Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Institute of Hematology, Rabin Medical Center, Petah-Tikva, Israel.
Acta Haematol. 2021;144(2):229-235. doi: 10.1159/000509723. Epub 2020 Oct 5.
Histiocytic sarcoma (HS) is a rare, malignant, and aggressive subtype of histiocytosis. We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss potential targeted approaches to treat these rare tumors.
组织细胞肉瘤（HS）是一种罕见的、恶性的、侵袭性的组织细胞增生症亚型。我们报告了一例罕见的侵袭性 HS，其发生于胃肠道和胆囊，并在经历数线化疗后进展为白血病期。我们在本病例中回顾了 HS 的临床、病理和分子特征，并对涉及消化系统的 HS 文献以及该病的明显白血病期文献进行了复习。HS 通常在晚期诊断，死亡率高。我们讨论了 HS 患者的治疗方法。我们强调 RAF-MEK-ERK 通路中的过度表达和体细胞改变在 HS 发病机制中的作用，并讨论了治疗这些罕见肿瘤的潜在靶向方法。
