Dermatofibrosarcoma protuberans: Recurrence is related to the adequacy of surgical margins

Introduction

The aim of the study was to investigate the results of surgical treatment in primary and recurrent dermatofibrosarcoma protuberans (DFSP), with respect to local tumor control.

Patients and methods

Thirty-eight patients were treated between 1971 and 2005 at the University Medical Center Groningen (UMCG). Thirty patients presented with primary disease (79%) and 8 patients with locally recurrent disease (21%). The treatment consisted of surgical resection and in case of marginal or positive resection margins (R1 resection) adjuvant radiotherapy.

Results

Adequate surgical margins as a single modality was associated with 100% local control in all primary DFSPs. Two patients whose resection specimens had microscopically positive resection margins had withdrawn from adjuvant radiotherapy and developed local recurrence (LF rate 7%). Two of the 8 patients referred with a local recurrence developed a second recurrence (LF rate 25%); one of these patients developed distant disease and ultimately died of systemic disease. None of the five patients with DFSP-FS developed LF after treatment at the UMCG.After a median follow-up of 89 (12–271) months, the 10-year disease-free survival was 85% and the 10-year disease specific survival was 100%.

Conclusion

After wide surgical resection of a DFSP or DFSP-FS, or an R1 resection combined with adjuvant radiotherapy the risk of local recurrence is extremely low.     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

Objective： To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans. Methods： From August 1990 to November 1999, 74 patients with dermatofibrosarcoma protuberans （DFSP） confirmed by pathology were treated in Cancer Hospital of Sun Yat-sen University. 72 cases were given wide excision and 2 cases were given local excision. All of 74 cases, 52 cases had surgical resection alone, and 22 cases had surgical resection combined with radiotherapy. Total dose of radiotherapy was 50-70 Gy. Results： The rate of recurrence was 36.1% for all patients. The 5-year recurrence-free survival （RFS） rate was 66%. The 5-year recurrence-free survival rates for resection alone and combined with radiotherapy were 58% and 90%, respectively （P=-0.0187）. The 5-year recurrence-free survival rates for positive microscopic margins and negative microscopic margins were 57% and 75%, respectively （P=0.0468）. Conclusion： Post-operation radiotherapy is an effective treatment to decrease the recurrence rate for the patients with positive microscope, or the patients without suitable surgical treatment.     

Aggressive fibromatosis

The purpose of this article is to review the pertinent literature and to define the optimal treatment of patients with aggressive fibromatosis. Data indicate the likelihood of local recurrence after surgery is high, particularly if margins are positive. Moderate-dose radiotherapy alone for gross disease or after a microscopically incomplete resection yields local control rates of approximately 75% to 80%. Treatment with pharmacologic agents results in objective response rates of approximately 40% to 50%; duration of response is variable. Thus, the optimal management for aggressive fibromatosis depends on tumor location and extent. Surgery is indicated if the lesion appears to be completely resectable with acceptable function and cosmesis. Radiotherapy is indicated for patients with unresectable tumors; those with positive margins after resection should be considered for adjuvant radiotherapy depending on the location and extent of the tumor. Pharmacologic treatment should be considered for patients with progressive disease after unsuccessful local-regional therapy.     

Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution

BACKGROUND: Despite optimal surgical therapy for patients with dermatofibrosarcoma protuberans (DFSP), some patients still continue to develop local recurrence. The authors' objective was to identify and analyze clinicopathologic factors for disease free survival in a large group of patients who were followed prospectively at a single institution. METHODS: Prospectively collected data and pathology slides were available for review from 159 patients with primary or recurrent DFSP who underwent treatment between July 1950 and July 1998. The study group was comprised of patients with either the "classic" form of DFSP or the fibrosarcomatous "high grade" variant of DFSP (FS-DFSP). Patient, tumor, pathologic, and treatment factors were analyzed using the log rank test for univariate influence and Cox regression analysis for multivariate influence. Local recurrence free survival was determined by the Kaplan-Meier actuarial method. RESULTS: Of the 159 patients who comprised the current study group, 134 (84%) had the classic form of DFSP. The FS-DFSP variant was found in the remaining 25 patients (16%). The overall 5-year local recurrence free survival rate was 75%, with a median follow-up of 4. 75 years. The 5-year recurrence free survival rate for each group was 81% and 28%, respectively. On univariate analysis, age > 50 years, very close (< 1 mm) to positive microscopic margins, FS-DFSP variant, high mitotic rate, and increased cellularity were unfavorable prognostic factors. Multivariate analysis determined very close (< 1 mm) to positive microscopic margins and FS-DFSP variant to be independent adverse prognostic factors. For the 34 patients who developed a recurrence after surgical resection (21%), the median time to local recurrence was 32 months. Of the patients in this group, two died from metastatic disease. CONCLUSIONS: The prognosis after surgical resection with negative and sometimes positive microscopic margins for patients with DFSP is very good. However, increased age, high mitotic index, and increased cellularity are predictors of poor clinical outcome. The FS-DFSP variant represents a much more aggressive tumor with metastatic potential. Patients who are treated with curative intent for FS-DFSP should undergo aggressive attempts at complete surgical resection. Patients with recurrent classic DFSP without evidence of adverse prognostic features may benefit from conservative management, especially in the setting of potentially unresectable disease.     

Dermatofibrosarcome protuberans récidivant du sein: à propos d’un cas

The dermatofibrosarcoma protuberans (DFSP) is a sarcoma of low rank developing slowly from the fibroblasts of the dermis. The main characteristic of this tumour is its big potential for local recurrence. We report the case of a patient aged 44 presenting a DFSP of the breast that relapsed on two occasions. The remaining extension was negative. She benefited from a large timorous excision. The mammary localization of a DFSP is rare. The diagnosis is difficult. The therapeutic objective of DFSP is to reach a possible most complete surgical resection; the conventional surgical excision must let healthy margins (of 3 to 5 cm) with the inconvenience of the substance loss. The technique of Moh, verifying the limits of excision in preoperative, can palliate this inconvenience. The risk of recurrence is low when the resection margins are healthy. Radiotherapy as adjuvant treatment is very useful when the margins are not sufficient. The surveillance must be prolonged.     

Desmoid tumors: a novel approach for local control

BACKGROUND AND OBJECTIVES: After resection, desmoid tumors are associated with a recurrence rate that is typically 25-50%. Although this is an unusual problem, we instituted a prospective cohort study with neoadjuvant chemotherapy and radiation, followed by surgical resection, in an effort to improve local control. METHODS: Between 1985 and 1999, 13 patients with potentially resectable disease were managed with a treatment protocol of preoperative doxorubicin (30 mg continuous infusion daily for 3 days) and radiotherapy (10 x 300 cGy). Resection was performed 4-6 weeks later. All lesions were resected with an intended margin of 1 cm, but clear adventitial margins were accepted in order to preserve critical structures. RESULTS: The median follow-up was 71 months (range, 22-109). Six patients (46%) presented after failure of a previous surgery. Clear microscopic margins were obtained in 11 patients, and 2 patients had positive margins. There were two local recurrences (15% local recurrence). Both recurrences followed resection of large thigh lesions, which appeared at 30 and 49 months of follow-up. In one patient with a chest wall tumor, two new primary desmoid tumors developed outside the treatment area, in the ipsilateral arm and forearm. Eleven patients have been disease free for a median of 71 months (range, 22-109). CONCLUSIONS: For potentially resectable lesions, this protocol provides excellent local control, even in those with recurrent disease. Neoadjuvant treatment with doxorubicin and radiotherapy appears to be a better option than surgery alone, or surgery and adjuvant radiotherapy. These results need to be confirmed in larger, prospective randomized trials.     

