Body wall and visceral nonrhabdomyosarcoma soft tissue sarcomas in children and adolescents

Background

Predictors of outcome have not been established for pediatric visceral and body wall nonrhabdomyosarcoma soft tissue sarcomas (NRSTS).

Methods

The study used a retrospective review of clinical features and outcome of 61 patients with visceral and body wall NRSTS evaluated at our institution between March 1962 and December 1999.

Results

Median age at diagnosis was 9.9 years (range, birth to 17.4 years). Tumors were greater than 5 cm in 43 (70%), high grade in 33 (54%), invasive in 25 (41%), and metastatic at presentation in 14 (23%) patients. Visceral tumors (n = 27) were more likely than body wall tumors (n = 34) to be greater than 5 cm (93% vs 53%; P < .001) and invasive (70% vs 18%; P < .001) and were less likely to be resected at diagnosis (44% vs 85%; P = .001). Estimated 10-year event-free survival (EFS) and overall survival (OS) for the entire cohort were 45.5% ± 6.9% and 56.8% ± 6.7%, respectively. The 10-year EFS and OS were better for patients with body wall sites than for those with visceral sites (61.8% ± 8.5% and 67.5% ± 8.2% vs 24.2% ± 9.4% and 43.0% ± 10.3%; P = .004 and P = .004). The 10-year estimated cumulative incidence (CI) of local recurrence was higher for patients with visceral sites than for those with body wall sites (64.3% ± 9.8% vs 26.5% ± 7.7%; P = .004), whereas CI of distant recurrence was similar for the 2 sites (15.2% ± 7.2% vs 23.5% ± 7.4%; P = .39).

Conclusions

Pediatric patients with visceral NRSTS are more likely to have invasive, large, and unresectable tumors compared to those with body wall tumors. More than two thirds of visceral NRSTS recur locally, and fewer than half of patients with visceral tumors survive.     

Combined modality treatment of extremity soft tissue sarcomas

Background: Substantial progress has been made in the multimodality treatment of soft tissue sarcoma over the past decade. This review summarizes current state-of-the-art management for patients with extremity soft tissue sarcoma. Methods: Literature-based review of combined modality treatment of extremity sarcoma. Results: The revised staging system of the International Union Against Cancer/American Joint Committee on Cancer (UICC/AJCC) for this disease is described and a practical approach to diagnosis, evaluation, management, and follow-up of patients is presented. Brief summaries of the roles of surgical therapy, pre- and postoperative radiotherapy, pre- and postoperative chemotherapy, and concurrent chemoradiation in the management of extremity soft tissue sarcoma are provided, with summaries of the available data from randomized prospective trials. The data referenced and discussed herein provide the basis for stage-specific treatment recommendations for the management of patients with localized soft tissue sarcoma of the extremities. Conclusions: Soft tissue sarcoma remains a challenging disease to treat. The majority of patients require an integrated combined modality approach. Future research should be directed at developing more effective systemic therapies and minimizing the toxicities of local therapies.     

Oncosurgical and reconstructive concepts in the treatment of soft tissue sarcomas: a retrospective analysis

Background  Patients with soft tissue sarcomas are initially treated in community hospitals and in the private practice setting in the majority of cases. Thus, this disease entity is initially treated by surgeons with limited experience and expertise regarding the complex and demanding therapeutic regimen necessary to address this distinct group of malignancies. Methods  A retrospective analysis was conducted with a particular focus on factors affecting patient outcome, oncosurgical intervention, and plastic surgical reconstruction. Results  A total of 75 patients were included in the study. Only 66% of all patients presented with a primary tumor. The remaining 34% presented with either local recurrence or distant metastasis. The oncosurgical procedure most commonly performed was wide excision (69%). However, amputation was unavoidable in 7% of cases. Single-step reconstruction included primary closure (19%), skin grafting (19%), local fascio- or myocutaneous flap transfer (31%), as well as free flap transfer (31%). 17 patients (20%) have deceased due to their underlying disease. Yet another 10% are alive with evidence of residual tumor. Kaplan–Meier analysis displayed significant differences in overall survival rate depending on disease stage upon initial presentation. Conclusion  Based on our data favorable oncological outcomes with satisfactory functional results and limb preservation are achievable if oncological principles for resection are respected and reconstruction is performed according to plastic surgical principles.     

