背景:纤维样型纤维瘤病(DTF),也被称为侵袭性纤维瘤病,是一种罕见的肿瘤,起源于筋膜或肌膜膜组织。虽然是良性的,以缓慢的生长为特征,它表现出局部侵袭性,缺乏特定的临床特征。然而,在相当比例的患者中,它可能无症状,并在常规临床检查中意外发现。医学文献中只报道了少数由腰大肌引起的DTF病例。
方法:一名24岁男性,无症状且无明显的个人或家族病史,在常规体检中通过腹部超声检查诊断为2级肾积水。此诊断是在进行简单的心脏直视手术以修复房间隔缺损15天后做出的。
方法:静脉肾盂造影显示肾积水伴骨盆管系统扩张。输尿管镜检查排除了输尿管的任何内在病变。对比增强计算机断层扫描发现腹膜后有3.5×2×5.2cm肿块,与腰大肌密切相关,并包围与髂动脉相邻的输尿管。术后病理分析证实了散发性DTF的明确诊断。
方法:患者接受腹部探查手术,在此期间切除肿瘤,没有任何术中并发症。
结果:经过5年的密切监测,其中包括定期体检,磁共振成像,和超声检查,未发现局部复发.
结论:在涉及源自腰大肌并包裹输尿管的腹膜后肿瘤的病例中,实现准确的术前诊断是一个挑战。然而,双J支架的插入被认为是手术过程中的关键步骤,促进输尿管与肿瘤的解剖和隔离,同时保留肾功能。
BACKGROUND: Desmoid-type fibromatosis (DTF), also known as aggressive fibromatosis, is a rare neoplasm originating from the fascial or musculoaponeurotic tissues. While benign and characterized by slow growth, it exhibits local aggressiveness and lacks specific clinical characteristics. However, in a considerable percentage of patients, it could be asymptomatic and discovered by accident during routine clinical examinations. Only a few cases of DTF arising from the psoas major muscle have been reported in the medical literature.
METHODS: A 24-year-old male, asymptomatic and without significant personal or family medical history, was diagnosed with grade 2 hydronephrosis by abdominal ultrasonography during a routine physical examination. This diagnosis was made 15 days after undergoing uncomplicated open-heart surgery to repair an atrial septal defect.
METHODS: Intravenous pyelogram revealed hydronephrosis with dilation of the pelvicalyceal system. Ureteroscopy ruled out any intrinsic lesions of the ureter. Contrast-enhanced computed tomography identified a 3.5 × 2 × 5.2 cm mass in the retroperitoneum, closely associated with the psoas muscle and enveloping the ureter adjacent to the iliac artery. Postoperative pathological analysis confirmed a definitive diagnosis of sporadic DTF.
METHODS: The patient underwent exploratory abdominal surgery, during which the tumor was resected without any intraoperative complications.
RESULTS: After close monitoring over a 5-year follow-up period, which included periodic physical examinations, magnetic resonance imaging, and ultrasonography, no local recurrence was detected.
CONCLUSIONS: Achieving an accurate preoperative diagnosis presents a challenge in cases involving retroperitoneal tumors originating from the psoas major muscle and encasing the ureter. However, the insertion of a double J stent is deemed a crucial step in the surgical process, facilitating the dissection and isolation of the ureter from the tumor while preserving kidney function.