Reza Ershadi, Hesam Amini, Sara Soltanmohammadi, Shahab Rafieian
Department of Thoracic Surgery, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.
Department of Pulmonology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.
Int J Surg Case Rep. 2023 Feb;103:107912. doi: 10.1016/j.ijscr.2023.107912. Epub 2023 Feb 3.
fibrous dysplasia is a slow-growing bone tumor and is caused by the failure of bone maturation. It is usually asymptomatic thus it is generally found incidentally in radiologic evaluations. Computed tomography is the best radiologic modality for its evaluation. The characteristic findings are ground-glass lesions surrounded by a rim or shell of reactive bone.
This study presents a 52-year-old male patient with a huge chest wall tumor arising from the posterolateral aspect of the right fourth to seventh ribs measuring 38 cm. He underwent a thoracotomy and the tumor was resected. For safe margin, the third and eighth ribs were also resected. The defect was reconstructed with a prolene mesh patch and a pectoralis major flap. The final pathology report stated a tumor composed of spindle cells without pleomorphism or mitotic figures with intervening branching and anastomosing bone trabeculae. The margins were tumor-free and on the follow-up, the patient's condition was decent.
Primary tumors of the rib account for 5 % to 7 % of all primary bone neoplasms. Fibrous dysplasia makes up 0.8 % of primary bone tumors. Fibrous dysplasia usually causes no symptoms although it can get massive enough to get symptomatic. Its diagnosis is made through clinical, radiological, and histopathological investigations. CT scan findings are the cornerstone for radiologic evaluations. An individualized approach based on the patient's age and symptoms should be considered.
Considering that the malignant degeneration of the tumor is uncommon, early diagnosis and surgical resection of the tumor can be curative.
骨纤维异常增殖症是一种生长缓慢的骨肿瘤,由骨成熟障碍引起。它通常无症状,因此一般在影像学检查中偶然发现。计算机断层扫描是评估该病的最佳影像学方法。其特征性表现为磨砂玻璃样病变,周围有一层反应性骨形成的边缘或骨壳。
本研究报告了一名52岁男性患者,其右胸壁后外侧第四至第七肋骨处出现一个巨大的胸壁肿瘤,大小为38厘米。他接受了开胸手术,肿瘤被切除。为确保切缘安全,第三和第八肋骨也被切除。缺损处用聚丙烯网片和胸大肌瓣进行了重建。最终病理报告显示肿瘤由梭形细胞组成,无多形性或有丝分裂象,其间有分支和吻合的骨小梁。切缘无肿瘤残留,随访时患者情况良好。
肋骨原发性肿瘤占所有原发性骨肿瘤的5%至7%。骨纤维异常增殖症占原发性骨肿瘤的0.8%。骨纤维异常增殖症通常无症状,尽管其体积可能增大到出现症状。其诊断需通过临床、影像学和组织病理学检查。CT扫描结果是影像学评估的基石。应考虑根据患者年龄和症状采取个体化治疗方法。
鉴于该肿瘤恶变罕见,早期诊断并手术切除肿瘤可治愈。