Central nervous system involvement in anaplastic large cell lymphoma in childhood: results from a multicentre European and Japanese study.
performance status was 2 and Ann Arbor stage III. Inguinal node biopsy demonstrated neoplastic cells that were immunopositive for CD30, CD5, CD45RO, and immunonegative for CD3, CD8, CD10, and CD15. Cytogenics evaluation revealed t(2;5)(p23;q35). Subsequent ALK analysis was positive, and a final diagnosis of ALK+ ALCL, common monomorphic type was established. She was started on CHOP chemotherapy (cyclophosphamide, Adriamycin, vincristine and prednisone) cycled every 3-weeks. After 2 cycles she had a Positron emission tomography–computed tomography (PET–CT), which showed good response and a decrease in size of all the enlarged nodes. She presented with a 3-day history of fever, chills, nausea, vomiting, and headache 1 week after her third cycle. An infectious cause was initially pursued by workup at readmission. This evaluation included a magnetic resonance imaging (MRI) of the brain and subsequent lumbar puncture. MRI (Fig. 1) demonstrated early intracranial leptomeningeal disease. Bedside lumbar puncture revealed no evidence of cerebrospinal fluid (CSF)-based infection or lymphoma. However, empiric cefepime and vancomycin were initiated for possible meningitis without any improvement in symptoms. Based on infectious disease consult recommendations, full treatment with cefepime, vancomycin, anti-TB therapy, voriconazole, ampicillin, and acyclovir was begun. No improvement was seen on the antibiotic regimen, and all infectious studies continued to be negative. On the second day of admission, the patient became lethargic. On day 4 she was stuporous, and by day 11 she was unresponsive. An Ommaya reservoir was placed on day 5 of her hospitalization. At that time, biopsy of the right frontal convexity brain and dura was done which did not demonstrate tumor. Repeat MRI (Fig. 2) performed on day 8 revealed more robust and widespread leptomeningeal disease due to frank leptomeningeal carcinomatosis. Introduction