Case report: diagnostic challenges of primary central nervous system anaplastic large cell lymphoma, ALK-positive

Primary central nervous system lymphoma in children is rare, and the highly variable radiological presentation presents a significant diagnostic challenge. The reported subtype: anaplastic large cell lymphoma, kinase positive (ALCL, ALK+) is an exceedingly rare entity, with the largest case series comprising only 34 cases. Involvement of the choroid plexus is particularly uncommon, with only one previously reported case arising in the lateral ventricle. This case report highlights the diagnostic and management challenges associated with this unusual presentation and lesion location.

A 16-year-old male presented with a 3-week history of fever and vomiting followed by progressive neurological deterioration, including confusion and collapse. Brain computed tomography (CT) revealed significant vasogenic edema and a hyperdense lesion in the right lateral ventricle. Magnetic resonance imaging (MRI) demonstrated a lobulated contrast-enhancing choroid plexus lesion with entrapment of the temporal horn and midline shift. Biopsy confirmed the diagnosis of PCNS ALK + ALCL. Subsequent chemotherapy resulted in remission of the lesion by the time of last follow-up, and aggressive surgical resection was not required. The rarity of this lesion posed diagnostic difficulties clinically and radiologically.

This case illustrates the challenges of diagnosing childhood PCNSL, particularly when the lesion arises in an atypical location such as the choroid plexus. The excellent response to adjuvant therapy highlights the importance of considering this diagnosis in young patients with atypical brain lesions in order to avoid unnecessarily aggressive and potentially morbid surgical approaches.