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硬化性上皮样纤维肉瘤(SEF)是罕见的具有侵袭性的恶性肿瘤,其特点是上皮样细胞呈小巢状或条索状分布于致密的胶原纤维中。骨原发性SEF鲜见报道。由于致密的细胞外胶原基质可被当作骨样基质,易误诊为骨肉瘤,因此对于骨SEF的认识非常重要。近年来MUC4和SATB2抗体分别被应用于硬化性上皮样纤维肉瘤和骨肉瘤的病理诊断,骨肉瘤中SATB2阳性,MUC4阴性,软组织SEF中SATB2阴性、MUC4阳性。作者收集8例骨原发性SEF,探讨其临床病理学及免疫表型特征。其中男性5例,女性3例,年龄25~
Sclerotic epithelioid fibrosarcoma (SEF) is a rare and aggressive malignant tumor characterized by small nests or cords distributed in dense collagen fibers. Bone primary SEF rarely reported. Since dense extracellular collagen matrix can be used as a bone-like matrix, easily misdiagnosed as osteosarcoma, so the understanding of bone SEF is very important. In recent years, MUC4 and SATB2 antibodies were applied to the pathological diagnosis of sclerosing epithelioid fibrosarcoma and osteosarcoma respectively. SATB2 positive in osteosarcoma, MUC4 negative, SATB2 negative in soft tissue SEF and MUC4 positive. The authors collected 8 cases of bone primary SEF, to explore its clinical pathology and immunophenotypic characteristics. Including 5 males and 3 females, aged 25 ~