Primary peripheral T-cell central nervous system lymphoma

Cylaina E. Bird, Jeffrey I. Traylor, Jenna Omas, James P. Caruso, Benjamin Kafka, Flavia Rosado, Kyle M. Blackburn, Kimmo J. Hatanpaa, Kalil G. Abdullah

Research output: Contribution to journalArticlepeer-review


Background: Primary peripheral T-cell central nervous system lymphoma (PCNSL) is a rare, aggressive tumor that arises in the craniospinal axis and has an increased risk in individuals who are immunocompromised. This lesion often mimics other benign and malignant processes on radiographic imaging, leading to misdiagnosis and delays in treatment. We present a case of a patient with a history of Sjögren’s syndrome and progressive neurologic symptoms who underwent craniotomy for diagnosis. Case Description: A 61-year-old woman with a history of Sjögren’s syndrome, progressive aphasia, left facial droop, and right-sided paresthesias for 4 months presented for evaluation and management. An enhancing, infiltrative lesion in the left frontal lobe with underlying vasogenic edema was appreciated and suggestive of a primary or metastatic neoplasm. The patient underwent an open biopsy for further evaluation of the lesion. Extensive histopathologic evaluation revealed a diagnosis of T-cell PCNSL. The patient was started on induction methotrexate and temozolomide followed by consolidative radiotherapy. Conclusion: Autoimmune conditions are a risk factor for T-cell PCNSL development. T-cell PCNSL has radiographic and gross histologic features that are consistent with a broad differential, including gliomas and inflammatory processes. Prompt diagnosis and extensive histopathological evaluation is essential to ensure appropriate treatment.

Original languageEnglish (US)
Article number465
JournalSurgical Neurology International
StatePublished - 2021


  • Autoimmune disease
  • Glioma
  • Primary central nervous system lymphoma
  • Sjögren’s syndrome

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology


Dive into the research topics of 'Primary peripheral T-cell central nervous system lymphoma'. Together they form a unique fingerprint.

Cite this