Outcomes of surgery for dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP. HYPOTHESIS: This study was done to review our experience of the time interval to recurrence of DFSP. DESIGN: A retrospective review was undertaken to identify patients with DFSP in our university teaching hospital. METHODS: All patients received their primary surgical treatment in our department between February 1968 and June 2001. Treatment consisted of wide local excision with margins of at least 3 cm. The chi-square test and Fisher's exact test were performed to determine the relationship between recurrence and clinicopathological variables. We evaluated the prognostic variables using the Kaplan-Meier method with log-rank comparison. RESULTS: The median follow-up period was 59 months. The 5 and 10-year disease-free survival (DFS) were 86 and 76%, respectively. The overall recurrence rate was 16.7%. The mean time to recurrence was 38+/-12 months (range 1-100 months). In 30% of those patients with recurrences, the local regional recurrence was after 5 years. CONCLUSION: Wide local excision with good margins decreases local regional recurrences in patients with DFSP. Close surveillance is necessary even beyond 5 years because late recurrences occur.     

Free osseous and soft tissue surgical margins as prognostic factors in mandibular osteosarcoma

Osteosarcoma is an infrequent, locally aggressive neoplasm in the head and neck region. To date, surgery is the mainstay of treatment. However, patients with mandibular osteosarcomas usually have a locally advanced disease at diagnosis and therefore represent a therapeutic challenge because surgical margins are difficult to obtain due to aesthetic and functional concerns. To evaluate possible prognostic factors implicated in recurrence, persistence or relapse in osteosarcoma of the mandible, with special reference to the soft tissue and bone surgical margins. A series of 20 patients with mandibular osteosarcomas treated at the Instituto Nacional de Cancerología (México) from 1985 to 1999 are reviewed. There were 14 female and 6 male patients. Twelve cases were treated with surgery alone, 3 patients with surgery and adjuvant radiotherapy, 1 had neoadjuvant chemotherapy followed by surgery, 1 had neoadjuvant chemotherapy, surgery and postoperative radiotherapy, 1 with surgery and adjuvant chemotherapy, 1 with surgery followed by adjuvant chemotherapy and radiotherapy and one patient rejected treatment. Between 1985 and 1992 these neoplasms were treated by means of total mandibulectomy, independently of tumor size, but between 1993 and 1999 the policy was to practice smaller resections but long enough to obtain macroscopic surgical free margins. In the first period the relationship between mandibular size resection and tumor size was 1.9, meanwhile in the second period the relation was 1.5. There was not significant difference between both periods in terms of tumor size (6.0 cm vs. 6.02 cm at the time of surgery) nor in local control and survival. Soft tissue involvement as reported by histological study was strongly associated with recurrence (p = 0.0024). Overall 5-year survival was 20%. A policy of total mandibulectomy is not associated with a better local control or survival. Extent of resection must be tailored with tumor size. Extent of margins in soft tissue is the limiting factor for local control.     

Significance of dose in postoperative radiotherapy for soft tissue sarcoma

PURPOSE: For soft tissue sarcoma, adjuvant postoperative radiotherapy improves the local control rate over surgery alone. However, the issue of a dose-control relationship is controversial and was addressed in this study. METHODS AND MATERIALS: We retrospectively reviewed the records of 775 consecutive patients who received postoperative external beam radiotherapy and used univariate and multivariate analysis to determine whether a dose-control relationship exists for the whole group and for specific high-risk subgroups. RESULTS: With a median follow-up of 12.3 years, overall local control rates were 82%, 80%, and 79% at 5, 10, and 15 years. Factors adverse for local control were positive resection margins; tumor location in the head and neck and deep trunk; presentation with locally recurrent disease; patient age >64 years; histopathologic subtype of malignant fibrous histiocytoma, neurogenic sarcoma or epithelioid sarcoma; and tumor size >10 cm. In multivariate analysis, radiation dose > or = 64 Gy vs. <64 Gy independently correlated with improved local control. Significant interactions were noted between increased effectiveness of a higher dose and presentation with locally recurrent disease, with head and neck and deep trunk tumor sites, and with positive or uncertain resection margins such that local control was specifically improved under these circumstances with doses on the order of 64-68 Gy compared with doses on the order of 60 Gy. Higher dose, however, did not completely abrogate the adverse effect of positive margins. CONCLUSION: After gross total resection, soft tissue sarcoma with features predictive of a higher than average local recurrence rate benefited from doses of 64-68 Gy compared with 60 Gy.     