冷冻消融软组织肉瘤消融率的影响因素

目的探讨冷冻消融治疗软组织肉瘤消融率的影响因素。方法收集接受氩氦刀冷冻消融治疗的60例软组织肉瘤的资料,于冷冻消融治疗1个月后,根据患者情况选择CT或MRI检查判断消融率,对冷冻消融软组织肉瘤消融率的影响因素进行统计学分析。结果单因素分析显示,肿瘤最长径和肿瘤生长部位与软组织肉瘤冷冻消融术后消融率有关(χ~2=10.408,P=0.015;χ~2=36.778,P=0.006)。多因素分析显示,肿瘤生长部位是冷冻消融术后消融率的独立影响因素(P0.05)。结论冷冻消融治疗软组织肉瘤安全有效。软组织肉瘤生长部位是冷冻消融后消融率的独立影响因素。     

Prospective cohort study of neoadjuvant treatment in conservative surgery of soft tissue sarcomas

Background: 1994 marked a decade since the inception of a prospective population-based study on the value of a neoadjuvant approach for soft tissue sarcomas of head, neck, and limbs at the Tom Baker Cancer Centre, Calgary, Alberta. To date, 42 patients have been followed for a minimum of 5 years or until death. Methods: Each patient received a protocol of 60 mg to 90 mg of Adriamycin infused intraarterially or intravenously over 3 days into a vessel feeding the involved area, 30 Gy of radiotherapy given over 10 days, and complete resection of the sarcoma 4 to 6 weeks later. The lower dose was used empirically for smaller limbs (e.g., arm). Results: Two of the 42 patients were immediate failures of protocol, with one requiring amputation and one requiring later reexcision. In the 38 appendicular lesions, the ultimate limb salvage rate was 97.5%. All tumors were associated with a high risk of local recurrence, with 15 being previous local failures. The rest were deep and grade 2 or 3 lesions. Serious local complications were seen in one patient (2.5%) who had wound necrosis requiring reoperation. Minor wound complications were seen in five patients (12.5%) (one wound infection, one resolved edema, three long-term drainage). There was one local recurrence; thus 5-year local control was 97%. No patient had long-term morbidity related to the treatment. No effect on systemic control was suggested. Conclusion: Our report demonstrates that this combined modality approach provides superior local control of soft tissue sarcomas with low postoperative morbidity.     

腹膜后肿瘤外科治疗进展和争议

腹膜后肿瘤,主要是软组织肉瘤是一类来源于结缔组织的少见恶性肿瘤,异质性高,病理类型复杂,推荐应在具备多学科协作团队的区域性肉瘤中心进行综合治疗。对局部肿瘤施以切缘阴性的完整手术切除是对原发性腹膜后肉瘤（RPS）患者标准的、潜在根治性治疗,可以说外科治疗是RPS综合治疗的基础。近年来,腹膜后肿瘤的外科治疗取得了一些进展,同时存在一些争议。外科治疗在进展和争议中逐步成熟。     

Soft tissue sarcomas of the hand and foot: Management and survival

Background: Soft tissue sarcomas of the hands and feet present a challenge for limb-preserving resections. Methods: A retrospective review of 19 patients with sarcomas of the hand or foot was done. Wide or local excision was performed in 14 patients (74%), and amputation in 5 patients (26%). Of the latter group, three amputations involved a digit or toe, and two (10%) were major amputations (one Syme amputation and one below-knee amputation). When the minimum surgical margin was narrow (1 to 2 mm), adjuvant radiation was given postoperatively (n=4). Results: Local recurrence was observed in four patients (21%). Two of these required an amputation for local control. Local recurrence was observed in one of four patients (25%) treated with marginal resection and radiation and three of 15 (20%) of those with resection alone. Conclusions: A sizable percentage (37%) of patients with soft tissue sarcomas of the hand and foot ultimately required an amputation, although often the amputation was a minor one involving only a toe or a digit. Limb preservation was successful in the majority of patients (63%). The local recurrence rate was 21%, which may be improved with more frequent use of adjuvant therapy. The 5-year survival rate was 82%, which is better than that usually quoted for overall extremity soft tissue sarcomas.     

