ArticleFrontiers in oncology2026
Case Report: Recurrent malignant phyllodes tumor with malignant peripheral nerve sheath tumor-like differentiation and loss of H3K27me3/RB1 expression.
Article in Frontiers in oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Malignant phyllodes tumor (MPT) is a rare breast neoplasm with high recurrence rates and potential for heterologous sarcomatous differentiation. Malignant peripheral nerve sheath tumor (MPNST)-like differentiation in MPT is exceptionally rare and presents considerable diagnostic and therapeutic challenges. We report a case of a 24-year-old female with recurrent MPT exhibiting MPNST-like differentiation and integrated immunophenotypic and genomic characterization. The patient initially presented in November 2023 with a 3.8 cm right breast MPT treated by wide local excision. Five months later, ultrasound revealed local recurrence (5.8 cm), prompting right mastectomy in April 2024. Sixteen months after mastectomy, a 1 cm right chest wall nodule was identified and completely excised. Pathological examination revealed a high-grade spindle cell malignant neoplasm with MPNST-like morphology, brisk mitotic activity (>10 mitoses/10 HPF), and a Ki-67 index of 80-90%. Immunohistochemistry demonstrated focal S100 positivity, partial SOX10 expression, complete loss of H3K27me3, and absent RB1 protein expression, with cytoplasmic β-catenin positivity supporting phyllodes stromal lineage. A structured differential diagnosis excluded metaplastic spindle cell carcinoma, monophasic synovial sarcoma, fibromatosis/desmoid-type fibromatosis, myofibroblastic sarcoma, undifferentiated pleomorphic sarcoma, and primary
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