Evolution of a Pediatric Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma on Serial MRI

Abstract

Background: Primary central nervous system lymphoma (PCNSL) is a rare central nervous system tumor, especially in the pediatric population. There are fewer than 20 described cases of pediatric primary central nervous system anaplastic large cell lymphoma. The child described in our case report demonstrated a dramatic evolution of this tumor in the first 4 weeks on serial imaging. Methods: Serial MRI imaging was performed followed by biopsy and chemotherapy. Results: Initial imaging revealed a T2 hyperintense lesion in the frontal lobe with abnormally enhancing sulci and minimal surrounding edema and diffusion restriction. Serial imaging revealed progressive increase in the degree of gadolinium enhancement, and the hyperintense T2 edema progressed markedly to exert mass effect. The lesion itself grew marginally. Biopsy revealed an anaplastic large cell lymphoma, only described in 14 previous pediatric patient case reports. The patient was successfully treated with chemotherapy and autologous stem cell transplant. Conclusions: Our case demonstrates the rapidity with which a PCNSL lesion can develop, and the evolution of the imaging characteristics prior to definitive diagnosis and treatment. Serial imaging by MRI may help differentiate the behavior of a PCNSL from other imitating lesions.

Pediatric Anaplastic Large Cell Lymphoma with Concomitant Involvement of Spine and Central Nervous System: A Case Report and Review of Literature

“…CNS involvement in children with ALCL is also extremely rare, both as primary and secondary localization: in an international study of systemic childhood ALCL [21], CNS disease was recognized in only 2.6% of cases, most of them being secondary involvement. After a review of English literature, we identified 23 pediatric cases of primary CNS ALCL (Table 4) [21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38]. Age ranged from 23 months to 18 years (median 11 years).…”

Primary anaplastic large cell lymphoma of the central nervous system in a child

“…In previous studies, about 83% of the cases were diagnosed with CNS infection for the first time. [ 10 ] This case presented an 8-year-old boy with headache and persistent intracranial hypertension as the prominent manifestation. At the beginning of the disease, white blood cells in the CSF increased slightly.…”

“…[ 21 ] In children, the proportion of intracranial hypertension is similar to that in adults. [ 10 ] Previously, 4 cases of PCNSL with intracranial hypertension were reported, of which, 1 deceased due to increased intracranial pressure. Although continuous CSF drainage in the lumbar cistern can be relieved temporarily, the viscous CSF leads to poor drainage, and the patient died of cerebral hernia in 8 weeks.…”

Primary central nervous system anaplastic large cell lymphoma in children: Case presentation and systematic review of literature

“…The enhancement pattern does not clearly distinguish it from meningiomas, metastasis, or gliomas. 4 A 9-year-old Caucasian male was admitted to our oncology department with a history of seizures, asthenia, and dizziness from 2 weeks.…”

“…Only other nine pediatric cases of primary CNS ALCL were reported in literature (Supporting Materials, Supplemental Table S1). [4][5][6][7][8][9][10][11][12] In five out of nine, the initial clinical suspicion concerned an infectious disease (Supporting Material S2).…”