Leibel S A, Wara W M, Hill D R, Bovill E G, de Lorimier A A, Beckstead J H, Phillips T L
Int J Radiat Oncol Biol Phys. 1983 Aug;9(8):1167-71. doi: 10.1016/0360-3016(83)90175-x.
Desmoid tumors are benign neoplasms, arising from musculoaponeurotic tissues, which tend to be locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Nineteen patients with desmoid tumors underwent radiation therapy at the University of California, San Francisco, between 1970 and 1980. Fifteen patients were referred with local recurrence following one or more surgical resections. Three patients were referred for initial radiation therapy with unresectable tumors, and one patient received planned postoperative irradiation following subtotal tumor resection. At the time of treatment, 8 patients had nonresectable disease measuring greater than 10 cm. Five patients had residual tumor masses measuring 4 to 6 cm, and six had only microscopic disease following resection. The majority of patients were treated to a tumor dose of 50-55 Gy at 1.6 to 1.8 Gy per fraction. With a median follow-up of 8 years, 13 patients remained free of recurrent disease following radiation therapy. The 5 year relapse free survival was 72% with 10 patients continuing to be free of disease 5 to 11 years following therapy. Local control was not related to the amount of disease present at the time of treatment. Of the 6 patients who developed recurrent disease, only 1 patient had a true in-field recurrence. Four patients recurred at the margin of the radiation field 1 to 5 years following therapy. Of these four patients, 3 were successfully salvaged while 1 died as a result of tumor extension into a major vessel. One patient with an extensive mesenteric mass did not respond to therapy and died 1 month post irradiation. The patient with the in-field recurrence and 1 patient with a marginal recurrence were successfully treated with combination chemotherapy. Moderate dose radiation therapy to desmoid tumors can result in lasting local control when surgical resection is not possible. Post operative radiation can improve the rate of local control for patients with a high risk of recurrence. As desmoid tumors tend to be locally infiltrative, fields must be very generous to prevent marginal recurrence. Systemic chemotherapy offers an alternative to ablative surgery in the event of local failure following radiation therapy.
硬纤维瘤是一种良性肿瘤，起源于肌筋膜组织，具有局部浸润性，导致手术切除后局部复发率很高。1970年至1980年间，19例硬纤维瘤患者在加利福尼亚大学旧金山分校接受了放射治疗。15例患者在一次或多次手术切除后出现局部复发而前来就诊。3例患者因肿瘤无法切除而前来接受初始放射治疗，1例患者在肿瘤次全切除后接受了计划中的术后放疗。治疗时，8例患者患有不可切除的疾病，肿瘤直径大于10厘米。5例患者有残留肿瘤肿块，直径为4至6厘米，6例患者在切除后仅有微小病灶。大多数患者接受的肿瘤剂量为50 - 55 Gy，每次分割剂量为1.6至1.8 Gy。中位随访8年，13例患者在放射治疗后未出现疾病复发。5年无复发生存率为72%，10例患者在治疗后5至11年仍无疾病复发。局部控制与治疗时存在的疾病数量无关。在6例出现疾病复发的患者中，只有1例为真正的野内复发。4例患者在治疗后1至5年在放射野边缘复发。在这4例患者中，3例成功挽救，1例因肿瘤扩展至大血管而死亡。1例患有广泛肠系膜肿块的患者对治疗无反应，放疗后1个月死亡。野内复发的患者和1例边缘复发的患者通过联合化疗成功治疗。当无法进行手术切除时，对硬纤维瘤进行中等剂量放射治疗可实现持久的局部控制。术后放疗可提高复发风险高的患者的局部控制率。由于硬纤维瘤往往具有局部浸润性，照射野必须非常大以防止边缘复发。在放射治疗后出现局部失败的情况下，全身化疗为根治性手术提供了一种替代方案。
