodal involvementApproximately 50% of patients present with both nodal and extranodal disease; 10-15% have exclusively extranodal involvement.
Poor prognostic factorsInclude age over 60 years, Ann Arbor stage III or IV, elevated serum lactate dehydrogenase (LDH), poor performance status, and involvement of more than one extranodal site.