Schallenberg Simon, Schulte Miriam, Dragomir Mihnea P, Jarosch Armin, Hartmann Wolfgang, Wardelmann Eva
Institute of Pathology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany.
Gerhard-Domagk-Institut Für Pathologie, Universitätsklinikum Münster, Münster, Germany.
Diagn Pathol. 2025 Apr 10;20(1):39. doi: 10.1186/s13000-025-01640-3.
Dedifferentiated liposarcoma (DDLPS) with inflammatory myofibroblastic tumor (IMT)-like features is a rare and diagnostically challenging variant of soft tissue sarcoma. We report the case of a 74-year-old man who presented with a mesenteric mass in 2022 and recurrent tumors in 2024. Tissue from both primary and recurrent tumors were submitted to our reference center for pathological reevaluation, with a suspicion of IMT being suspected. Although the tumors exhibited morphological characteristics consistent with those observed in IMT, they displayed distinctive histological, immunohistochemical and molecular features suggestive of DDLPS with IMT-like features, including amplification of the MDM2 gene. This report highlights the morphological spectrum of DDLPS, the diagnostic role of molecular pathology, and the importance of differentiating this aggressive neoplasm from benign entities such as IMT.
具有炎性肌成纤维细胞瘤(IMT)样特征的去分化脂肪肉瘤(DDLPS)是一种罕见且诊断具有挑战性的软组织肉瘤变体。我们报告了一例74岁男性病例,该患者在2022年出现肠系膜肿块,2024年出现复发性肿瘤。原发肿瘤和复发性肿瘤的组织均被送至我们的参考中心进行病理重新评估,怀疑为IMT。尽管肿瘤表现出与IMT中观察到的形态特征一致,但它们显示出独特的组织学、免疫组化和分子特征,提示为具有IMT样特征的DDLPS,包括MDM2基因扩增。本报告强调了DDLPS的形态谱、分子病理学的诊断作用以及将这种侵袭性肿瘤与IMT等良性实体区分开来的重要性。