Philipp Karschnia1,2,3, Tracy T Batchelor1, Justin T Jordan1, Brian Shaw1, Sebastian F Winter1, Frank J Barbiero2, Leon D Kaulen2, Niklas Thon3, Joerg-Christian Tonn3, Anita J Huttner4, Robert K Fulbright5, Jay Loeffler6, Jorg Dietrich1, Joachim M Baehring2. 1. Division of Neuro-Oncology, Department of Neurology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, Massachusetts. 2. Department of Neurology, Yale School of Medicine, New Haven, Connecticut. 3. Department of Neurosurgery, Ludwig Maximilian University, Munich, Germany. 4. Department of Pathology, Yale School of Medicine, New Haven, Connecticut. 5. Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, Connecticut. 6. Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
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 mm2 /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.
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 mm2 /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.
Authors: Philipp Karschnia; Nico Teske; Mario M Dorostkar; Sebastian Siller; Jonathan Weller; Joachim M Baehring; Jorg Dietrich; Louisa von Baumgarten; Jochen Herms; Joerg-Christian Tonn; Niklas Thon Journal: Sci Rep Date: 2020-11-12 Impact factor: 4.379
Authors: Cylaina E Bird; Jeffrey I Traylor; Jenna Thomas; James P Caruso; Benjamin Kafka; Flavia Rosado; Kyle M Blackburn; Kimmo J Hatanpaa; Kalil G Abdullah Journal: Surg Neurol Int Date: 2021-09-13
Authors: Furqaan Ahmed Kaji; Nicolás Martinez-Calle; Vishakha Sovani; Christopher Paul Fox Journal: Br J Haematol Date: 2022-03-16 Impact factor: 8.615