肢体软组织肉瘤34例外科治疗体会

目的探讨肢体软组织肉瘤的诊断与手术疗效。方法回顾性分析外科手术治疗并经病理证实的34例肢体软组织肉瘤患者的诊治资料。结果术后病理:韧带样瘤型纤维瘤病4例,纤维肉瘤5例,浅表型纤维瘤病1例,隆突性皮肤纤维肉瘤4例,恶性纤维组织细胞瘤4例,脂肪肉瘤5例,平滑肌肉瘤5例,恶性周围神经鞘膜瘤2例,滑膜肉瘤2例,原始神经外胚层瘤2例。28例获术后随访1～4 a,4例(14.3%)复发,复发时间12～26个月,平均20个月。结论外科手术是肢体软组织肉瘤最重要的治疗手段,合理应用综合治疗和个体化治疗可提高切除率、降低复发率。     

Curative limb saving operation (curative wide resection)

The recent results of surgical treatment of soft tissue sarcomas have dramatically improved. The result of the group study of soft tissue sarcomas, which was supported by the Ministry of Health and Welfare (1962-1976) showed that the local recurrence rate was 50%, and the metastatic rate was 60%. In contrast with this result, the recent results of our curative wide resection showed the local recurrence rate of 10%, and the metastatic rate of 30%. These procedures are based on the clinico-pathological findings which we studied. Curative wide resection is a method of radical wide resection, similar to radical local resection (reported by Enneking) and radical local excision (reported by Stenar). Our procedures are the least aggressive of the three radical wide resections, but our results are the same as the other wide resection methods. To obtain good results with these procedures, accurate preoperative diagnosis and skillful surgery are necessary.     

原发性阴囊皮下平滑肌肉瘤（附2例报告）

目的：结合文献探讨原发性阴囊皮下平滑肌肉瘤的临床特点及诊断、治疗方法。方法：对2例原发性阴囊皮下平滑肌肉瘤的临床资料进行分析。结果：2例均行根治性切除术,病理诊断为平滑肌肉瘤。分别随访11及8个月,未见复发及转移。结论：原发性阴囊皮下平滑肌肉瘤少见。好发于中老年人。诊断主要依靠病理诊断。治疗需以根治性手术为主,需跟踪随访,早期预后效果较好。     

Management of pelvic sarcoma

Pelvic sarcomas are a rare and heterogenous group of tumors divided into two groups: soft tissue sarcomas and bone sarcomas. Soft tissue sarcomas of the pelvis include most commonly liposarcoma, leiomyosarcoma, gastrointestinal stromal tumors, malignant peripheral nerve sheath tumors, and solitary fibrous tumors. Bone sarcomas of the pelvis most commonly include osteosarcoma and chondrosarcoma. Multidisciplinary treatment at a center experienced in the treatment of sarcoma is essential. Management is dictated by histologic type and grade. Surgical resection with wide margins is the cornerstone of treatment for pelvic sarcomas, although this is often challenging due to anatomic constraints of the pelvis. Multimodal treatment is critical due to the high risk of local recurrence in the pelvis.     

Leiomyosarcoma of the skin: A case report

A middle-aged man presented with an ulcerated nodule of the right posterior thigh that was histologically evaluated as leiomyosarcoma of the skin. A wide excision of the tumor was followed by split-thickness skin grafting. Leiomyosarcomas of the skin and subcutaneous tissue are rare tumors, usually occurring in the proximal lower extremities. The treatment of choice is a wide local excision with a 3- to 5-cm margin including the subcutaneous tissue and fascia. The defect is covered by a split-thickness skin graft.     