Defining the best available treatment for neurocytomas in children

BACKGROUND: In children, neurocytomas are extremely rare tumors in the central nervous system. Since this entity was introduced in 1982, approximately 60 cases have been reported among patients age /= 54 Gy when compared for local control (P = 1.0) and survival rates (P = 1.0). Radiotherapy-related psychomotor retardation or secondary brain tumors were not reported. CONCLUSIONS: The prognosis of children with neurocytomas is extremely good. CTR was associated with better local control and survival rates than ITR. After ITR, radiotherapy improves local control, but not survival. If postoperative radiotherapy is considered, a dose of 50 Gy was appropriate for long-term local control in children, whereas higher doses were required in adults.     

The role of radiotherapy in the treatment of desmoid tumours

From 1974 to 1983 in the Netherlands Cancer Institute, 21 patients with desmoid tumours were treated with radiation therapy. Nineteen patients were irradiated postoperatively (11 patients had micro- or macroscopic residual disease, 8 patients treated for recurrent disease had narrow surgical margins), 2 patients with inoperable tumours were treated with radiation alone. The entire involved muscle received a dose of 40 Gy, while a boost of 20 Gy was delivered to the tumour bed. Local control was achieved in 19 out of 21 patients, with an actuarial 5 year disease-free survival of 90%. No relation could be found between the amount of tumour present and local control. With careful set-up of treatment fields and long-term physical therapy, complications like fibrosis, ankylosis and oedema could be minimised. These excellent results with radiotherapy for minimal residual tumour, or even for macroscopic tumour, makes mutilating surgery unnecessary.     

Postoperative radiotherapy in carcinoma of the cervix with microscopically positive resection margin

Background Patients with cervical cancer who have positive surgical resection margins after radical hysterectomy are at increased risk for local recurrence. The results of postoperative pelvic radiotherapy for cervix cancer with microscopically positive surgical resection margins were analyzed to evaluate the role of radiotherapy. Methods Between 1979 and 1992, 60 patients with cervix carcinoma were treated with postoperative radiotherapy after radical hysterectomy and pelvic lymphadenectomy because of microscopic positive vaginal (48 patients), or parametrial (12 patients) resection margins. Patients were treated with external beam radiation therapy (EBRT) alone (12 patients), or EBRT plus vaginal ovoid irradiation (VOI) (48 patients). The median follow-up period was 55 months. Results The 5-year actuarial disease-free and overall survival rates for all patients were 75% and 84%, respectively. The overall recurrence rate was 23% (14/60). Among the 48 patients with positive vaginal resection margins, 4 had pelvic recurrence (8%), and 7 had distant metastasis (15%); the recurrence rate was 21% (9/43) in those treated with EBRT and VOI, and 40% (2/5) in the EBRT-only treated group. In the 12 patients with positive parametrial margins, 3 patients (25%) had distant metastases. The most significant prognostic factor was lymph node metastasis. Complications resulting from radiotherapy occurred at a rate of 32% (19/60), and grade III complications occurred in 3 patients (5%). Conclusion Postoperative radiotherapy can produce excellent control rates in patients with microscopically positive resection margins. In patients with positive vaginal margins, whole pelvic EBRT and VOI is recommended.     

Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy

BACKGROUND: Patients with localized soft tissue sarcoma (STS) who present to specialist centers after undergoing apparent macroscopic total resection often have a significant incidence of residual tumor and may benefit from reresection of the tumor bed. The potential benefits of such reresection have not been documented adequately. METHODS: The clinicopathologic features and disease outcome for 666 consecutive patients with localized STS who presented after undergoing apparent macroscopic total tumor resection were analyzed to elucidate the relative merits of reresection. Actuarial univariate and multivariate methods were used to compare disease outcome of patients who presented with positive or uncertain microscopic resection margins according to whether they underwent reresection. All patients received adjuvant radiation therapy. RESULTS: Two hundred and ninety-five patients underwent reresection of their tumor bed, and residual tumor was found in 136 patients (46%), including macroscopic tumor in 73 patients (28%). Final resection margins among patients who underwent reresection were negative in 257 patients (87%), positive in 35 patients (12%), and uncertain in 3 patients (1%). Patients who did not undergo reresection had final margins that were negative in 117 patients (32%), positive in 47 patients (13%), and uncertain in 207 patients (56%). Local control rates at 5 years, 10 years, and 15 years for patients who underwent reresection were 85%, 85%, and 82%, respectively; for patients who did not undergo reresection, the respective local control rates were 78%, 73%, and 73% (P = 0.03). Reresection remained a significant determinant of local control when other prognostic factors were incorporated into a multivariate proportional hazards regression analysis. A similar beneficial effect of reresection was found for metastasis free survival and disease specific survival. CONCLUSIONS: Patients with localized STS who were referred to a specialist center after undergoing apparent macroscopic total resection of their tumor had a high incidence of residual tumor in their tumor bed and benefited from undergoing reresection, even if radiation was administered routinely.     

Radiotherapy does not compensate for positive resection margins in rectal cancer patients: report of a multicenter randomized trial

PURPOSE: Circumferential resection margin (CRM) involvement is a prognostic factor for local recurrence in rectal cancer. In a randomized trial comparing preoperative radiotherapy (5 x 5 Gy), followed by total mesorectal excision (TME) with TME alone, we demonstrated the beneficial effect of short-term preoperative radiotherapy on local recurrences. Here we evaluate the effect of radiotherapy on local recurrence rates in patients with different CRM involvements. METHODS AND MATERIALS: Circumferential margins were defined as positive (< or =1 mm), narrow (1.1-2 mm), or wide (>2 mm). Postoperative radiotherapy was mandatory for surgery-only patients with a positive CRM, but was not always administered and enabled us to compare local recurrence rates for patients with or without postoperative radiotherapy. Furthermore, the effect of preoperative radiotherapy was assessed in the different margin groups. RESULTS: Of 120 patients in the surgery-only group with a positive CRM, 47% received postoperative radiotherapy. There was no difference in the local recurrence rate between the irradiated and nonirradiated patients (17.3% vs. 15.7%, p = 0.98). Preoperative radiotherapy was effective in patients with a narrow CRM (0% vs. 14.9%, p = 0.02) or wide CRM (0.9 vs. 5.8%, p < 0.0001), but not in patients with positive margins (9.3% vs. 16.4%, p = 0.08). CONCLUSION: Preoperative hypofractionated radiotherapy has a beneficial effect in patients with wide or narrow resection margins, but cannot compensate for microscopically irradical resections resulting in positive margins.     

Merkel Cell Carcinoma – Current Controversies and Future Directions

Merkel cell carcinoma is a rare, aggressive neuroendocrine skin malignancy. Evidence for management comes from case series and single-arm trials. Optimal outcomes require assessment of the patient in a multidisciplinary team setting. Rapid diagnosis and staging are essential for locoregional control and may reduce metastasis. Sentinel lymph node biopsy (SLNB) adds prognostic information. FDG-positron emission tomography has high sensitivity and specificity and affects management in a quarter of cases. Surgical excision and radiotherapy provide good locoregional control even with positive margins. Wide surgical margins are needed if adjuvant radiotherapy is not used. It is uncertain whether adjuvant radiotherapy or elective surgery for uninvolved nodes or for patients selected by positive SLNB improves survival. Total doses of 50 Gy provide high levels of control for microscopic disease but at least 60 Gy should be given for macroscopic disease. Chemotherapy can be given safely with radiotherapy, but the benefit of adjuvant chemotherapy remains uncertain. Trials of adjuvant immune therapy are underway. Unresectable primaries might be controlled with radiotherapy alone or combination systemic therapy, radiotherapy and surgery. Metastatic disease often responds to chemotherapy, but the response duration can be short. Immunity is central to disease control. Immune checkpoint inhibitor treatment resulted in high response rates in chemotherapy-naive patients and lower rates in chemotherapy-refractory patients. Durable responses are observed.     

