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Evolution of a Pediatric Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma on Serial MRI
Mary J Dunbar1, Ash Singhal, Shahrad Rod Rassekh
1British Columbia Children's Hospital, University of British Columbia, Vancouver, B.C., Canada.
Insights
Pediatric primary central nervous system lymphoma (PCNSL) can rapidly evolve. Serial MRI imaging revealed dramatic changes in this rare anaplastic large cell lymphoma, aiding diagnosis and treatment.
Area of Science:
- Pediatric oncology
- Neuroradiology
- Hematology-oncology
Background:
- Primary central nervous system lymphoma (PCNSL) is a rare malignancy in children.
- Pediatric primary central nervous system anaplastic large cell lymphoma is exceptionally rare, with fewer than 20 reported cases.
- This case highlights the rapid progression of PCNSL in a pediatric patient within the first four weeks.
Observation:
- Initial MRI showed a T2 hyperintense frontal lobe lesion with enhancing sulci, minimal edema, and diffusion restriction.
- Serial imaging demonstrated progressive gadolinium enhancement and marked T2 hyperintense edema, leading to mass effect.
- The tumor size increased marginally, but imaging characteristics evolved rapidly.
Findings:
- Biopsy confirmed anaplastic large cell lymphoma, a rare diagnosis in pediatric PCNSL.
- The patient received successful treatment with chemotherapy and autologous stem cell transplant.
- Rapid evolution of imaging features was observed over four weeks.
Implications:
- This case underscores the rapid development and changing imaging characteristics of PCNSL in children.
- Serial MRI can be crucial in differentiating PCNSL from other central nervous system lesions.
- Early recognition of rapid tumor evolution on imaging is vital for timely diagnosis and intervention in pediatric PCNSL.
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.

