Hamdy Omar, Ayman Reem, Abdelaal Randa, Elhadidy Ransy, Tarek Mariam, Saleh Gehad Ahmad, Rafat Sara
Surgical Oncology Department, Oncology Center, Mansoura University, Mansoura, Egypt.
Faculty of Medicine, Mansoura University, Mansoura, Egypt.
J Chest Surg. 2024 Nov 5;57(6):547-552. doi: 10.5090/jcs.24.054. Epub 2024 Oct 22.
Diaphragmatic fibromatosis is an exceptionally rare condition, with only 2 previously reported cases in the literature. We present the case of a 23-year-old woman with a left hypochondrial swelling measuring 19×18×13 cm on computed tomography. An endoscopic ultrasound-guided biopsy indicated low-grade papillary proliferation. Surgical exploration revealed a large mass originating from the left hemidiaphragm. Complete excision of the mass and reconstruction of the diaphragm with double-layer mesh were performed. Microscopic examination of the mass revealed a bland-looking spindle cell proliferation. Immunohistochemical staining showed a positive nuclear reaction in tumor cells for β-catenin, a focal positive reaction for SMA, and negative reactions for S100, CD34, and desmin. Diaphragmatic fibromatosis is an extremely rare tumor for which complete excision and reconstruction of the diaphragm is the best suggested modality of treatment.
膈肌纤维瘤病是一种极其罕见的疾病，文献中此前仅报道过2例。我们报告一例23岁女性病例，计算机断层扫描显示左季肋部有一个大小为19×18×13 cm的肿块。内镜超声引导下活检显示为低度乳头状增生。手术探查发现一个起源于左半膈肌的大肿块。对肿块进行了完整切除，并使用双层网片对膈肌进行了重建。对肿块的显微镜检查显示为外观温和的梭形细胞增生。免疫组织化学染色显示肿瘤细胞中β-连环蛋白核反应阳性，平滑肌肌动蛋白局灶性阳性反应，而S100、CD34和结蛋白反应阴性。膈肌纤维瘤病是一种极为罕见的肿瘤，对于该病，膈肌完整切除并重建是最佳的建议治疗方式。
