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 extraaxial 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.
Anti-glial nuclear antibody type 1 ADEM Acute demyelinating encephalomyelitis AEGM Autoimmune encephalitis predominantly affecting the grey matter AID Autoimmune disorder CK Creatine kinase CNS Central nervous system CSF Cerebral spinal fluid CTCAE Cancer Institute's Common Terminology Criteria for Adverse Events CTE Number of cycles to neurotoxicity event CTLA-4 Cytotoxic T-lymphocyte-associated antigen 4 DM1 Diabetes mellitus type 1 ECG Electrocardiogram EEG Electroencephalography EMG Electromyography FDA U.S. Food and Drug Administration GAD65 Glutamic acid decarboxylase 65 antibody Immuntherapie. Onkologe (2021).
A 44-year-old woman presented with 4 weeks of increasing neck pain and newly arising dysphagia and hoarseness. Examination was remarkable for left tongue atrophy and fibrillations, tongue deviation to the left, left vocal cord paralysis, and trapezius and sternocleidomastoid muscle wasting (figure) (Collet-Sicard syndrome, CN IX-CNXII paralysis 1,2 ). Workup revealed a partially calcified left cervical glomus jugulare tumor extending from the jugular foramen into the parapharyngeal space (figure, A). The tumor encased the left internal carotid artery, CNXI, and CNXII. The inoperable lesion was treated with external beam radiation therapy (45 Gy, 25 fractions). Her neurologic syndrome remains stable with improved pain.
Background: We present an uncommon cause of intracranial hemorrhage in a young adult. Tuberous sclerosis complex is a rare genetic disorder characterized by skin changes, benign systemic or central nervous system tumors [subependymal giant cell astrocytoma (SEGA)], mental retardation, or epilepsy. Hemorrhage into SEGA is exceedingly rare. Case Presentation: We evaluated a 21-year-old college student with migraine. Biopsy of numerous popular skin lesions on his nose revealed adenoma sebaceum. Magnetic resonance imaging brain showed a subependymal nodule near the foramen of Monro suspected to be SEGA. Genetic analysis identified a tuberous sclerosis complex-1 germ line mutation. Surveillance imaging was recommended for the subependymal tumor. Fourteen months later, he presented with spontaneous hemorrhage into the tumor. Hematoma evacuation and tumor resection revealed SEGA. The college graduate was able to return to full-time work. Conclusions: We present an unusual cause of intracranial hemorrhage in a young adult. Thorough work-up and recognition of an underlying genetic predisposition can curtails diagnostic delay when life-threatening complications occur.
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