Na Yong-Sub, Park Sang-Gon
Department of Pulmonology, Chosun University Hospital, Gwangju, Republic of Korea.
Department of Hemato-oncology, Chosun University Hospital, 365 Pilmun-daero, Dong-gu, Gwangju, 61453, Republic of Korea.
J Med Case Rep. 2018 Sep 9;12(1):253. doi: 10.1186/s13256-018-1796-7.
Inflammatory myofibroblastic tumor is a rare benign neoplasm that frequently involves the lung and abdominopelvic region, and is found mainly in children and young adults. Inflammatory myofibroblastic tumor tends to be locally invasive or recurrent, and rarely metastasizes.
A 76-year-old Korean man presented with a history of upper back pain for 2 months and motor weakness in both lower extremities for 2 days. Contrast-enhanced computed tomography of his chest and abdomen showed a large heterogeneous pleural mass involving the right fifth rib and vertebral body and a mass infiltrating the right renal hilum. Computed tomography-guided percutaneous needle biopsy of the pleural mass was performed. The histological findings on hematoxylin and eosin staining showed proliferation of spindle cells with infiltration of lymphocytes and plasma cells. Immunohistochemistry showed neoplastic cells positive for CD68, focally positive for smooth muscle actin, and negative for cytokeratin and desmin. Inflammatory myofibroblastic tumor was diagnosed based on the histological examination. Treatment with glucocorticoids (methylprednisolone 1 mg/kg) and radiotherapy (5 days/week for 3 weeks at 3 Gy/fraction, 45 Gy/15 days) was started. After 1 month, chest computed tomography showed a reduction in the size of the pleural mass, and abdominopelvic computed tomography showed decreased infiltration around the right renal pelvis.
Inflammatory myofibroblastic tumor is a rare neoplasm of intermediate malignant potential due to a tendency for local recurrence and it rarely develops distant metastases. Complete surgical resection is the primary treatment. However, unresectable and metastatic inflammatory myofibroblastic tumor can be treated with systemic therapy, including glucocorticoids, radiotherapy, and/or chemotherapy.
炎性肌纤维母细胞瘤是一种罕见的良性肿瘤，常累及肺和腹盆腔区域，主要见于儿童和年轻人。炎性肌纤维母细胞瘤往往具有局部侵袭性或复发性，很少发生转移。
一名76岁的韩国男性，有2个月的上背部疼痛病史和2天的双下肢运动无力。胸部和腹部增强计算机断层扫描显示一个巨大的异质性胸膜肿块，累及右第五肋骨和椎体，以及一个浸润右肾门的肿块。对胸膜肿块进行了计算机断层扫描引导下的经皮针吸活检。苏木精和伊红染色的组织学结果显示梭形细胞增殖，伴有淋巴细胞和浆细胞浸润。免疫组织化学显示肿瘤细胞CD68阳性，平滑肌肌动蛋白局灶性阳性，细胞角蛋白和结蛋白阴性。根据组织学检查诊断为炎性肌纤维母细胞瘤。开始使用糖皮质激素（甲泼尼龙1mg/kg）和放疗（每周5天，共3周，每次3Gy，15天共45Gy）进行治疗。1个月后，胸部计算机断层扫描显示胸膜肿块大小缩小，腹盆腔计算机断层扫描显示右肾盂周围浸润减少。
炎性肌纤维母细胞瘤是一种罕见的具有中等恶性潜能的肿瘤，因其有局部复发倾向，很少发生远处转移。完整的手术切除是主要治疗方法。然而，不可切除和转移性炎性肌纤维母细胞瘤可采用全身治疗，包括糖皮质激素、放疗和/或化疗。
