Primary peripheral T-cell central nervous system lymphoma.
Autoimmune disease
Glioma
Primary central nervous system lymphoma
Sjögren’s syndrome
Journal
Surgical neurology international
ISSN: 2229-5097
Titre abrégé: Surg Neurol Int
Pays: United States
ID NLM: 101535836
Informations de publication
Date de publication:
2021
2021
Historique:
received:
06
03
2021
accepted:
07
08
2021
entrez:
8
10
2021
pubmed:
9
10
2021
medline:
9
10
2021
Statut:
epublish
Résumé
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. 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. 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.
Sections du résumé
BACKGROUND
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
METHODS
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
CONCLUSIONS
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.
Identifiants
pubmed: 34621580
doi: 10.25259/SNI_224_2021
pii: 10.25259/SNI_224_2021
pmc: PMC8492444
doi:
Types de publication
Case Reports
Langues
eng
Pagination
465Informations de copyright
Copyright: © 2021 Surgical Neurology International.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
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