足底内侧岛状皮瓣联合腹股沟游离皮瓣修复足跟部软组织缺损

目的探讨足底内侧岛状皮瓣联合腹股沟游离皮瓣修复足跟部皮肤软组织缺损的临床效果。方法回顾性分析2015年10月至2020年12月榆林市第二医院烧伤整形手足外科收治的足跟部皮肤软组织缺损患者的临床资料。患者经急诊清创或扩创后,应用足底内侧岛状皮瓣修复足跟部创面,腹股沟游离皮瓣修复足底内侧供区创面,腹股沟供区直接缝合。术后观察皮瓣血运及存活、皮瓣供区愈合情况,并对足跟外形与功能恢复情况进行随访。结果共纳入8例患者,男7例,女1例,年龄20~71岁,平均32.2岁。其中5例为足跟外伤,1例足跟鳞状细胞癌,1例足跟冻伤,1例足跟溃疡。足跟部创面面积4 cm×3 cm~7 cm×6 cm。手术过程顺利,足跟岛状皮瓣及腹股沟皮瓣切取范围较足跟创面扩大0.5~1.0 cm。8例患者术后皮瓣血运良好,创面均一期愈合。术后随访3~12个月,所有患者足跟弹性佳,外形无臃肿,耐磨、耐压,感觉及行走功能满意;足底内侧供区无凹陷,无瘢痕增生及挛缩,无局部皮肤溃疡;腹股沟供区仅见线性瘢痕。结论足底内侧岛状皮瓣联合腹股沟游离皮瓣修复足跟部缺损,足部外观及功能恢复良好,效果满意。     

Management of extremity soft tissue sarcomas

Soft-tissue sarcomas are a rare and heterogeneous group of tumors. The last few decades have seen rapid strides in surgery with function preserving alternatives for local control in these lesions becoming the norm without compromising on overall disease survival. Good functional and oncological results can be achieved with a combination of excision of the tumor, and where required, suitable adjuvant therapies. These lesions are best managed at specialty centres where the requisite multidisciplinary care can be offered to the patient to optimise results. This overview is intended as a review of current understanding and the multimodality management of these challenging tumors.     

Dendritic cell and effector cell infiltration in soft tissue sarcomas with reactive lymphoid hyperplasia

The lymph node is the site of antigen presentation, and dendritic cells are sentinels for anti-tumor immunity. However, little is known about the histological features of lymph nodes and dendritic cells in soft tissue sarcomas. The reactive lymph node and infiltration of dendritic cells or effector cells were studied histologically in 10 soft tissue sarcomas with reactive lymphoid hyperplasia. The cases included four malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, one synovial sarcoma, one epithelioid sarcoma, one malignant granular cell tumor, and one liposarcoma. The proportions of the T zone, lymphoid follicle, and lymphoid sinus (which was occupied by cells immunopositive for antibodies against CD3, CD20, or CD68) were 33.4% ± 11.0%, 6.1% ± 4.9%, and 13.5% ± 6.5%, respectively. T zone hyperplasia was observed in all cases, and sinus histiocytosis was found in four. The proportion of the T zone in regional lymph nodes of soft tissue sarcoma patients was significantly higher than that in adult autopsy cases without a cancer history. CD8-, TIA-1-, or granzyme B-positive effector cells were found in each sarcoma tissue. Whereas CD1a-positive dendritic cells were not detected, S-100 protein-positive or CD83-positive dendritic cells were observed in five sarcoma tissues. The coefficient correlation between the numbers of effector cells and dendritic cells positive for CD83 or S-100 protein were demonstrated. Although this is a preliminary report, the present study demonstrated that some soft tissue sarcoma patients showed reactive lymphoid hyperplasia. Furthermore, the association between the infiltration of dendritic cells and that of effector cells was observed in patients with soft tissue sarcomas.     

Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma

Background Epithelioid sarcoma is a rare high grade soft tissue sarcoma with a known propensity for locoregional recurrence. The literature is limited on other characteristics such as frequency of multifocal disease at presentation, the relationship of presenting size of the primary lesion to prognosis, and the ability of current staging systems to predict prognosis. Methods Review of the Royal Marsden National Health Service Trust (RMH) experience of 37 cases over 21 years. Results The mean age was 29 years, with male predominance (2.7:1), and distal limb locatinis were most common (56%). Five patients presented with multifocal local disease. Median follow-up was 88 months in the 19 patients still alive. The 5- and 10-year actuarial overall survival was 70% and 42%, respectively. Tumors deep to the investing fascia had a worse prognosis than superficial tumors. Regional metastasis events were also associated with significantly worse overall survival. Local recurrence, size of 5 cm or larger, and regional metastasis events were predictive of worse distant metastasis-free survival. Tumor size (<5 cm vs.≥5 cm), local recurrence events, sex, and site were not significant predictors of survival. The American Joint Committee on Cancer/International Union Against Cancer staging systems and the recently proposed RMH staging system of the Royal Marsden National Health Service Trust provided poor differentiation of prognosis in epithelioid sarcoma. The 5-year actuarial local recurrence rate was 35%. The 5-year actuarial regional nodal metastasis rate was 23%. The actuarial 5-year distant metastasis rate was 40%, with pleuropulmonary metastases the most common site of metastatic disease, and 35% of pleuropulmonary metastases presented with pleural effusion. Median post-distant metastasis survival was 8 months. Conclusions Epithelioid sarcoma has unusual clinical behavior compared with other high grade soft tissue sarcoma. It has a propensity for multifocal disease at presentation, local recurrence, regional metastasis, and particularly poor prognosis after regional or distant metastatic disease. Size and stage according to the American Joint Committee on Cancer/International Union Against Cancer are unreliable predictors of prognosis.     

Neovascularity and clinical outcome in high-grade extremity soft tissue sarcomas

Background: Increased tumor neovascularity has been shown to correlate with poor prognosis in solid tumors. Methods: Microvessels were identified by factor VIII immunohistochemical staining. Analysis of microvessel counts, tumor characteristics, and resection details was performed on 119 primary, high-grade extremity soft tissue sarcomas (STS) and correlated with clinical outcome. Results: Tumor characteristics and resection details were analyzed and patient outcome was examined with respect to local recurrence, distant metastasis, and disease-specific survival. Factors found to be significant on univariate analysis for all outcome variables were positive microscopic margin and tumor size. A positive microscopic margin was found to be a significant risk factor for local recurrence (P=.03), distant metastasis (P=.006), and disease-specific survival (P=.004). A primary tumor greater than 10 cm in diameter was a poor prognostic factor for distant metastasis (P=.03) and disease-specific survival (P=.006) when compared to tumors smaller than 10 cm. Microvessel count did not correlate with survival nor did it predict distant metastasis or local recurrence. Histologic subtypes of STS that have a prominent vascular pattern as a diagnostic criterion (i.e., angiosarcoma, liposarcoma, hemangiopericytoma) form a subgroup of all STS. Neovascularity in these subtypes showed no relationship to clinical outcome. Conclusions: These data confirm the prognostic importance of microscopic margin and tumor size in high-grade extremity STS. Neovascularity measured by factor VIII staining had no prognostic significance in these mesenchymal tumors, in contradistinction to carcinomas. Alternatively, microvessel counts may not accurately represent the angiogenic capacity of STS. Therefore, patients with STS who are eligible for anti-angiogenesis clinical trials cannot be identified solely by microvessel count.Presented at the 50th Annual Cancer Symposium of The Society of Surgical Oncology, Chicago, Illinois, March 20–23, 1997.     

