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

Abstract

Cutaneous T-cell lymphoma (CTCL) is a rare malignancy of the lymphocytes, which primarily presents with dermatologic lesions. Rarely, these lesions can progress to include visceral metastases, which, as reported here, can include metastases to the central nervous system (CNS). • This case series reports four cases of CTCL with CNS involvement, with varying histories, pathological characteristics, treatment regimens, treatment responses, and outcomes. • These four cases demonstrate the poor prognosis that CNS involvement of CTCL carries, as our patients had a median survival of 160 days following recognition of CNS involvement. • As CNS involvement does result in high morbidity and mortality, clinicians should be mindful of this potential manifestation of metastatic disease, despite its rarity. • Despite this poor prognosis, some patients did display clinical benefit following CNS-directed therapies, such as radiation, surgery, and high dose methotrexate. Importantly, these treatments may also provide symptomatic palliation, which is important for patient quality of life, even in the absence of survival benefit. • Given that outward manifestations of CNS involvement, such as neurologic symptoms, may be subtle or difficult to detect, careful follow-up and interdisciplinary evaluation of these patients are warranted. • While imaging and CSF sample are often used together to establish the diagnosis of CNS involvement in CTCL, our cases demonstrate that imaging alone may be sufficient in some cases. • Two of our cases demonstrate periorbital and ocular involvement. Our report, along with others, reinforce the importance of considering this presentation as high-risk for potential CNS progression. • This case series demonstrates the variable presentations and difficult prognoses represented by CNS metastases in CTCL. Further studies, improved treatment regimens, and more robust guidelines for management are needed to continue to improve outcomes for patients with CNS-metastatic CTCL.