Patel S R, Evans H L, Benjamin R S
Department of Melanoma/Sarcoma Medical Oncology, University of Texas M. D. Anderson Cancer Center, Houston 77030.
Cancer. 1993 Dec 1;72(11):3244-7. doi: 10.1002/1097-0142(19931201)72:11<3244::aid-cncr2820721118>3.0.co;2-d.
Desmoid tumors are locally aggressive tumors, with no metastatic potential, that generally are amenable to local treatments, such as surgery and radiation therapy. Systemic therapy is considered for selected cases that are not amenable to local treatment.
The authors reviewed their experience with chemotherapy in desmoid tumors. A patient population was identified through a search of the data base maintained by the Department of Patient Studies.
Between January 1971 and December 1991, 180 patients with a histologically confirmed diagnosis of desmoid tumor were seen at the authors' institution. Twelve patients (8 male and 4 female patients; age range, 16-66 years; median age, 29 years) received chemotherapy. Eleven patients received doxorubicin (60-90 mg/m2) plus dacarbazine (750-1000 mg/m2)-based regimens for a median of 5 cycles (2-10 cycles). Six of the nine patients who could be evaluated for response had an objective response (two complete responses and four partial responses), one patient had a minor response, and two patients had stable disease. Two other patients treated in the early 1970s could not be evaluated objectively because of lack of modern imaging; however, they were reported to have "responses" that enabled resection of axillary and pelvic disease. All four patients with Gardner syndrome experienced disease response. One of these four patients had a complete response twice with doxorubicin-based chemotherapy and eventually died with an ejection fraction of 0.22. Five patients are alive with no evidence of disease (NED), four are alive with disease, and two are lost to follow-up after having an NED status at their last visit.
The authors conclude that desmoid tumors in adults are responsive to chemotherapy, and such treatment should be considered before embarking on radical treatment to avoid obvious functional consequences and delayed complications.
硬纤维瘤是局部侵袭性肿瘤，无转移潜能，通常适合局部治疗，如手术和放射治疗。对于不适合局部治疗的特定病例，可考虑全身治疗。
作者回顾了他们在硬纤维瘤化疗方面的经验。通过搜索患者研究部维护的数据库确定了患者群体。
1971年1月至1991年12月期间，作者所在机构共诊治了180例经组织学确诊的硬纤维瘤患者。12例患者（8例男性和4例女性；年龄范围16 - 66岁；中位年龄29岁）接受了化疗。11例患者接受了以阿霉素（60 - 90 mg/m²）加达卡巴嗪（750 - 1000 mg/m²）为基础的方案，中位疗程为5个周期（2 - 10个周期）。9例可评估反应的患者中，6例有客观反应（2例完全缓解和4例部分缓解），1例有轻微反应，2例病情稳定。20世纪70年代早期治疗的另外2例患者因缺乏现代影像学检查无法进行客观评估；然而，据报道他们有“反应”，使得腋窝和盆腔疾病得以切除。所有4例加德纳综合征患者均有疾病反应。这4例患者中的1例接受基于阿霉素的化疗两次获得完全缓解，最终因射血分数为0.22而死亡。5例患者无疾病证据存活，4例患者带瘤存活，2例在最后一次随访时处于无疾病证据状态后失访。
作者得出结论，成人硬纤维瘤对化疗有反应，在进行根治性治疗之前应考虑这种治疗方法，以避免明显的功能后果和延迟并发症。
