Kawasaki Tomonori, Tashima Tomoaki, Onohara Kojiro, Hirano Yasumitsu, Yamato Misuzu, Shirotake Suguru, Torigoe Tomoaki, Yazawa Yasuo, Hirasaki Masataka, Wako Masanori, Fujimaki Taro, Ichikawa Jiro
Department of Pathology, Saitama Medical University International Medical Center, Saitama, Japan.
Department of Gastroenterology, Saitama Medical University International Medical Center, Saitama, Japan.
Front Oncol. 2024 Nov 11;14:1425809. doi: 10.3389/fonc.2024.1425809. eCollection 2024.
Dedifferentiated liposarcoma is a malignant lipomatous tumor that rarely occurs in the gastrointestinal tract, including the ileocecal region. In this case, computed tomography and magnetic resonance imaging showed no fatty mass located in the mesenteric or submucosal lesion, and positron emission tomography-computed tomography showed a high maximum standardized uptake value, collectively indicating the gastrointestinal stroma tumor and lymphoma. The pathological findings resemble leiomyosarcoma; the immunohistochemistry findings including mouse double minute 2 homolog and cyclin D-dependent kinase-4 and amplification of mouse double minute 2 homolog in fluorescence hybridization just favored the diagnosis of dedifferentiated liposarcoma with leiomyosarcoma phenotype and not leiomyosarcoma. Recently, a new inhibitor for mouse double minute 2 homolog and cyclin D-dependent kinase-4 has been developed, and clinical trials for dedifferentiated liposarcoma are currently ongoing. This could change the treatment strategy drastically compared with other soft tissue sarcomas. Hence, a correct diagnosis of dedifferentiated liposarcoma is required.
去分化脂肪肉瘤是一种罕见发生于胃肠道包括回盲部的恶性脂肪瘤性肿瘤。在该病例中，计算机断层扫描和磁共振成像显示肠系膜或黏膜下病变处无脂肪肿块，正电子发射断层扫描-计算机断层扫描显示最大标准化摄取值较高，综合提示为胃肠道间质瘤和淋巴瘤。病理结果类似平滑肌肉瘤；免疫组织化学结果包括小鼠双微体2同源物和细胞周期蛋白D依赖性激酶4以及荧光杂交中小鼠双微体2同源物的扩增仅支持诊断为具有平滑肌肉瘤表型的去分化脂肪肉瘤而非平滑肌肉瘤。最近，一种针对小鼠双微体2同源物和细胞周期蛋白D依赖性激酶4的新型抑制剂已被研发出来，目前针对去分化脂肪肉瘤的临床试验正在进行。与其他软组织肉瘤相比，这可能会极大地改变治疗策略。因此，需要对去分化脂肪肉瘤做出正确诊断。
