Department of Pediatrics, Peking University International Hospital, Beijing, China.
Retroperitoneal Tumours Centre, Peking University International Hospital, Beijing, China.
J Coll Physicians Surg Pak. 2022 Sep;32(9):1212-1215. doi: 10.29271/jcpsp.2022.09.1212.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare and aggressive inflammatory myofibroblastic tumour (IMT) variant. This report identifies the first case of EIMS with leukemoid reaction. This is also the first case in which pancreatic infiltration occurred from the disease onset. A 14-year male patient presented with an 18×18×10 cm mass at the retroperitoneal space and a white blood cell (WBC) count of 85×109/L. The mass and the invaded tissues were surgically removed with tumour-free margins. Histopathology and bone marrow aspiration confirmed the diagnosis of EIMS with leukemoid reaction. The tumour recurred with hepatic and pulmonary metastasis one month after the surgery. WBC count also increased progressively with the tumour recurrence. There is no consensus on the treatment of EIMS. Since ALK rearrangement presents in all the EIMS cases, surgical resection combined with crizotinib or other targeted drugs may improve the prognosis. Key Words: Sarcoma, Soft tissue neoplasms, Leukemoid reaction, Crizotinib.
上皮样炎性肌纤维母细胞肉瘤（EIMS）是一种罕见且侵袭性的炎性肌纤维母细胞瘤（IMT）变体。本报告确定了首例伴有类白血病反应的 EIMS 病例。这也是首例从发病开始就发生胰腺浸润的病例。一名 14 岁男性患者，腹膜后间隙出现 18×18×10 cm 大小的肿块，白细胞计数（WBC）为 85×109/L。肿块和受侵犯的组织通过无肿瘤边缘的手术切除。组织病理学和骨髓抽吸证实了伴有类白血病反应的 EIMS 的诊断。术后一个月，肿瘤复发并伴有肝和肺转移。随着肿瘤复发，WBC 计数也逐渐增加。对于 EIMS 的治疗尚无共识。由于所有 EIMS 病例均存在 ALK 重排，因此手术切除联合克唑替尼或其他靶向药物可能改善预后。关键词：肉瘤，软组织肿瘤，类白血病反应，克唑替尼。
