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

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.

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.

Graphical abstract

Keywords::

• brain
• metastasis
• mycosis fungoides
• Sezary syndrome
• visceral

Author contributions

All authors directly participated in the clinical care of the patients described herein and/or were involved in chart review of individual cases. JD Preston, CS Jansen, T Niyogusaba, and TZ Zhuang were primarily responsible for drafting this manuscript. All images presented in Figures 1–3 were obtained by S Kosaraju, and JD Preston was responsible for the creation and organization of Figures 1–4 and Table 1. SW Iwamoto, SK Hutto, MJ Lechowicz, and PB Allen all provided editorial feedback and/or revisions to the manuscript. PB Allen functioned in an advisory role for this project. All authors read and approved the final copy of this manuscript.

Acknowledgments

The graphical abstract for this article was created with BioRender.com, and the upper right panel in the graphical abstract was adapted from “Icon Pack - Radiation”, by BioRender.com (2023). Retrieved from https://app.biorender.com/biorender-templates.

Financial disclosure

JD Preston and CS Jansen are supported by the Medical Scientist Training Program Grant (T32 GM008169). CS Jansen is supported by National Cancer Institute Grant (F30CA243250). JD Preston is supported by National Cancer Institute Grant (R01CA264519). PB Allen is supported by Conquer Cancer Foundation (Award # 0000059354) and Lymphoma Research Foundation (Award # 0000059101). The content is solely the responsibility of the authors and does not necessarily represent the official views of NIH or NCI. PB Allen reports advisory board and research funding from Kyowa Kirin, Daichii Sankyo, Secura Bio, and BostonGene. MJ Lechowicz reports advisory board and honorarium from EUSA Pharma, LLC, Secura Bio, Inc. and Kyowa Kirin. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

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.

Writing disclosure

No writing assistance was utilized in the production of this manuscript.

Ethical conduct of research

This study was approved by Emory University’s institutional review board under IRB protocol #08102020 and was compliant with all relevant guidelines and regulations. In case #1, consent was provided by the patient and their family to conduct additional review of their medical records and publish de-identified details of their case. The other cases had a waiver of consent given the retrospective nature of the study and lack of feasibility to collect consent. All protected health information was de-identified in accordance with the Health Insurance Portability and Accountability Act.

Additional information

Funding

JD Preston and CS Jansen are supported by the Medical Scientist Training Program Grant (T32 GM008169). CS Jansen is supported by National Cancer Institute Grant (F30CA243250). JD Preston is supported by National Cancer Institute Grant (R01CA264519). PB Allen is supported by Conquer Cancer Foundation (Award # 0000059354) and Lymphoma Research Foundation (Award # 0000059101). The content is solely the responsibility of the authors and does not necessarily represent the official views of NIH or NCI. PB Allen reports advisory board and research funding from Kyowa Kirin, Daichii Sankyo, Secura Bio, and BostonGene. MJ Lechowicz reports advisory board and honorarium from EUSA Pharma, LLC, Secura Bio, Inc. and Kyowa Kirin. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.