ALK-positive ALCL: first described adult case of synchronous CNS and systemic involvement at presentation: a case report and review of the literature.

Ju W, De Tommasi C, Wernick M, Chai K.

ReviewAbstract only — full text under license

AI summary — for doctors, generated from the abstract only

A case report describes the first documented adult case of a rare lymphoma (ALK-positive ALCL) presenting simultaneously in the brain and body, successfully treated to complete remission with combination chemotherapy and stem cell transplant.

What was studied

A single case report of a 31-year-old woman who presented with headache and seizures and was found to have a brain lesion that, after craniotomy and pathological analysis, was ultimately diagnosed as ALK-positive anaplastic large cell lymphoma (ALCL) with simultaneous central nervous system and systemic involvement, treated with a modified chemotherapy protocol and carmustine-based autologous stem cell transplant.

Key findings

Initial pathological diagnosis was unclear, with ALK-positive histiocytosis initially favored and ALK-positive ALCL considered as a differential diagnosis.
The patient was treated with a modified MARIETTA chemotherapy protocol (without rituximab) followed by carmustine-based autologous stem cell transplantation.
End-of-treatment PET-CT and brain MRI showed a complete metabolic response, indicating successful treatment.
The authors state this is the first reported case of synchronous CNS and systemic ALK-positive ALCL presentation in an adult.

Clinical relevance

The findings suggest that this specific, very rare lymphoma presentation can achieve complete remission with a multidisciplinary combination treatment approach, which may be useful information for clinicians encountering similarly unusual or diagnostically uncertain brain lesions with a lymphoma differential.

Who should care

Neurosurgery

Limitations

This is a single case report, so the treatment approach and outcome cannot be generalized to other patients with this rare presentation.

A rare, first-described case of simultaneous brain and systemic ALK-positive ALCL was successfully treated to complete remission with combination chemotherapy and stem cell transplant, highlighting the value of multidisciplinary diagnosis for unusual brain lesions.

This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.

Abstract

Background

Anaplastic large cell lymphoma (ALCL) represents a group of heterogenous CD30+ mature T Cell lymphomas, which vary in their clinical, histological, and molecular characteristics. A majority of ALCL are ALK positive, which is characterised by translocation t(2;5) leading to the fusion of the ALK gene to the nucleophosmin gene. CNS presentations of ALCL are rare and restricted to case reports. A synchronous CNS and systemic presentation in an adult has never been reported.

Case presentation

A 31-year-old woman presented with headache and tonic-clonic seizures. MRI revealed a 15-mm enhancing left frontal convexity lesion with broad dural contact. A left frontal craniotomy was performed, and pathological analysis was conducted. Initially the diagnosis was unclear, with the most likely diagnosis being thought to be ALK-positive histiocytosis, with a differential of ALK-positive ALCL. The patient was treated with a modified MARIETTA protocol (without rituximab) and consolidated with a carmustine-based autologous stem cell transplant. End of treatment PET-CT and MRI brain showed a complete metabolic response. shows complete metabolic response on PET-CT and MRI brain.

Conclusions

This case presents the first successful treatment of a synchronous presentation of CNS and systemic ALK-positive ALCL, and the importance of a multidisciplinary approach for diagnosis.

DOI

10.1080/02688697.2025.2611165

PMID

41619778

Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.

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