Machado Isidro, Martí Ibor Encarnación, Berbegall Ana P, Alcácer Fernández-Coronado Javier, Claramunt Reyes, Duarte Novo Jose Enrique, Mosquera Reboredo Juan, Concha López Ángel, Alcácer García Javier
Departamento de Patología, Instituto Valenciano de Oncología, Valencia, España; Departamento de Patología, Patologika Laboratorio, Hospital Quirón-Salud, Valencia, España.
Departamento de Patología, Patologika Laboratorio, Hospital Quirón-Salud, Valencia, España; Departamento de Patología, Hospital Peset Alexandre, Valencia, España.
Rev Esp Patol. 2022 Jul-Sep;55(3):212-217. doi: 10.1016/j.patol.2021.07.002. Epub 2021 Oct 7.
We report the histological, immunohistochemical, and molecular findings of a dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features occurring in the paratesticular region. Histologically, the dedifferentiated component closely resembled an inflammatory myofibroblastic tumor. The neoplastic cells were positive for smooth muscle actin with focal CD56, CD99, Bcl2 and EMA expression. WT1, calretinin, myogenin, CK(AE1/AE3), desmin, H-caldesmon, CD34, ALK, CKIT, DOG1, MUC4 and STAT6 were negative. MDM2 showed diffuse and strong nuclear positivity in neoplastic cells and fluorescence in situ hybridization (FISH) revealed amplified MDM2 (high level) but no SYT rearrangement. Although a lipomatous component was evident macroscopically, well-differentiated liposarcomatous components were not evident in the section examined. Dedifferentiated liposarcoma can have prominent inflammatory myofibroblastic tumor-like features. Pathologists should be aware of this histological variant in order to avoid misdiagnosing dedifferentiated liposarcoma as inflammatory myofibroblastic tumor or other spindle cell tumors which have different behavioral patterns and treatment requirements.
我们报告了一例发生于睾丸旁区域的具有炎性肌纤维母细胞瘤样特征的去分化脂肪肉瘤的组织学、免疫组织化学及分子学检查结果。组织学上,去分化成分与炎性肌纤维母细胞瘤极为相似。肿瘤细胞平滑肌肌动蛋白呈阳性,局灶性表达CD56、CD99、Bcl2和上皮膜抗原(EMA)。WT1、钙视网膜蛋白、生肌调节因子、细胞角蛋白(CK[AE1/AE3])、结蛋白、H-钙调蛋白、CD34、间变性淋巴瘤激酶(ALK)、原癌基因c-KIT、盘状结构域受体1(DOG1)、黏蛋白4(MUC4)和信号转导及转录激活因子6(STAT6)均为阴性。MDM2在肿瘤细胞核内呈弥漫性强阳性,荧光原位杂交(FISH)显示MDM2基因扩增(高水平),但无SYT重排。尽管大体上可见脂肪成分,但在所检查的切片中未发现高分化脂肪肉瘤成分。去分化脂肪肉瘤可具有显著的炎性肌纤维母细胞瘤样特征。病理学家应了解这种组织学变异,以避免将去分化脂肪肉瘤误诊为炎性肌纤维母细胞瘤或其他具有不同行为模式和治疗需求的梭形细胞肿瘤。