Shao Mingyan, Fan Sisi, Qi Wanling, Luo Zhehuang, Xu Rong, Liao Fengxiang
Department of Nuclear Medicine, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China.
Department of Pathology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Nanchang, Jiangxi, China.
Front Med (Lausanne). 2024 Jul 31;11:1415042. doi: 10.3389/fmed.2024.1415042. eCollection 2024.
Angiomatoid fibrous histiocytoma (AFH) is a clinically rare, low-grade malignant soft tissue tumor that occasionally metastasizes. It accounts for 0.3% of all soft tissue tumors and most frequently occurs in the extremities, followed by the trunk, and the head and neck. Primary angiomatoid fibrous histiocytoma (PAFH) of the pulmonary bronchus is rare. In this paper, the clinical and imaging data of a case of PAFH of the pulmonary bronchus are reported, and the literature is reviewed.
A 57-year-old female patient presented with a six-month history of cough without apparent cause, characterized by paroxysmal dry cough, chest tightness, and shortness of breath, which worsened with activity. She did not experience fever, chills, chest pain, hemoptysis, or night sweats. Laboratory tests revealed an elevated C-reactive protein and ferritin levels, while tumor markers such as AFP, CEA, CA199, CA125, CA50, and T-SPOT were negative. A chest CT scan showed bronchial obstruction, atelectasis, and a soft tissue density in the right middle lobe of the lung. The enhanced scan demonstrated uneven enhancement of endobronchial nodules. An 18F-FDG PET/CT scan revealed a nodular soft tissue density shadow in the right lung bronchus with uneven density, clear boundaries, and increased 18F-FDG uptake, with a maximum standard uptake value (SUVmax) of 11.2. Bronchoscopy revealed a nodular or polypoid mass that was yellow and tough. Based on imaging findings, the preoperative diagnosis favored lung cancer. However, the postoperative pathological diagnosis confirmed primary angiomatoid fibrous histiocytoma (PAFH) of the pulmonary bronchus.
The incidence of primary angiomatoid fibrous histiocytoma (PAFH) is very low, and its clinical manifestations and imaging findings lack specificity, with the final diagnosis relying on pathology. PET/CT imaging has a certain value in the diagnosis of PAFH and holds significant application value in preoperative staging, postoperative efficacy evaluation, and follow-up monitoring. In conclusion, this case report further expands the spectrum of lung and bronchial tumors.
血管样纤维组织细胞瘤（AFH）是一种临床罕见的低度恶性软组织肿瘤，偶可发生转移。它占所有软组织肿瘤的0.3%，最常发生于四肢，其次为躯干以及头颈部。肺支气管原发性血管样纤维组织细胞瘤（PAFH）罕见。本文报道1例肺支气管PAFH的临床及影像资料，并复习相关文献。
一名57岁女性患者，无明显诱因出现咳嗽6个月，表现为阵发性干咳、胸闷、气短，活动后加重。无发热、寒战、胸痛、咯血或盗汗。实验室检查显示C反应蛋白和铁蛋白水平升高，而甲胎蛋白、癌胚抗原、CA199、CA125、CA50和T-SPOT等肿瘤标志物均为阴性。胸部CT扫描显示支气管阻塞、肺不张及右肺中叶软组织密度影。增强扫描显示支气管内结节强化不均匀。18F-FDG PET/CT扫描显示右肺支气管结节状软组织密度影，密度不均匀，边界清晰，18F-FDG摄取增加，最大标准摄取值（SUVmax）为11.2。支气管镜检查发现结节状或息肉样肿物，呈黄色且质地坚韧。基于影像学表现，术前诊断倾向于肺癌。然而，术后病理诊断证实为肺支气管原发性血管样纤维组织细胞瘤（PAFH）。
肺支气管原发性血管样纤维组织细胞瘤（PAFH）发病率极低，其临床表现及影像学表现缺乏特异性，最终诊断依赖于病理检查。PET/CT成像在PAFH诊断中具有一定价值，在术前分期、术后疗效评估及随访监测中具有重要应用价值。总之，本病例报告进一步拓宽了肺及支气管肿瘤谱。
