Margarint Irina Maria, Youssef Tammam, Filip Cristina, Bizubac Ana-Mihaela, Popescu Alexandru, Rotaru Iulian, Untaru Olguta, Manolache Stefan, Iliescu Vlad Anton, Vladareanu Radu
Faculty of Medicine, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania.
Department of Cardiac Surgery, Emergency Clinical Hospital for Children "Maria Skłodowska Curie", 077120 Bucharest, Romania.
Life (Basel). 2025 Jan 17;15(1):123. doi: 10.3390/life15010123.
Desmoid tumors are a rare entity, especially in the pediatric population. There are no reports of such a tumor in newborns. They are associated with high rates of morbidity and mortality, even though they are benign soft tissue tumors. This is due to them exhibiting locally aggressive growth with the compression or invasion of adjacent structures. Abdominal localization is most commonly reported, although there are reports of mediastinal desmoid tumors. We present the case of a 6-day male patient with a mediastinal desmoid tumor that led to severe aortic coarctation with hemodynamic instability. The tumor also compressed the left pulmonary artery and obstructed the left main bronchus. The initial management consisted of successful emergency surgery with partial resection of the tumor mass and coarctation repair. In the postoperative setting, the patient evolved with severe respiratory dysfunction which was managed with tracheostomy, allowing weaning the child from the mechanical ventilation one month after surgery, along with chemotherapy. We also review the literature, focusing on the management of desmoid tumors.
硬纤维瘤是一种罕见的疾病,在儿科人群中尤为罕见。目前尚无新生儿患此类肿瘤的报道。尽管它们是良性软组织肿瘤,但发病率和死亡率却很高。这是因为它们表现出局部侵袭性生长,会压迫或侵犯相邻结构。虽然有纵隔硬纤维瘤的报道,但最常见的部位是腹部。我们报告了一例6天大的男性患者,患有纵隔硬纤维瘤,导致严重的主动脉缩窄并伴有血流动力学不稳定。肿瘤还压迫了左肺动脉并阻塞了左主支气管。初始治疗包括成功的急诊手术,部分切除肿瘤肿块并修复缩窄。术后,患者出现严重的呼吸功能障碍,通过气管切开术进行处理,术后一个月患儿得以脱离机械通气,并接受了化疗。我们还回顾了文献,重点关注硬纤维瘤的治疗。