Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma in a Child

Duke University, Durham, North Carolina, United States
Pediatric Neurosurgery (Impact Factor: 0.33). 02/2007; 43(6):516-21. DOI: 10.1159/000108799
Source: PubMed


A 4-year-old African American male was referred to the Pediatric Neurosurgery Service for evaluation of new onset seizures and worsening mental status. An MRI of the brain revealed a pineal region mass with diffuse leptomeningeal enhancement and compression of the basilar cisterns. A biopsy of the brain revealed histologic and immunophenotypic findings characteristic of ALK-1+ anaplastic large cell lymphoma (ALCL). ALCL rarely occurs in the central nervous system and poses a significant diagnostic challenge often leading to a delay in the initiation of appropriate treatment. We describe a case of a rapidly deteriorating clinical course in a child with central nervous system ALCL and review the current literature on ALCL occurring in the central nervous system.

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    • "Case Age (yr)/Sex Location Clinical pre- sentation Immune status Pathological features Treat- ment Outcome (post- diagnosis) Hallmark cells Positive Tcell markers Positive Bcell markers Lin- eage EMA Necro- sis ALK-1 positive cases 1. Havlioglu et al. 1995 [6] 4/F Multifocal lobes, brain stem, spinal cord Headaches, neck stiffness Normal Y N N Null Pos N Ex, R, C NED at 73 months 2. Buxton et al. 1998 [7] 10/F Rt. parietal lobe, falx Left hemiparesis Normal ND CD3, CD45RO ND T ND Y Ex, R, C Dead at 6 months from post chemo sepsis 3. Abdulkader et al. 1999 [8] 13/M Rt. fontal and parietal lobe (meningeal involve- ment) As mycobacterial CNS infection Normal Y CD45RO N T Pos Y C Dead shortly after chemotherapy 4. Ponzoni et al. 2002 [9] 29/M Lt. fontal and temporal lobes Fever, headache, generalized seizures ND Few CD3, CD45RO N T Pos Y R, C NED at 19 months 5. George et al. 2003 [10] 17/M Rt. parietal dura ND Normal ND CD3, CD43, CD45RO N T ND Y Ex, R NED at 57 months 6. George et al. 2003 [10] 18/F Lt. temporal lobe (dural involvement) ND Normal ND CD45RO N T ND N Ex, R, C NED at 62 months 7. Rupani et al. 2005 [11] 17/M Rt. fronto-parietal lobe Headache, left partial seizures Normal Y CD43 ND T Pos N Ex, R, C Dead at 4 month 8. Cooper et al. 2006 [12] 39/M Rt. parieto-occipital lobe Headaches, seizures Normal Few CD3, CD43 N T Pos ND Ex, R, C NED at 9 months 9. Carmichael et al. 2007 [13] 38/M Rt. parieto-occipital lobe Seizure, left hemi- paresis Normal ND CD45 ND T ND ND R, C NED at 15 months 10. Karikari et al. 2007 [14] 4/ M Frontal and parietal lobe (leptomeningeal involvement ), pineal region Generalized tonic clonic seizures ND ND CD3, CD4, CD7 N T ND ND R, C ED at 1 months 11. Merlin et al. 2008 [15] "
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    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.
    Full-text · Article · Aug 2013 · International journal of clinical and experimental pathology
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    • "According to a report in 2007, seven of 14 patients with primary CNS ALCL died; two of them had ALK(+)ALCL, three had ALK(-)ALCL and the remaining two did not go through the test for ALK positivity.5 Given the insufficient sample size to be statistically significant, prognosis of primary CNS ALK(+)ALCL compared to ALK(-)ALCL is inconclusive.5,9,11 In addition, there are no statistically significant data on prognosis of primary CNS ALCL compared to CNS lymphomas in general, but the mortality of the former seemed to be greater than that of the latter.5,17 "
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    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.
    Full-text · Article · May 2013 · Yonsei medical journal
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    ABSTRACT: Primary Central Nervous System Lymphoma (PCNSL) is an extremely rare condition in childhood as compared to adults. It is seen in immunocompromised patients. We report the case of PCNSL in a child in Civil Hospital, Solapur which is a district anti-retroviral treatment (ART) centre. This 15 months old child presented with focal neurological deficit. The CT and MRI scan were suggestive of PCNSL. Stereostatic biopsy could not be done in this child. But CSF study showed 20 cells and all were lymphoblasts.
    No preview · Article · · Journal of Krishna Institute of Medical Sciences University
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