Liu Dan, Wang Yun, Zheng Yu, Zhang Han-Lu, Wang Zi-Hao
Division of Thoracic Surgery, West China Hospital of Sichuan University, Chengdu City.
Department of The First Surgery, The Traditional Chinese Medical Hospital, AnYue City, Sichuan Province, PR China.
Medicine (Baltimore). 2020 Jan;99(5):e18992. doi: 10.1097/MD.0000000000018992.
Malignant solitary fibrous tumor (MSFT) of the diaphragm is extremely rare, and to the best of our knowledge, only three cases have been reported in the past two decades. In all these cases, the diaphragms were usually reconstructed with artificial diaphragm patch because of the extensive resection.
We reported a male patient with complaints of dyspnea, chest pain and massive pleural effusion in the left chest detected by chest X-ray. A huge mass of 20 × 20 cm was seen in the left lower chest in the computed tomography (CT) scan.
The diagnosis of MSFT originating in the diaphragm was made by post-operative immunohistochemical examination.
After draining 4000 ml of pleural effusion by Pleurx catheter to relieve the pressure symptom, the patient underwent en-block resection by left posterolateral thoracotomy. A pedicle tumor originating in the left diaphragm was found, which was smooth, lobular, did not invade surrounding tissues or organs, and received blood supply from the left phrenic vessels. The diaphragm was successfully sutured without tension and did not require artificial reconstruction as the defect was small.
After 2 months follow-up, the left lung was restored to normalcy, and no pleural effusion or new occupying neoplasm was found in follow-up CT.
膈肌恶性孤立性纤维瘤（MSFT）极为罕见，据我们所知，在过去二十年中仅报道过三例。在所有这些病例中，由于广泛切除，膈肌通常用人造膈肌补片进行重建。
我们报告了一名男性患者，主诉呼吸困难、胸痛，胸部X线检查发现左胸大量胸腔积液。计算机断层扫描（CT）显示左下胸部有一个20×20厘米的巨大肿块。
术后免疫组化检查确诊为起源于膈肌的MSFT。
通过Pleurx导管引流4000毫升胸腔积液以缓解压迫症状后，患者接受左后外侧开胸整块切除。发现一个起源于左膈肌的带蒂肿瘤，表面光滑，呈分叶状，未侵犯周围组织或器官，由左膈血管供血。膈肌成功无张力缝合，由于缺损小，无需人工重建。
随访2个月后，左肺恢复正常，随访CT未发现胸腔积液或新的占位性肿瘤。