侵犯关节周围骨组织的软组织肉瘤外科治疗策略

目的探索侵犯关节周围骨组织的肢体软组织肉瘤的外科手术方法,并分析术后并发症、肢体功能和患者的生存状况。方法回顾性分析2004年5月至2011年10月期间,我中心所收治的30例局部侵犯关节周围骨组织的软组织肉瘤患者的临床资料。其中男14例,女16例,平均年龄51岁（17～75岁）。恶性纤维组织细胞瘤12例,脂肪肉瘤8例,原始神经外胚层肿瘤（PNET）4例,滑膜肉瘤、腺泡状软组织肉瘤和恶性神经鞘瘤2例。其中10例发病位于股骨近端,9例股骨远端,8例肱骨近端,2例胫骨近端,1例累及整个股骨。所有患者均采取了肿瘤连同受累骨组织一起广泛切除、以肿瘤型假体重建骨缺损的手术方式。术后定期复查患肢功能、x线片、肺CT等,并且密切随访,内容包括术后并发症的发生情况、肢体功能恢复情况及肿瘤学与生存状况等。结果平均随访25个月（3—84个月）,1例暂时性腓总神经麻痹;3例术后伤口愈合不良行清创手术,其中1例因深部感染不愈而截肢;2例假体断裂行翻修手术;4例肿瘤复发,局部复发率13．3％,其中1例接受截肢手术。总的肺转移为15例,且11例死亡患者中均为肺转移,其中3例合并骨转移。至最后一次随访时14例无瘤生存,5例带瘤生存,平均MSTS评分股骨近端90％,股骨远端82％,胫骨近端73％,肱骨近端71％,全股骨为60％。2年和5年生存率分别为61．6％和30．8％。结论将受累骨组织和肿瘤一起广泛切除可获得无瘤边界,降低复发率,骨缺损采用肿瘤型假体重建可恢复良好的肢体功能。巨大肢体软组织肉瘤侵犯骨组织可能是患者预后不良的因素。     

Limb sparing surgery in soft tissue sarcoma of extremities: An Indian Perspective

This is a retrospective study of 57 patients with extremity soft tissue sarcomas treated at Gujarat Cancer Research Institute, Gujarat, India from January 2005 to December 2007. It aims to review the feasibility of limb sparing surgery for extremity soft tissue sarcoma in Indian population. Twenty-eight percent (28%) of tumors were located in the upper limbs and 72% in the lower extremities. The goal of treatment was limb salvage if feasible. The treatment consisted of amputation in 17 (30%) and local wide excision i.e. limb salvage surgery (LSS) in 40 (70%) patients. The most common histology was pleomorphic sarcoma (21%). Sixty-four percent (64%) of the patients were treated with surgery alone, 32% with surgery plus radiation and 4% with surgery plus systemic chemotherapy. Inadequate margins were present in 12% of the patients. All the patients were followed up for at least 2 years. Recurrence in the form of local and distant occurred in 39% of the patients. Local recurrence occurred in 18% of the patients who underwent LSS and in 35% of those who underwent amputation. Pulmonary metastasis was detected in 16% of patients. The 2 year mortality was 10.5% with the most common etiology being lung metastasis and the most common histology being leiomyosarcoma. We concluded that limb salvage surgery for soft tissue sarcoma is feasible in the Indian population (70%) although the rate is lower than that of world literature at 90%. Keywords: soft tissue sarcoma, wide excision, amputation, limb salvage surgery, Indian population.     

Clinical course of nonvisceral soft tissue leiomyosarcoma in 225 patients from the Scandinavian Sarcoma Group

BACKGROUND: Leiomyosarcoma of nonvisceral soft tissues is an uncommon malignant tumor; thus, only small numbers of cases have been reported. This study was based on a large series of patients from the Scandinavian Sarcoma Group Register acquired during a 15-year period (from 1986 to 2001). Follow-up information was available for all patients. METHODS: The authors analyzed the clinical features of 225 patients with cutaneous, subcutaneous, or deep-seated leiomyosarcoma of the extremities, trunk wall, and superficial parts of the head and neck region to determine the natural course of the disease. Only patients who received their treatment at a specialist sarcoma center were included. Re-evaluation of histopathology was performed. RESULTS: The age of the patients (121 women and 104 men) ranged from 20 years to 98 years (median, 70 years), and the tumors ranged in size from 0.6 cm to 35 cm (median, 4.0 cm). Eighty-two percent of the tumors were classified as high grade. The median follow-up for survivors was 5.5 years. The local treatment was adequate in 154 of 206 patients (75%) who were without metastasis at presentation. At 10 years, 84% of the 206 patients with localized disease at presentation were free from local recurrence, 66% remained metastasis free, and 49% were alive. Multivariate analysis showed that higher malignancy grade (P = .006), larger tumor size (P = .003), and deeper tumor location (P = .002) were correlated significantly with decreased metastasis-free survival, inadequate local treatment was correlated with local recurrence (P = .007), and high malignancy grade was correlated with decreased overall survival (P = .007). CONCLUSIONS: The long-term prognosis for patients with subcutaneous and deep-seated soft tissue leiomyosarcoma remains poor despite the ability to achieve adequate local control through nonmutilating surgery with or without radiotherapy.     

