PDF(Pubmed)
Abstract：
Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare subtype of sarcoma that appears histologically low-grade yet usually has a clinically aggressive course with a high rate of local recurrence and distant metastasis. However, these recurrences and metastases often occur years after initial treatment. Metastases can be to the lung as well as extra-pulmonary sites. In this case report, we discuss a patient who developed SEF in the deep soft tissue with metastases. This patient underwent checkpoint inhibitor therapy, with disease response. Thus, SEF is a sarcoma subtype with a unique tumor biology, and immunotherapy may be a promising avenue for treatment.
摘要：
硬化性上皮样纤维肉瘤（SEF）是一种极为罕见的肉瘤亚型，在组织学上表现为低级别，但通常具有临床侵袭性病程，局部复发率和远处转移率高。然而,这些复发和转移通常在初始治疗后数年发生。转移可以是肺以及肺外部位。在这个案例报告中,我们讨论了一名在深部软组织发生SEF转移的患者。这名患者接受了检查点抑制剂治疗，疾病反应。因此，SEF是一种具有独特肿瘤生物学特性的肉瘤亚型,和免疫疗法可能是一个有希望的治疗途径。
