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Review
. 2023 Dec 1;12(4):CNS105.
doi: 10.2217/cns-2023-0014. Epub 2023 Oct 25.

Cutaneous T-cell lymphoma with CNS involvement: a case series and review of the literature

Joshua D Preston  1   2 , Caroline S Jansen  1   3 , Siddhartha Kosaraju  4 , Tim Niyogusaba  5 , Tony Z Zhuang  6 , Sally W Iwamoto  7 , Spencer K Hutto  7 , Mary Jo Lechowicz  8 , Pamela B Allen  8
Affiliations
Review

Cutaneous T-cell lymphoma with CNS involvement: a case series and review of the literature

Joshua D Preston et al. CNS Oncol. .

Abstract

Cutaneous T-cell lymphoma (CTCL) is a rare hematologic malignancy that traditionally presents with cutaneous lesions, though metastases are not uncommon in progressive disease. We describe four cases of CTCL with central nervous system (CNS) involvement, detailing the history, pathological characteristics, treatment response, and progression. Median time from initial diagnosis to CNS metastasis was ∼5.4 years (range 3.4-15.5 years) and survival after metastasis was ∼160 days (range 19 days-4.4 years). No patients achieved long-term (>5 years) survival, though some displayed varying degrees of remission following CNS-directed therapy. We conclude that clinicians must be attentive to the development of CNS metastases in patients with CTCL. The growing body of literature on such cases will inform evolving therapeutic guidelines on this rare CTCL complication.

Keywords: Sezary syndrome; brain; metastasis; mycosis fungoides; visceral.

Plain language summary

Cutaneous T-cell lymphoma (CTCL) is a rare cancer of the blood, which typically manifests with skin lesions, such as itchy, scaly rashes that may thicken to form tumors on the skin. Though uncommon, metastases do occur in CTCL. A particularly rare location for these metastases is the central nervous system. This case series recounts the story of four unique patients and the presentation, diagnosis, and treatment of their CTCL, which unfortunately progressed to involve the central nervous system. Outcomes with central nervous system involvement in CTCL are poor, but may occur sometime later than a patient's initial diagnosis. Our patients had a median time from initial diagnosis to central nervous system metastases of ∼5.4 years and a survival of ∼160 days after central nervous system metastases. Some types of therapy, such as radiation, surgery, or chemotherapy, may be beneficial in extending survival or providing symptomatic relief for patients. It can be difficult to recognize symptoms of central nervous system metastases, so this case series emphasizes that vigilance for potential metastases and use of interdisciplinary teams is important in caring for these patients. This case series demonstrates the importance of continued research in this area, with the hope of improving outcomes for patients with central nervous system metastases of CTCL.

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Conflict of interest statement

Competing interests disclosure

The authors have no competing interests or relevant affiliations with any organization or entity with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Figures

Figure 1.
Figure 1.. Images of case 1.
T2 MRI image showing hyperintensity representing degeneration in the transverse pontocerebellar tracts and median pontine raphe nuclei (“hot cross bun sign”) (A). FLAIR image showing edema in the medial right temporal lobe (B). Diffusion-weighted MRI image (B = 1000) demonstrating restricted diffusion in the area of FLAIR abnormality (C). Post-gadolinium image demonstrating peripheral enhancement corresponding to the areas of restricted diffusion (D). PET image with right temporal hypermetabolism corresponding to areas of restricted diffusion and enhancement (E).
Figure 2.
Figure 2.. Pretreatment, post-treatment, and recurrence images for case 2.
Pretreatment FLAIR image showing edema in the superior cerebellar vermis (A). Pretreatment axial (B) and coronal (C) post-gadolinium images showing mass-like enhancement in the superior cerebellum and leptomeningeal enhancement along the folia. Post-treatment FLAIR image showing significantly improved edema (D). Post-treatment axial (E) and coronal (F) post-gadolinium images showing near-complete resolution of cerebellar enhancement. Subsequent MRI 2 months later showing recurrent abnormal cerebellar FLAIR signal abnormality (G) with corresponding restricted diffusion (H) and abnormal hypermetabolism (I).
Figure 3.
Figure 3.. Case 3 images.
PET CT showing hypermetabolic right parietal lesion (A). Post-gadolinium MRI showing enhancing lesion corresponding to hypermetabolism (B). FLAIR image showing edema surrounding the lesion (C). Post-treatment pre-contrast MRI showing resection cavity (D). Post-treatment post-gadolinium MRI showing essentially complete resolution of the enhancing lesion (E).
Figure 4.
Figure 4.. Graphical timeline of cases 1–4.
Time ‘0’ represents the first point where a biopsy-confirmed diagnosis was made, and the listed age of the patients corresponds with their age at time ‘0.’ The various shapes on the graph correspond with specific sets of imaging obtained in Figures 1–3, as defined by the shape legend on the right.
See this image and copyright information in PMC

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