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Int J Clin Exp Pathol 2013;6(8):1643-1651

Case Report
Primary anaplastic large cell lymphoma in the dura of the brain: case report and
prediction of a favorable prognosis

Min-Kyung Kim, Chang-Ho Cho, Woo-Jung Sung, Hun-Mo Ryoo, Ho-Jun Lee, Sung-Won Youn, Kwan-Kyu Park

Department of Pathology, Department of Hemato-oncology, Department of Radiation oncology, 4Department of Radiology, School of Medicine,
Catholic University of Daegu, Daegu, Republic of Korea

Received June 18, 2013; Accepted July 10, 2013; Epub July 15, 2013; Published August 1, 2013

Abstract: Anaplastic large cell lymphoma (ALCL) is a rare T-cell lymphoma composed of CD30-positive lymphoid cells. ALCL arising in the
dura matter of the brain is even more infrequent, in which only one case has been reported worldwide so far. We report a case of a 30-year-old
immunocompetent male with a dura-based mass, radiographically consistent with meningioma. However, the excised mass via a left
parieto-occipital craniotomy was composed of large, pleomorphic lymphoid cells to be immunopositive for CD3, CD30, anaplastic lymphoma
kinase protein-1 (ALK-1) and epithelial membrane antigen (EMA), and immunonegative for CD20, CD15 and CD68. Multiple ALK gene fusion
signals in the ALK locus were detected by fluorescence in situ hybridization (FISH) analysis. The patient was treated with CHOP chemotherapy
and intrathecal methotrexate along with brain radiation therapy, which resulted in a complete remission. In an analysis of 25 previously
reported primary CNS ALCLs, ALK-1 positivity was shown to be prevalent in younger age, as ALCL occurs outside the brain. Patient less than
23 years, ALK-1 positivity and unifocal tumor may be associated with a better prognosis. However, sex, dural or leptomeningeal involvement,
immune status, and tumor necrosis do not appear to have any influence on survival. (IJCEP1306011).

Keywords: Anaplastic large cell lymphoma, ALK-positive, dura, prognosis, primary tumor

Address correspondence to: Dr. Kwan-Kyu Park, Department of Pathology, School of Medicine, Catholic University of Daegu, 3056-6
Daemyung-4 Dong, Nam-gu, Daegu, 705-718, Republic of Korea. Tel: 82-53-650-4149; Fax: 82-53-650-3050; E-mail: kkpark@cu.ac.kr