Adult head and neck soft tissue sarcomas

BACKGROUND: The purpose was to determine the optimal treatment for adult patients with head and neck soft tissue sarcomas. METHODS: We conducted a review of the pertinent literature. RESULTS: Local control after surgery alone or combined with radiotherapy was obtained in approximately 60% to 70% of the patients. The probability of local control is influenced by histologic grade, tumor size, and surgical margins. Patients with high-grade tumors and/or positive margins have improved local control if adjuvant radiotherapy is used. Distant metastases occurred in 10% to 30% of patients. The 5-year overall and cause-specific survival rates varied from approximately 60% to 70% and are affected by age, histologic grade, previous treatment of tumor, invasion of deep structures, and adequacy of surgery. CONCLUSIONS: The optimal treatment for adult head and neck soft tissue sarcomas is surgery. Adjuvant radiotherapy improves outcomes for those with high-grade tumors and/or positive margins. Radiotherapy alone will cure a small subset of patients with unresectable tumors.     

Contributions from surgeons to clinical trials and research on the management of soft tissue sarcoma

Background: Surgical resection is the primary treatment for soft tissue sarcoma. Surgeons are in a position to develop and define appropriate treatment strategies for this disease. In an effort to define the contributions of surgeons to the management of sarcoma, the surgical and clinical oncology literature from January 1983 through June 1996 was reviewed. Methods: A computerized literature search of the Cancerlit database for January 1983 to June 1996 was performed. The search was limited to the topic of soft tissue sarcoma and was further confined to 15 journals that publish articles relevant to surgical management. These studies were then categorized by multiple parameters and analyzed. Results: The Cancerlit file contained 4478 articles in which sarcoma was the primary topic. When the search was limited to 15 journals frequently read by surgeons, 479 references (11%) were retrieved. Within the surgical literature, 95 of the 479 articles (20%) described prospective studies, of which 33 were prospective and randomized. These studies represent all but three of the prospective randomized trials within the literature during the time period reviewed. The management of patients with sarcoma was evaluated in 26 of the prospective randomized trials; of these, 13 trials evaluated adjuvant chemotherapy, three evaluated adjuvant radiotherapy, and ten evaluated the chemotherapeutic treatment of metastatic disease. Surgical oncologists were the first or senior author on 10 of the 16 prospective randomized studies regarding adjuvant radiation or chemotherapy. Four of the 16 trials evaluating adjuvant therapy contained more than 100 patients, and three of those four were from cooperative group efforts. All but one of the studies of adjuvant therapy with less than 100 patients were from single institutional trials. Conclusions: Although the surgical and clinical oncology literature on soft tissue sarcoma is composed primarily of retrospective reviews, the prospective randomized trials reported represent almost all of the randomized trials in the literature and have significant contributions from surgeons. Surgeons can guide and design clinical trials, but overall patient accrual as represented by soft tissue sarcoma is low, and may be improved through cooperative group efforts.The opinions contained herein do not necessarily reflect the opinions of the Department of the Army or the Department of Defense.     

The management of soft tissue tumours

Summary Malignancy must be suspected in any mass of uncertain diagnosis in the trunk or limbs. The history and clinical, and radiological examination, including angiography, will facilitate a reasonable presumptive diagnosis. Biopsy should only be undertaken by the surgeon who will perform the definitive operation and should be planned so that the biopsy route can be excised later with the tumour. Extirpation biopsy can be performed if conditions are favourable.The value of angiography as a diagnostic aid is emphasized.Ten illustrative cases are reported.

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Résumé On doit soupçonner la malignité en présence de toute tumeur du tronc ou des membres de diagnostic incertain. L'histoire, l'examen clinique et radiologique, y compris l'angiographie, sont susceptibles d'aider à porter un diagnostic de présomption raisonnable. La biopsie doit être réalisée par le chirurgien qui exécutera la totalité du traitement et elle sera effectuée de telle sorte que la cicatrice puisse être excisée secondairement avec la tumeur. On pourra réaliser une biopsie-exérèse si les conditions sont favorables.L'auteur insiste sur l'intérêt de l'angiographie pour établir le diagnostic et présente 10 cas démonstratifs.

