Department of Radiological Sciences-Oncology and Pathological Anatomy, and Department of Radiotherapy, University of Rome "La Sapienza", 00161 Rome, Italy.
World J Gastroenterol. 2012 Jun 28;18(24):3173-6. doi: 10.3748/wjg.v18.i24.3173.
A desmoid tumor, also known as aggressive fibromatosis, is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues. It can occur in any anatomical location, most commonly the abdominal wall, shoulder girdle and retroperitoneum. The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures. It is associated with a high local recurrence rate after resection. Many issues regarding the optimal treatment of desmoid tumors remain controversial. Aggressive surgical resection with a wide margin (2-3 cm) remains the gold standard treatment with regard to preserving quality of life. Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions. Desmoid tumors tend to be locally infiltrative, therefore, the fields must be generous to prevent marginal recurrence. The radiation dose appropriate for treating desmoid tumors remains controversial. We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection, treated with radiotherapy. The patient achieved complete tumor regression at 4 mo after radiotherapy, and he is clinically free of disease at 12 mo after the end of treatment, with an acceptable quality of life. The patient developed short bowel syndrome as a complication of second surgical resection. Consequently, radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.
腹壁硬纤维瘤,又称侵袭性纤维瘤病,是一种罕见的良性肿瘤,起源于筋膜或肌膜组织。它可以发生在任何解剖部位,最常见于腹壁、肩部和腹膜后。典型的临床表现为无痛性肿块,缓慢进行性侵犯相邻结构。切除后局部复发率较高。许多关于腹壁硬纤维瘤最佳治疗方法的问题仍存在争议。广泛切除(2-3cm)仍然是保留生活质量的金标准治疗方法。单独放疗已被证明对不可切除或复发性病变的控制有效。腹壁硬纤维瘤往往具有局部浸润性,因此,照射野必须足够大,以防止边缘复发。治疗腹壁硬纤维瘤的适当放射剂量仍存在争议。我们报告了一例 25 岁白人男性,在多次手术切除后出现腹壁硬纤维瘤局部复发,采用放疗治疗。患者在放疗后 4 个月完全消退肿瘤,治疗结束后 12 个月临床无疾病,生活质量可接受。患者因第二次手术切除发生短肠综合征。因此,放疗可能加重了已存在的吸收不良,从而导致脂肪性肝炎。