Primary dural lymphomas: Clinical presentation, management, and outcome. Issue 12 (16th March 2020)

• Record Type: Journal Article
• Authors: Karschnia, Philipp; Batchelor, Tracy T.; Jordan, Justin T.; Shaw, Brian; Winter, Sebastian F.; Barbiero, Frank J.; Kaulen, Leon D.; Thon, Niklas; Tonn, Joerg‐Christian; Huttner, Anita J.; Fulbright, Robert K.; Loeffler, Jay; Dietrich, Jorg; Baehring, Joachim M.
• Is Part Of: Cancer. Volume 126:Issue 12(2020)
• Page Start: 2811
• Page End: 2820
• Publication Date: 2020-03-16
• Subjects: central nervous system -- imaging -- lymphoma -- primary dural lymphoma -- therapy; Cancer -- Periodicals; Cancer -- Cytopathology -- Periodicals; 616.99405
• DOI: 10.1002/cncr.32834
• Languages: English
• ISSNs: 0008-543X

Abstract

Abstract : Background: Clinical experience is limited for primary central nervous system (CNS) lymphoma that arises from the dura mater, which is denoted with the term primary dural lymphoma (PDL). This study was aimed at determining the relative incidence, presentation, and outcomes of PDL. Methods: The institutional databases of the Divisions of Neuro‐Oncology at the Massachusetts General Hospital and the Yale School of Medicine were retrospectively searched for patients with primary CNS lymphoma. Patients with pathologically confirmed dural lymphoma and no evidence of primary cerebral or systemic involvement were identified. Clinical data, diagnostic findings, treatments, and outcomes were recorded. Results: A total of 20 patients with PDL were identified, and they represented 6.3% of the individuals with primary CNS lymphomas (20 of 316). Histopathological examination of PDL revealed the following underlying subtypes: diffuse large B‐cell lymphoma (10 of 20 patients), marginal zone lymphoma (6 of 20), follicular lymphoma (2 of 20), undefined B‐cell non‐Hodgkin lymphoma (1 of 20), and T‐cell non‐Hodgkin lymphoma (1 of 20). On imaging, all tumors appeared as extra‐axial masses with avid contrast enhancement and mostly mimicked meningioma. The median apparent diffusion coefficient value was 667 ± 26 mm 2 /s. Cerebrospinal fluid analyses and symptoms were nonspecific, and the diagnosis rested on tissue analysis. Therapeutic approaches included surgery, radiotherapy, and chemotherapy. The median overall survival was not reached after 5 years. Three patients were deceased at database closure because of tumor progression. The extent of tumor resection correlated positively with overall survival ( P = .044). Conclusions: PDL is a rare variant of primary CNS lymphoma that can be radiographically mistaken for meningioma. The outcome is excellent with multimodality treatment, and aggressive surgery may convey a survival advantage in select cases. Abstract : Primary dural lymphoma is a rare variant of primary central nervous system lymphoma and most often represents a diffuse large B‐cell lymphoma arising from the cranial dura mater. Its diagnosis rests on advanced imaging modalities as well as tissue analysis, and the outcome may be excellent with multimodality treatment.