Leiomyosarcoma of Nose a Very Rare Tumor Case Report: Unusual Case Leiomyosarcoma Inferior Turbinate

Leiomyosarcoma (LMS) accounts for approximately 7% of all soft tissue sarcomas and occurs most frequently in the gastrointestinal tract and uterus. In the head and neck, however, LMS makes up only 2.3%. LMS of the nasal cavity and Para nasal sinuses are very rare and only about 20 cases of LMS of the nose and Para nasal sinuses have been reported in the literature. Initially, LMS should be treated by extensive surgical excision, long-term follow-up is essential due to the high rate of local recurrence. Radiotherapy and chemotherapy are insufficient therapeutic approaches. Frequency of recurrence and prognosis depend on the tumor site.     

Feasibility of limb salvage and survival in soft tissue sarcomas

One hundred nine consecutive patients with soft tissue sarcomas were treated in the period 1977 through 1983. Of 85 patients with extremity sarcomas, only 3 patients (4%) were managed with amputation, whereas in the previous decade, 40% of such patients were treated with amputation in our institute. The current 5-year survival rate is 63%; in the previous decade it was 45%. In the current series, for extremity locations, patients with minimum surgical margins of 2 cm or greater and no further local therapy had a 5-year local recurrence rate of 17%, whereas those with minimum surgical margins of less than 2 cm and who were treated with adjuvant postoperative radiation had a local recurrence rate of 7%. In the previous period, the local recurrence rate was 30% after wide resection and 66.6% after local excision. With a combination of modalities, limb salvage can be practiced currently in the majority of patients with extremity soft tissue sarcomas without any adverse effect on recurrence rates and survival.     

Retroperitoneal soft tissue sarcoma

The objective of the current study was to define the optimal treatment for patients with retroperitoneal soft tissue sarcomas (RPS). The authors conducted a review of the pertinent literature and found that the majority of patients had locally advanced RPS at the time of diagnosis. Surgery was the mainstay of treatment, and macroscopic total excision was feasible in approximately 50-67% of patients. The 5-year probability of local control was approximately 50%, and the likelihood of developing distant metastasis was approximately 20-30%. The 5-year survival rate was approximately 50%, and deaths due to recurrent RPS continued to occur after 5 years. Postoperative radiotherapy (RT) decreased the likelihood of local recurrence, although it demonstrated no impact on survival. Adjuvant chemotherapy had no proven efficacy. The factor that was found to have the greatest impact on the prognosis for patients with RPS was the ability to achieve a macroscopic total resection. The authors concluded that RPS usually is advanced at the time of diagnosis. The optimal treatment was complete resection. Most patients experienced recurrent disease after surgery, and the most common site of failure was local. Adjuvant RT reduced the risk of local recurrence, but its impact on survival was questionable.     

Sarcomas of the breast: a clinico-pathological study of 67 patients with long term follow-up

A national Danish series of 68 breast sarcomas from 66 women and one man (age 17-86 years) was investigated. Tumour sections from 22 stromal sarcomas (SS), 24 phyllodes tumors of malignant type (MCSP), seven phyllodes tumors of borderline type (BLCSP), four malignant fibrous histocytomas (MFH), eight liposarcomas, two angiosarcomas and one leiomyosarcoma were reviewed retrospectively, and all patients were traced with a minimum follow-up of 15 years. Tumor contours appeared to be the best prognostic factor in predicting the risk of metastatic spread. Stromal overgrowth of MCSP was considered less utilizable due to difficulties in distinguishing between MCSP with marked stromal overgrowth and SS, which we consider as a variety of MCSP and which only showed slightly increased death rates compared to MCSP (45% versus 38%). Both angiosarcomas and the leiomyosarcoma proved lethal, and the other sarcoma subtypes had a death frequency of about 50%, with the exception of BLCSP, neither of which proved lethal. All patients, who died from metastases, were dead within 5 years irrespective of treatment. No positive lymph nodes were identified at the time of primary treatment, supporting the findings of previous investigators. We therefore advocate simple mastectomy or local excision with a wide margin as sufficient treatment of breast sarcomas. The indications for adjuvant therapy is as yet unclarified.     