     

Retroperitoneal sarcomas in children: outcomes from an institution

Background

Retroperitoneal sarcomas are uncommon in children and tend to present as large tumors with advanced locoregional involvement of abdominopelvic structures. Surgical control of these tumors remains to be a management challenge. We reviewed our institutional experience with retroperitoneal sarcomas in children.

Materials and Methods

In a retrospective review of charts dating between 1975 and 2005, we identified patients younger than 18 years who were diagnosed with a histologically confirmed retroperitoneal sarcoma. Patients were followed prospectively through clinic visits and mail correspondence. Standard statistical methods were used for comparative, risk, and survival analyses.

Results

Twenty-one children with a mean age at presentation of 9 ± 1 years were identified. There were more boys than girls (male/female ratio = 1.6). The most common presentations were abdominal mass/distention (76%) and pain (62%). The mean tumor size was 14.2 ± 1.4 cm, with locoregional involvement in 62% of the patients. The common histologic types were rhabdomyosarcoma (33%) and fibrosarcoma (33%). Seventy-six percent of the patients underwent primary or secondary resection, 71% received neoadjuvant and/or adjuvant chemotherapy therapy, and 38% received radiation therapy. Complete resection was achieved in 48% of the patients, including 3 who required inferior vena cava resection and reconstruction. The 5-year disease-specific survival rates for patients who underwent complete resection and those who underwent incomplete resection were 90% and 36% (P = .018), respectively. For all patients, the mean survival time was 103 ± 16 months and the 5-year disease-specific survival rate was 62%. Survival was significantly better for patients with low-grade sarcomas than for those with high-grade sarcomas (90% vs 36%, P = .008). Among those who underwent an initial complete resection, 50% had a recurrence at a mean time of 88 ± 52 months (range = 3-261 months). The 30-day postoperative mortality and morbidity rates were 0% and 24%, respectively; in addition, 14% of the patients experienced long-term complications.

Conclusions

Resection of retroperitoneal sarcomas can be performed safely with minimal morbidity and mortality. Complete resection and low histologic grade are associated with significantly better disease-specific survival.     

原发性腹膜后软组织肉瘤的诊断与治疗

目的：探讨原发性腹膜后软组织肉瘤的诊断及治疗方法。方法：回顾性分析1995年8月～2006年5月收治的12例原发性腹膜后软组织肉瘤患者的临床资料,并就其临床表现、辅助检查及手术方法进行讨论。结果：12例中,男3例,女9例,年龄27～73岁,平均55岁。体检发现者3例,有腹部或腰部疼痛症状者6例,腹部包块者2例,下肢疼痛者1例。肿瘤最大径5～21cm,平均11cm,均行手术切除。手术时间120-360min,平均210min;失血200～1500ml,平均524ml,其中输血5例,占41％,输血4001000ml,平均600ml。同时切除右肾1例,右半结肠1例,无重大手术并发症。病理检查结果为脂肪肉瘤5例,平滑肌肉瘤3例,神经纤维肉瘤2例,恶性纤维组织细胞瘤1例,横纹肌肉瘤1例。随访8例,时间3～60个月,平均28个月,2例分别于术后6、9个月复发,再次行手术切除,1例术后1．5年因肝、肺转移而死亡,2例术后3～12个月局部复发,无法手术切除,转行放、化疗。结论：原发性腹膜后软组织肉瘤术前主要靠影像学检查诊断;手术完整切除是其主要的有效治疗手段。     

A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group.

