Acker J C, Bossen E H, Halperin E C
Department of Radiation Oncology, Duke University Medical Center, Durham, NC 27710.
Int J Radiat Oncol Biol Phys. 1993 Aug 1;26(5):851-8. doi: 10.1016/0360-3016(93)90501-l.
To determine the efficacy of different treatment modalities for desmoid tumors.
We reviewed the treatment of 40 patients with histologically confirmed desmoid tumors seen at Duke University Medical Center between 1974 and 1990.
Radiotherapy was administered to 16 patients (Group I)--14 with recurrent disease s/p surgery and in two as initial treatment. The average size of the irradiated lesions was 9.3 +/- 3.9 X 8.4 +/- 3.5 cm. With a median follow-up of 57.5 months and a median administered dose of 5400 cGy (mean 5286 cGy, range 4960-5620 cGy), local control has been obtained in 15/16 patients (94%). Complete regression (5/16), partial regression (5/16), or stable disease (5/16) was produced in 15 patients while one patient failed and was salvaged via gross total resection. Continued regression has been seen up to 60 months after treatment. Fourteen patients underwent primary gross total resection and two underwent subtotal resection (Group II). None received post-operative radiotherapy. Three of 14 patients (21%) recurred after gross total resection. All three were salvaged with subsequent gross total resection. After subtotal resection, 2/2 patients recurred. With a mean follow-up of 52 months, 14 patients are without evidence of disease, one is dead with disease (unrelated cause of death), and one was lost to follow-up after recurrence. Eight patients have been treated with combinations of chemotherapy, NSAIDS, anti-estrogens, and immunotherapy with mixed results (Group III). A subset of seven patients with retroperitoneal disease taken from all three groups had large tumor burden (mean size 17 X 15 cm), an infiltrative nature, as well as a difficult location. The disease was surgically resectable in three patients. One is without evidence of disease 9 years after gross total resection alone. Disease has been stabilized with radiotherapy in the other two patients after multiple unsuccessful surgical resections. Of four patients with unresectable disease, two are dead of disease, one died of unrelated causes with disease, and regression of disease was obtained in the other with Gamma-interferon after unsuccessful treatment with tamoxifen and vincristine, doxorubicin, and cyclophosphamide chemotherapy.
Gross total resection is the indicated initial therapy, if it can be performed without significant disfigurement. Radiotherapy is also excellent for obtaining local control, even in patients with a large burden of recurrent disease. Doses in the range of 50 to 55 Gy give a chance of local control equal to that obtained with higher doses previously reported.
确定不同治疗方式对硬纤维瘤的疗效。
我们回顾了1974年至1990年间在杜克大学医学中心确诊的40例硬纤维瘤患者的治疗情况。
16例患者接受了放疗（第一组）——14例为手术后复发患者，2例为初始治疗患者。照射病灶的平均大小为9.3±3.9×8.4±3.5厘米。中位随访时间为57.5个月，中位给予剂量为5400 cGy（平均5286 cGy，范围4960 - 5620 cGy），16例患者中有15例（94%）获得了局部控制。15例患者出现完全缓解（5/16）、部分缓解（5/16）或病情稳定（5/16），1例患者治疗失败，后通过根治性切除术挽救。治疗后长达60个月仍可见持续缓解。14例患者接受了根治性切除术，2例接受了次全切除术（第二组）。均未接受术后放疗。14例根治性切除术后患者中有3例（21%）复发。这3例均通过后续根治性切除术挽救。次全切除术后，2/2例患者复发。平均随访52个月，14例患者无疾病证据，1例死于疾病（与疾病无关的死因），1例复发后失访。8例患者接受了化疗、非甾体抗炎药、抗雌激素和免疫疗法联合治疗，结果不一（第三组）。从所有三组中选取的7例腹膜后疾病患者肿瘤负荷大（平均大小17×15厘米），具有浸润性，且位置不佳。3例患者的疾病可通过手术切除。1例仅接受根治性切除术后9年无疾病证据。另外2例患者在多次手术切除失败后，通过放疗使病情稳定。4例不可切除疾病患者中，2例死于疾病，1例死于与疾病无关的原因，另1例在他莫昔芬、长春新碱、阿霉素和环磷酰胺化疗失败后，使用γ干扰素使疾病缓解。
如果能在不造成明显毁容的情况下进行根治性切除术，则应作为首选初始治疗。放疗对于获得局部控制也非常有效，即使是复发疾病负担较大的患者。50至55 Gy范围内的剂量获得局部控制的机会与先前报道的更高剂量相同。