乳腺外派杰氏病三例报告附文献综述

探讨手术、放疗、化疗在乳腺外派杰氏病（ＥＭＰＤ）治疗中的作用，材料与方法报告我院３例ＥＭＰＤ的治疗情况，并复习１９９０年以来英语医学文献及国内文献中有关ＥＭＰＤ治疗的病例１３５例，合计１３８例。分析手术、放疗、化疗在ＥＭＰＤ治疗中的可行性及局限性，结果手术组９５例，切缘阳性者４５例，其中单纯手术组局部复发率４８．６％（１８／３７），行术前或术后放疗者无局部复发（０／８）：切缘阴性者５０例，局部复发率２６％（１３／５０）。单独放疗者２５例，２例局部未控。３例疗后１２－２４月局部复发，局部控制率为８０％。化疗组１３例，单独化疗治愈２例，化疗＋放疗治愈１例，手术＋化疗治愈１例。结论尽管手术为ＥＭＰＤ的首选治疗方式，但局部复发率较高，配合放疗，可降低其局部复发率，即使是单独放疗也可取得满意的局部控制效果。化疗可取得一定的疗效，但不主张作为一线治疗方案。     

Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.

PURPOSE: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP. PATIENTS AND METHODS: Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied. RESULTS: One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins. CONCLUSION: This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.     

Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. METHODS: The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. RESULTS: Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). CONCLUSIONS: Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution.     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

目的　探讨隆突型皮肤纤维肉瘤 (DFSP)的治疗及其影响预后的因素。方法　对 74例经病理证实的隆突型皮肤纤维肉瘤 (DFSP)患者进行回顾性分析。其中 ,72例行广泛切除术 ,2例行局部切除。 5 2例行单纯手术治疗 ,2 2例加术后放疗。放射治疗采用 6Mev电子线 +60 Coγ线 ,或 6Mev电子线 +后装 ,或单纯电子线照射。 17例采用连续照射 ,5例采用分段治疗 ,照射剂量 5 0～ 70Gy。全部患者均未行化疗和其他治疗。以Cox比例风险模型进行多因素分析 ,以Kaplan Meier法计算无复发生存时间 ,以Logrank检验进行结果之间的比较。结果　剔除 2例失访病例 ,全组复发率为 2 8.4 % ,5年无复发生存率为 6 6 .6 % ,10年无复发生存率为 5 2 .5 %。多因素分析结果显示 ,放疗与否和术后病理切缘为独立预后因素。术后放疗组与单纯手术组的 5年无复发生存率分别为 90 .0 %和 5 8.4 % ,10年无复发生存率分别为 83.3%和 4 1.2 % (P <0 .0 5 ) ;病理切缘阴性和阳性的 5年无复发生存率分别为75 .0 %和 5 7.5 % ,10年无复发生存率分别为 5 6 .6 %和 4 1.4 % (P <0 .0 5 )。结论　术后放疗与否和术后病理切缘为DFSP的独立预后因素。     

Place and technical aspects of external beam radiation therapy in the treatment of adult soft tissue sarcomas]

In soft tissue sarcoma, surgical resection remains the cornerstone of therapy for localized disease. Quality of margins is very important to evaluate. In case of marginal or incomplete resection, a new enlarged surgical resection should always be discussed before administration of any adjuvant treatments. Many retrospective studies and 2 randomized studies (one of adjuvant brachytherapy and one of external beam radiotherapy) have shown that adjuvant radiotherapy after complete surgery reduces significantly the risk of local recurrence in extremity soft tissue sarcomas. Combination of surgery and pre- or postoperative radiotherapy has therefore become the standard treatment with a local recurrence rate 相似文献