Nonrhabdomyosarcoma soft tissue sarcomas in infants and children are rare malignancies with most of the clinical data gained by retrospective analysis. In 1986, a prospective multicentered study was initiated by the Pediatric Oncology Group (POG) with a total of 75 cases now entered for epidemiologic examination. Median age of presentation was 12 years (range, newborn to 20 years). The male to female ratio was 2.3 to 1. The most common soft tissue tumor was synovial cell sarcoma (32/75, 42%), followed by fibrosarcoma (10/75, 13%), malignant fibrous histiocytoma (9/75, 12%), and malignant neurogenic tumors (8/75, 10%). Sixty-five percent of all tumors presented on the extremities (44% lower extremity, 21% upper extremity). Tumors of the trunk accounted for 28% (abdomen 15%, thorax 13%), whereas head and neck tumors were 7%. By TNMG classification, 16% presented as stage I, 21% as stage II, 33% as stage III, and 30% as stage IV. Age at presentation did not affect clinical site or stage. All upper extremity tumors presented with localized disease, whereas lower extremity tumors presented with regional nodal disease in 7% and metastatic disease (pulmonary) in 23% of the cases. Seventy-eight percent of the abdominal tumors were metastatic at diagnosis; the other 22% had extensive regional disease. In the thorax 78% had localized lesions with 22% having extensive regional disease. Ninety percent of synovial cell sarcomas were on the extremities: 84% localized disease and 12% metastatic spread at presentation. Sixty percent of the fibrosarcomas presented on the extremities with 80% having localized disease and 20% metastatic spread. Only 25% of the neurogenic tumors presented with localized disease, whereas 50% had metastases at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Superficial extremity soft tissue sarcoma: An analysis of prognostic factors

Background: Experience with soft tissue sarcoma has suggested that superficial tumors have a favorable prognosis. We evaluated the prognostic features of this subset of sarcoma. Methods: Prospective data on 215 patients presenting to Memorial Sloan-Kettering Cancer Center with primary extremity superficial soft tissue sarcomas between July 1, 1982 and July 1, 1996 were analyzed. Superficial sarcomas were defined as subcutaneous tumors not invading the investing fascia of the muscle. Analysis was by univariate and multivariate tests for local recurrence, metastasis, and tumor mortality. Results: Ninety (42%) patients were over 50 years of age, 115 (53%) had high-grade tumors, 53 (25%) had tumors 5 cm, and 18 (8%) had positive margins following definitive resection. Median follow-up was 45 months (range 2 days to 151 months), 31 (14%) patients had local recurrences, 20 (9%) had distant metastases, and 15 (7%) died of disease. Five- and 10-year actuarial disease-specific survivals were 91% and 85%, respectively. On multivariate analysis, age >50 years predicted local recurrence (RR 5.7; 95% CI, 2.4–13.3;p<0.0001). High grade (RR 4.2; 95% CI, 1.4–12.7;p<0.006), and size 5 cm (RR 4.4; 95% CI, 1.8–11;p<0.002) predicted distant metastases. High grade (RR 7; 95% CI, 1.5–31.4;p<0.003), size 5 cm (RR 6.9; 95% CI, 2.3–20.8;p<0.0006), and positive margins (RR 3.8; 95% CI, 1.2–12.4;p<0.006) predicted tumor mortality. Conclusion: Primary superficial extremity soft tissue sarcomas have a favorable prognosis. Size and grade of superficial tumors are the strongest factors in predicting survival.Presented at the 50th Annual Cancer Symposium of The Society of Surgical Oncology, Chicago, Illinois, March 20–23, 1997.     

Reconstructive plastic surgery in soft tissue sarcomas of the extremities

Summary Limb-sparing resection in combination with radio/chemotherapy is recommended in cases of soft tissue sarcomas of the extremities. Plastic surgery techniques and clinical results in 69 patients with 71 malignant soft tissue tumors who had hadergone resection, as well as additional reconstructive procedures for functional improvement, are discussed. The integration of patients suffering from soft tissue sarcomas of the extremities into the treatment protocols of interdisciplinary tumor boards is recommended. Plastic surgery salvage procedures (including microsurgical free-tissue transfer) are discussed in detail. If the patient is judged unsuitable for these procedures or if distalization is not possible, amputation is the final option.