Primary Central Nervous System ALK Positive Anaplastic Large Cell Lymphoma with Predominantly Leptomeningeal Involvement in an Adult.
10.3349/ymj.2013.54.3.791
- Author:
Jae Sung PARK
1
;
Heejung PARK
;
Sanghui PARK
;
Suk Jin KIM
;
Ho Jun SEOL
;
Young Hyeh KO
Author Information
1. Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- Publication Type:Case Reports
- Keywords:
ALK-positive;
primary;
CNS;
anaplastic large-cell lymphoma;
leptomeningeal
- MeSH:
Adult;
Antineoplastic Agents/therapeutic use;
Biopsy;
Brain/metabolism/pathology;
Diagnosis, Differential;
Humans;
Immunohistochemistry;
Lymphoma, Large-Cell, Anaplastic/*diagnosis/drug therapy/pathology;
Male;
Meningeal Neoplasms/*diagnosis/drug therapy/pathology;
Receptor Protein-Tyrosine Kinases/*metabolism
- From:Yonsei Medical Journal
2013;54(3):791-796
- CountryRepublic of Korea
- Language:English
-
Abstract:
A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALK+ALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.