Annals of Oncology 13:1827-1832, 2002
© 2002 European Society for Medical Oncology
Clinical Investigations |
Primary brain CD30+ ALK1+ anaplastic large cell lymphoma (‘ALKoma’): the first case with a combination of ‘not common’ variants
Departments of 1 Pathology, 2 Hematology, 3 Radiochemotherapy, 4 Neuroradiology and 5 Neurosurgery, S Raffaele H Scientific Institute, Milan; 6 Department of Pathology, Ospedale S Maria Della Misericordia, Udine, Italy
Received 13 November 2001; revised 17 April 2002; accepted 8 May 2002
Abstract
Background:
Primary central nervous system lymphomas (PCNSLs) are rare tumors, mostly represented by diffuse large B cells. PCNSLs with a T phenotype are less frequently reported; even rarer are anaplastic large cell lymphomas (ALCLs). PCNSL ALCLs are commonly represented, like their systemic counterpart, by a variably prevalent amount of large pleomorphic tumor cells (‘hallmark cells’), and this feature enhances their recognition.
Patient and methods
We report the first case of primary brain CD30+ ALK-1+ ALCL with a T-cell phenotype, showing the combination of both the ‘lymphohistiocytic’ and the ‘small cell’ variants of the disease. A few elements consistent with ‘hallmark cells’ were recognizable. However, these cells were never prominent, increasing diagnostic difficulties. Immunohistochemistry results were critical for the correct interpretation. Our findings also differ from the majority of PCNSL ALCLs for the absence of tumor necrosis and the lack of prominent mitotic activity. The neuroimaging picture was not specific. A comparison with literature data concerning the clinical/instrumental features shows a very frequent meningeal involvement in PCNSL ALCLs, in contrast to the majority of PCNSLs.
Conclusion:
The occurrence of such a rare form of ALCL may widen the spectrum of differential diagnoses in PCNSL and their recognition may allow a rapid diagnosis, thus encouraging adequate treatment, which should take into account the high rate of meningeal involvement observed in these cases.
Key words: ALK, anaplastic, brain, central nervous system, hallmark cell, lymphoma
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