Knaj Duaa, Ahmad Ghanem, Alshehabi Zuheir, Ahmad Issa Yusuof
Department of Oncology, Tishreen University, Latakia, Syrian Arab Republic.
Department of Vascular Surgery, Tishreen University, Latakia, Syrian Arab Republic.
Int Med Case Rep J. 2025 Jul 18;18:909-914. doi: 10.2147/IMCRJ.S535403. eCollection 2025.
Adamantinoma is a low-grade, primary malignant bone tumor commonly found in the tibia with or without fibular involvement.
A 21-year-old female patient was admitted to the hospital with a mass on the upper lateral aspect of the left leg. She reported the onset of a small swelling in the left leg eight months prior to admission. It has gradually increased in size to involve the upper leg and part of the knee. The patient had limited range of motion in the knee joint and difficulty leaning on the affected limb. Radiographs showed a cortical-destroying osteolytic lesion at the head of the fibula extending into the surrounding soft tissue. Ultrasound imaging showed a mixed echo formation with indistinct borders and multiple chambers separated by trabeculae demonstrating blood flow. MRI revealed the absence of the fibular head as evidenced by cortical destruction and extension into adjacent soft tissue. CT scan showed a 12×13 cm mass in the left popliteal region extending to the knee margin. Enlarged lymph nodes were noted in the left groin. Multiple small metastases were observed in the lungs. Histologically, atypical epithelial cells and stromal spindle cells were observed. CK and CK19 staining was positive. The patient was treated with Sunitinib 50 mg orally, once daily for 4 weeks, followed by 2 weeks off for 2 cycles. During treatment (after 2 cycles) the tumor showed progressive growth, resulting in decreased function of the affected limb. She was referred to the surgical department where the appropriate surgical amputation was performed. After surgery, the patient's general condition improved. A further CT scan revealed a slight progression of metastatic lesions in the lungs, which prompted the decision to administer chemotherapy, but she refused to continue the treatment and did not receive chemotherapy. Two months later, she presented to the hospital in poor general condition. CT scan revealed extensive and progressive lesions in both lungs, leading to chest pain, hemoptysis, and hypoxia. Despite supportive care, the patient eventually died.
This case report presents a rare case of metastatic adamantinoma that did not respond to treatment with Sunitinib.
骨化性纤维瘤是一种低度恶性的原发性骨肿瘤，常见于胫骨，可伴有或不伴有腓骨受累。
一名21岁女性患者因左腿上外侧肿块入院。她报告称入院前八个月左腿出现一个小肿块。肿块逐渐增大，累及大腿上部和部分膝关节。患者膝关节活动范围受限，患肢负重困难。X线片显示腓骨头有一个破坏皮质的溶骨性病变，延伸至周围软组织。超声成像显示边界不清的混合回声形成，多个腔隙被小梁分隔，有血流信号。MRI显示腓骨头缺失，表现为皮质破坏并延伸至相邻软组织。CT扫描显示左腘窝区有一个12×13 cm的肿块，延伸至膝关节边缘。左腹股沟区可见肿大淋巴结。肺部观察到多个小转移灶。组织学检查发现非典型上皮细胞和间质梭形细胞。CK和CK19染色呈阳性。患者接受舒尼替尼治疗，口服50 mg，每日一次，共4周，随后停药2周，进行2个周期。治疗期间（2个周期后）肿瘤呈进行性生长，导致患肢功能下降。她被转至外科进行了适当的截肢手术。术后患者一般情况改善。进一步的CT扫描显示肺部转移灶略有进展，促使决定进行化疗，但她拒绝继续治疗，未接受化疗。两个月后，她因一般情况较差入院。CT扫描显示双肺广泛且进展性病变，导致胸痛、咯血和缺氧。尽管给予了支持治疗，患者最终死亡。
本病例报告展示了一例罕见的转移性骨化性纤维瘤，对舒尼替尼治疗无反应。
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