Xu Kai, Zhao Qi-Kang, Liu Jing-Shan, Zhou Dong-Hai, Chen Yong-Liang, Zhu Xing-Yi, Su Ming, Huang Kun-Quan, Du Wen, Zhao Hong-Yu
Department of General Surgery, Shougang Hospital, Peking University, Beijing 100041, China.
Department of Hepatobiliary Surgery, Chinese People's Liberation Army General Hospital, Beijing 100853, China.
World J Clin Cases. 2020 Nov 26;8(22):5758-5764. doi: 10.12998/wjcc.v8.i22.5758.
Ligamentoid fibromatosis is a rare borderline tumor that occurs in the muscles, fascia, and aponeurosis. It is a kind of soft tissue tumor of fibrous origin, also known as invasive fibromatosis, desmoid fibroma, neurofibromatosis, . The tumor is between benign and malignant tumors and rarely has distant metastasis. Its characteristics are mainly local invasion, destruction and growth and easy recurrence. The World Health Organization defines it as a fibroblast cloning value-added lesion originating from deep soft tissue, which causes local invasion and growth leading to tissue reconstruction, extrusion and destruction of important structures and organs. The incidence rate accounts for 0.03% of all tumors and less than 3% of all soft tissue tumors. Definite diagnosis mainly depends on postoperative pathology. Surgical resection is still the main way to treat the disease, and a variety of nonsurgical treatment methods are auxiliary. Combined treatment can effectively reduce the risk of postoperative recurrence.
The patient is a 57-year-old female. One week ago, she accidentally found a mass in the left upper abdomen while lying flat. There was no abdominal pain and abdominal distention, no fever, no black stool and blood in the stool and no nausea and vomiting. She had a 10-year history of glaucoma on the left side, underwent hysterectomy for uterine fibroids 5 years ago, had no hypertension, heart disease, diabetes, hepatitis or tuberculosis, had no history of smoking and had been drinking for 20 years.
Accurate preoperative diagnosis is difficult, surgical resection is the main treatment, and a variety of nonsurgical treatment methods are auxiliary. Combined treatment can effectively reduce the risk of postoperative recurrence. The prognosis is still good, and the risk of recurrence of secondary surgery is greatly increased.
韧带样型纤维瘤病是一种罕见的交界性肿瘤,发生于肌肉、筋膜和腱膜。它是一种纤维源性软组织肿瘤,也称为侵袭性纤维瘤病、硬纤维瘤、神经纤维瘤病等。该肿瘤介于良性和恶性肿瘤之间,很少发生远处转移。其特点主要是局部侵袭、破坏和生长,且易复发。世界卫生组织将其定义为起源于深部软组织的成纤维细胞克隆性增殖性病变,可导致局部侵袭和生长,进而引起组织重塑、挤压并破坏重要结构和器官。发病率占所有肿瘤的0.03%,占所有软组织肿瘤的不到3%。明确诊断主要依靠术后病理。手术切除仍是治疗该病的主要方式,多种非手术治疗方法为辅。综合治疗可有效降低术后复发风险。
患者为一名57岁女性。一周前,她平躺时偶然发现左上腹有一肿块。无腹痛、腹胀,无发热,无黑便及便血,无恶心、呕吐。她左侧有10年青光眼病史,5年前因子宫肌瘤行子宫切除术,无高血压、心脏病、糖尿病、肝炎或结核病史,无吸烟史,饮酒20年。
术前准确诊断困难,手术切除是主要治疗方法,多种非手术治疗方法为辅。综合治疗可有效降低术后复发风险。预后仍良好,二次手术复发风险大幅增加。