Iwabuchi Haruko, Kawashima Hiroyuki, Umezu Hajime, Takachi Takayuki, Imamura Masaru, Saitoh Akihiko, Ogose Akira, Imai Chihaya
Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuou-ku, Niigata, Niigata, 951-8510, Japan.
Department of Orthopedic Surgery, Niigata University Graduate School Medical and Dental Sciences, Niigata, Japan.
Int J Hematol. 2017 Aug;106(2):299-303. doi: 10.1007/s12185-017-2202-8. Epub 2017 Feb 28.
Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle. The patient has remained in complete remission more than 7 years from diagnosis.
组织细胞肉瘤是一种罕见的造血系统肿瘤，有组织细胞分化的证据，通常对传统化疗和放疗耐药，其预后一般较差。这种恶性肿瘤的最佳治疗方案尚未确定。我们报告一例8岁女童，患有累及左股骨的组织细胞肉瘤。该肿瘤对克拉屈滨和高剂量阿糖胞苷联合治疗迅速产生反应，这是一种用于难治性朗格汉斯细胞组织细胞增多症的积极挽救方案，在第一个疗程中肿瘤就变得触诊不到。该患者自诊断以来已完全缓解超过7年。
