Zhou Li, Zhang Xinhui, Feng Shanglong, Zhao Na, Hu Xing, Huang Liangliang, Zheng Changcheng
Department of Hematology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, People's Republic of China.
Department of Hematology, Anhui Provincial Hospital, Anhui Medical University, Hefei, People's Republic of China.
Onco Targets Ther. 2020 Jan 9;13:237-241. doi: 10.2147/OTT.S230478. eCollection 2020.
Intracranial myeloid sarcoma is a very rare disease with poor prognosis. We report a case of a 28-year-old male patient who was admitted with intense headache, vision disturbance and severe vomiting in June 2017. He had a history of neurosurgical tumor resection operation in April 2017, and the pathological diagnosis was intracranial myeloid sarcoma. Bone marrow aspirate and biopsy had been conducted in May 2017, which demonstrated 5.5% blasts expressing CD13, CD33, CD34, CD117 and MPO, and the cytogenetic analysis demonstrated t(8;21)(q22;q22), and molecular studies showed a positive RUNX1-RUNX1T1 rearrangement. The diagnosis of acute myeloid leukemia (AML) with t (8; 21) (q22; q22)/RUNX1-RUNX1T1 was made, however, the patient refused to receive any systemic chemotherapy. Emergency cranial CT demonstrated a circular hyperdense mass (54mm×37mm), which was surrounded by hypodense peritumoral edema in the left cerebellar hemisphere, and the density of the lesions was uniform and the margin was clear. Idarubicin (12mg/m·d×3 days) combined with high-dose cytarabine (2g/m q12h×3 days) was initiated for emergency chemotherapy. All of the above symptoms disappeared at the end of chemotherapy. On the first day after chemotherapy, the cranial CT indicated that the cranial lesion was markedly reduced (20mm×15mm), and on the sixth day after chemotherapy, the lesion was completely disappeared. Currently, there are no clear guidelines for the treatment of intracranial myeloid sarcoma, and our treatment approaches could provide a reference for this disease with such emergency situation.
颅内髓系肉瘤是一种非常罕见且预后较差的疾病。我们报告一例28岁男性患者,于2017年6月因剧烈头痛、视力障碍和严重呕吐入院。他在2017年4月有神经外科肿瘤切除手术史,病理诊断为颅内髓系肉瘤。2017年5月进行了骨髓穿刺和活检,结果显示5.5%的原始细胞表达CD13、CD33、CD34、CD117和MPO,细胞遗传学分析显示t(8;21)(q22;q22),分子研究显示RUNX1-RUNX1T1重排阳性。诊断为急性髓系白血病(AML)伴t(8;21)(q22;q22)/RUNX1-RUNX1T1,然而,患者拒绝接受任何全身化疗。急诊头颅CT显示左小脑半球有一圆形高密度肿块(54mm×37mm),周围有低密度的瘤周水肿,病变密度均匀,边界清晰。开始使用伊达比星(12mg/m·d×3天)联合大剂量阿糖胞苷(2g/m q12h×3天)进行急诊化疗。化疗结束时上述所有症状均消失。化疗后第一天,头颅CT显示颅内病变明显缩小(20mm×15mm),化疗后第六天,病变完全消失。目前,对于颅内髓系肉瘤的治疗尚无明确指南,我们的治疗方法可为这种紧急情况的疾病提供参考。