Clinical characteristics of the ALK fusion-positive bladder PMP and efficacy of lorlatinib: a two-case report and literature review

Pseudosarcomatous myofibroblastic proliferation (PMP) is a rare anaplastic lymphoma kinase (ALK), ALK fusion-associated tumor-like lesion that requires differentiation from inflammatory myofibroblastic tumor (IMT). This article reports two cases of bladder ALK fusion-positive PMP: a 16-year-old male achieved partial remission (PR) postoperatively with lorlatinib but experienced memory decline and mood disturbances, which resolved after dose reduction; a 34-year-old female showed significant tumor shrinkage (PR) without severe adverse reactions. The study confirms the marked efficacy of ALK inhibitors in ALK-positive PMP, but highlights the need to monitor neurotoxicity in adolescent patients. Through analysis of the FN1 (Fibronectin 1)-ALK fusion mechanism and literature review, this study emphasizes the importance of pathological differentiation, individualized treatment, and dynamic cognitive monitoring, providing insights for precision therapy in rare diseases.

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Publication Details

Journal
The Oncologist
Published
2025-11-20
DOI
https://doi.org/10.1093/oncolo/oyaf389
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
Field-Weighted Citation Impact
0.00

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article

Clinical characteristics of the ALK fusion-positive bladder PMP and efficacy of lorlatinib: a two-case report and literature review

Sheng Zhang, Ning Huang, Mengyang Ju, Hao Zhang et al.
The Oncologist
IgG4-Related and Inflammatory Diseases
article

Clinical characteristics of the ALK fusion-positive bladder PMP and efficacy of lorlatinib: a two-case report and literature review

Sheng Zhang, Ning Huang, Mengyang Ju, Hao Zhang, Meng Sun, Mingjuan Sun
article en

Abstract

Pseudosarcomatous myofibroblastic proliferation (PMP) is a rare anaplastic lymphoma kinase (ALK), ALK fusion-associated tumor-like lesion that requires differentiation from inflammatory myofibroblastic tumor (IMT). This article reports two cases of bladder ALK fusion-positive PMP: a 16-year-old male achieved partial remission (PR) postoperatively with lorlatinib but experienced memory decline and mood disturbances, which resolved after dose reduction; a 34-year-old female showed significant tumor shrinkage (PR) without severe adverse reactions. The study confirms the marked efficacy of ALK inhibitors in ALK-positive PMP, but highlights the need to monitor neurotoxicity in adolescent patients. Through analysis of the FN1 (Fibronectin 1)-ALK fusion mechanism and literature review, this study emphasizes the importance of pathological differentiation, individualized treatment, and dynamic cognitive monitoring, providing insights for precision therapy in rare diseases.

The OncologistVol. 30(12)
Shanghai Medical College of Fudan University (CN), Fudan University Shanghai Cancer Center (CN)
National Key Research and Development Program of China
Openalex Percentile: Top 49%
IgG4-Related and Inflammatory Diseases
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