Prognostic factors in soft tissue sarcomas: the Aarhus experience

In the present study, the outcome, patterns of local recurrence and survival, as well as prognostic factors, were evaluated in patients surgically treated for soft tissue sarcomas. Between January 1979 and July 1993, 316 consecutive patients were referred to the Sarcoma Centre in Aarhus with localised malignant soft tissue sarcoma of the extremities or trunk. If possible, the patients were treated with a limb-sparing resection, primarily by use of a wide excision. 50 patients received adjuvant radiotherapy. There were 161 men (51%) and 155 women (49%) median age 56 years (range 1-94 years). 94 patients (30%) had tumours in the trunk, including shoulder and buttock lesions, 163 (52%) in the lower extremity and 59 (19%) in the upper extremity. 52 patients (16%) had grade 1 tumour, 60 (19%) grade 2 and 204 (65%) grades 3A-3B. The 5-year local recurrence rate was 18% and the 5-year survival rate was 75%. Multivariate analysis indicated the following variables as independent unfavourable factors for local recurrence: extracompartmental location, histological high grade, local excision, no adjuvant radiotherapy and intralesional/marginal excision. Independent unfavourable factors for survival were advanced age, extracompartmental location, histological high grade, lower extremity location and large tumour size. If the variable local recurrence was included in the analysis, it was found to have a very strong influence on survival. Based on these variables, a prognostic model was developed.     

Treatment and local control of primary extremity soft tissue sarcomas.

BACKGROUND AND OBJECTIVES: Modern series of adult extremity soft tissue sarcomas utilize combinations of modalities in all patients. Remaining questions: 1) is it necessary to strive for wide margins in the multimodality era; 2) to use adjuvant therapy in every high-grade sarcoma? 3) Does previous partial or marginal resection seriously interfere with the definitive resection? METHODS: In a retrospective review of 194 extremity soft tissue sarcomas (1977-1994), limb preservation was possible in 181/194 (93%) of cases. Patients with narrow margins received adjuvant radiation. Some patients were referred after partial (n = 39) or "complete" (n = 63) excision. RESULTS: Local recurrence was observed in 181/141 (13%) of patients treated with wide or compartmental resection, and in 10 of 42 (24%) of those treated with conservative resection plus radiation (P = 0.14). The 5-year survival rate for grade III, >/=5-cm sarcomas was not significantly different (P = 0.82) with adjuvant (46%) or without (48%) adjuvant systemic chemotherapy. Five-year survival varied (P = 0.0001) according to grade. Patients referred with partial, or "complete" (63%, 38/63, had residual tumor at reoperation) excision had a local recurrence rate of 8% and 6%, and 5-year survival rates of 75% and 84%, respectively. CONCLUSIONS: 1) It is important to strive for wide margins even when adjuvant radiation is intended. 2) When a wide margin is possible, adjuvant radiation may not be necessary. 3) Adjuvant systemic chemotherapy may be considered for high-grade tumors, preferably within a prospective protocol. 4) A partial or "complete" excision of the tumor before referral to a tertiary center does not appear to compromise the limb preservation, local control, or survival rates of these patients.     

Low dose-rate interstitial brachytherapy in soft tissue sarcomas

Purpose. To assess the effectiveness of Ir-192 interstitial brachytherapy as an adjunct to wide local excision as a functionsaving strategy for soft tissue sarcomas.Subjects and methods. From September 1993 to April 1998, 20 consecutive patients diagnosed with soft tissue sarcomas were treated with a combination of wide local excision and interstitial brachytherapy. In 16 patients brachytherapy was done as an intraoperative procedure, while in four, the implant was performed post-operatively under local anesthesia. Eleven of the 20 patients also received external beam radiotherapy following the implant.Results. After a mean follow-up of 27 months (4-54) the local control rate for all 20 patients was 85% (17/20). In the 16 patients who had an intra-operative implant, local control was 94% (15/16). In the four patients who underwent a post-operative implant, local control was 50% (2/4). Actuarial 5-year survival was 90%. There were three cases (15%) of severe local complications.Conclusions. Wide local excision followed by low dose rate intersitital brachytherapy have yielded a 85% local control rate in 20 patients with soft tissue sarcomas. Local control rates were higher when the implants were done as an intra-operative procedure than as a post-operative one.     

Periosteal margin in soft-tissue sarcoma

BACKGROUND: Soft-tissue sarcomas frequently rest in contact with bone. The purpose of the study was to evaluate the risk of local recurrence for sarcomas adjacent to bone and to determine whether the periosteum provides an adequate margin of resection. METHODS: Fifty patients with soft-tissue sarcomas abutting bone were treated at a single institution between 1990 and 2004. All patients had high-grade, T2 (>5 cm), nonmetastatic disease in the lower extremity. Bone contact was verified by preoperative magnetic resonance imaging (MRI) and/or computed tomography (CT) scans. Forty-three of 50 patients received preoperative radiation with a mean dose of 50 Gy. In 11 cases a composite resection of bone and soft tissue was performed. In 39 cases the excision involved only soft tissue. RESULTS: True bone invasion was verified by histopathologic examination in 3 of 50 cases (6%). Local recurrence in the soft tissues developed in 8 of 50 (16%) patients. In no case did the recurrence involve destruction of cortical bone or erosion into bone. The recurrent tumor resided against the region of previous bone contact in 1 of 8 cases. There was no statistically significant difference in local recurrence between patients who had composite bone resection and patients who had soft-tissue resection only (P = .87). CONCLUSIONS: Relatively few sarcomas are able to penetrate cortical bone. Composite bone and soft-tissue resections are indicated primarily for frank bone invasion. In the absence of this, the periosteum is an adequate surgical margin for sarcomas treated with wide excision and radiation.     

Breast sarcoma. A clinicopathologic review of 25 cases

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.     

Extraskeletal subcutaneous osteosarcoma of the upper arm: A case report

Extraskeletal osteosarcoma (ESOS) occurs in approximately 1% of soft tissue sarcomas and 2-4% of all osteosarcomas. In particular, subcutaneous osteosarcoma is extremely rare, occurring in less than 10% of ESOS cases. This report presents a case of a subcutaneous tumor in the upper arm of a 79-year-old male. Imaging and pathological findings led to the conclusion that the soft tissue tumor should be diagnosed as subcutaneous osteosarcoma. Additionally, this case report documented the clinicopathological findings of the extraskeletal subcutaneous osteosarcoma in this case and discussed its clinical features by reviewing cases previously described in the literature.     

Chirurgische Therapie von Weichteilsarkomen der Extremitäten

Background

Surgery with curative intention in multimodal treatment concepts for patients with soft tissue sarcomas is the most important prognostic factor. Clear resection margins (R0) are one of the most important prognostic factors especially in the prevention of local recurrence and probably also in the overall survival of the disease. If R0 resection seems to be possible or can only be realized with mutilating procedures, neoadjuvant therapy concepts must be considered.

Objective

The principles of surgical therapy in patients with soft tissue sarcomas including multimodal strategies are discussed.

Material and methods

A systematic literature review of original articles and review articles over the last 15 years was performed. No prospective, randomized studies on surgery of soft tissue sarcomas were identified. The publications are discussed and assessed.

Results

In recent decades it could be shown that a compartmental resection has no significant advantages over wide resection with respect to local recurrence rate and overall survival. In the literature the rate of local recurrence is cited as being between 10?% and 40?% and the 5-year overall survival for all patients is approximately 50?%. In wide resections the ideal safety margin is not clearly defined. An R0 resection is therefore the most important criterion. A safety margin of at least 1 cm in all directions, as has been recommended for many years, can no longer be justified, the only exception being for liposarcoma (G1), the atypical lipoma of the extremities. Systemic chemotherapy (with or without hyperthermia) or radiotherapy can be beneficial and necessary in a multimodal neoadjuvant or adjuvant setting. With neoadjuvant radiotherapy a significantly increased rate of wound healing problems (>?30?%) in patients must be considered. Isolated hyperthermic limb perfusion (ILP) together with tumor necrosis factor alpha (TNF-alpha) and melphalan is an effective treatment option for patients with locally advanced soft tissue sarcomas of the extremities if an R0 resection could only be achieved by functional or anatomical amputations. Using this procedure allows resection of the soft tissue sarcoma and limb salvage in 81?% of patients. Reconstructive operative methods including flap surgery, vessel reconstruction and mesh grafts can be performed in approximately 20?% of patients.

Conclusions

A planned multidisciplinary concept from primary imaging, radiology, biopsy to histopathological investigation is necessary for defining the multimodal therapy and follow-up of patients with a soft tissue sarcoma. Surgery is still the key factor for local control and overall survival. The standard of care for soft tissue sarcomas of the extremities, with the exception of atypical lipoma, is a wide resection (R0). An ultraradical resection including vital structures for extending an already foreseeable free margin (R0) does not show any benefits. If a resection or re-resection cannot be performed in sano (i.e. R1), additional adjuvant or neoadjuvant radiotherapy should be included. The ILP procedure including TNF-alpha and melphalan is an effective treatment option in selected cases for patients with locally advanced soft tissue sarcomas of the extremities to avoid functional or anatomical amputations.     

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.