Soft tissue sarcomas of the popliteal fossa: a single-institution retrospective review

BACKGROUND:

Soft tissue sarcomas (STSs) arising from the popliteal fossa present a challenge with regard to local control of primary tumors. Due to concerns of functional morbidity and neurovascular compromise, there is debate about what represents the best therapy for these patients.

METHODS:

We conducted a retrospective medical record review of patients treated at The University of Texas M. D. Anderson Cancer Center for STS of the popliteal fossa from 1990 to 2008.

RESULTS:

There were 47 eligible patients, 28 of whom were male and 19 of whom were female. Synovial sarcoma was the most common diagnosis, with 12 cases. Most patients had T2b tumors (31 patients; 66%). The median duration of follow‐up was 3.8 years (range, 0.6‐17.9 years). The 5‐ and 10‐year overall survival rates were 63% and 51%, respectively. Metastasis at diagnosis was associated with poorer overall survival (5‐year overall survival, 74% versus 13%; P<.001) and poorer recurrence‐free survival (5‐year recurrence‐free survival, 51% versus 0%; P<.001) on univariate analysis. Radiation therapy improved local recurrence‐free survival (5‐year local recurrence‐free survival, 56% versus 17%; P = .004), whereas a trend was observed for surgical margin status (P = .07). Tumor size and neurovascular involvement did not influence outcome. Twenty‐two patients had recurrent disease, with 15 patients having local recurrence, and 16 patients died from progressive disease.

CONCLUSIONS:

Radiation therapy may play an important role in the treatment of popliteal fossa STS, but further study is needed to better define the best clinical application. Additional study is needed to re‐evaluate association of surgical margin status and outcome. Cancer 2011; © 2010 American Cancer Society.     

Soft tissue sarcomas of the head and neck: outcome and prognostic factors

Background. Soft tissue sarcomas of the head and neck are uncommon malignant tumors. Up till now, their treatment has not been standardized. We retrospectively reviewed the records of adult patients with soft tissue sarcomas of the head and neck to identify prognostic factors affecting local control and survival. Methods. The records of 48 adult patients with soft tissue sarcoma of the head and neck region treated between 1987 and 1997 were reviewed. The data were analyzed for the impact of potential prognostic factors on local control, disease-free survival, and overall survival. Factors evaluated were age, sex, tumor grade, T-stage, bone invasion, site, surgical margin, treatment modality, and radiation dose. Results. The 5-year actuarial local control rate of the entire group was 40%. Tumor size was the only predictor for local control on multivariate analysis. Combined surgery and radiotherapy appeared to yield superior local control compared with surgery alone (46% vs 35%); however, the difference was not significant (P < 0.06). The 5-year actuarial overall and disease-free survivals were 48% and 34%, respectively. On multivariate analysis, tumor size was a significant factor for local control and for overall and disease-free survivals, while histological grade was a significant predictor only for disease-free survival. Conclusion. Tumor size was a significant predictor factor for local control and for overall and disease-free survivals. Histological grade was another significant predictor, affecting only disease-free survival. There was a trend for better local control with the addition of postoperative radiotherapy, although the difference did not reach significance, because of probable selection bias among the patients who were indicated for radiotherapy. Received: December 14, 1998 / Accepted: May 24, 2000     

Soft tissue sarcomas of the lower extremity: surgical treatment and outcome.

AIMS: The aims of this retrospective follow-up study were to evaluate the justification for limb-saving multi-disciplinary treatment of soft tissue sarcomas (STS) and to report the results achieved by our treatment protocol. Local control was studied, with emphasis on tumour depth, operation margin and need for post-operative radiotherapy. METHODS: We examined 130 patients with STS in the lower limb referred to the multi-disciplinary group of Helsinki University Central Hospital. For the survival analysis 106 patients with local disease remained. The goal of treatment was to preserve a functional limb. Wide excision was attempted. If the margin was less than 2.5 cm, post-operative radiotherapy (RT) was delivered to all except 20 patients. RESULTS: Ninety-two per cent of the patients were treated by limb salvage. The success rate of free flaps was 16 out of 18. The 5-year disease-specific overall survival was 76%, metastasis-free survival 72% and local control 79%. Prognostic factors for local recurrence were extracompartmental site and large size; for development of metastases high grade, extracompartmental site and large size; for decreased disease-specific overall survival high grade, large size and advanced age. Local control of intramuscular tumours (n=6) was 100%, subcutaneous tumours (n=38) 94% and tumours penetrating the deep or muscle fascia and those locating extracompartmentally (n=62) 67%. CONCLUSIONS: Limb salvage in patients with STS is possible with an acceptable outcome by selective combination of treatment modalities. Modern plastic surgical methods with free tissue transfers are successful and often needed. Subcutaneous and intramuscular tumours have good local control. The outcome is poorest for tumours penetrating the deep or muscle fascia and for those located extracompartmentally, and patients with such tumours might be a target for adjuvant therapy. Treatment should be coordinated by multidisciplinary teams.     

Soft tissue sarcomas

Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body, particularly the trunk, retroperitoneum, or the head and neck. Pretreatment radiologic imaging is critical for defining the local extent of a tumor, staging the disease, guiding biopsies, and aiding in diagnosis. Core-needle biopsy is the preferred biopsy technique for diagnosing soft tissue sarcomas. The American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcomas is based on histologic grade, the tumor size and depth, and the presence of distant or nodal metastases. Despite improvements in local control rates with wide local resections and radiation therapy, metastasis and death remain a significant problem in 50% of patients who present with high-risk soft tissue sarcomas. The most common site of metastasis is the lungs, and metastasis generally occurs within two to three years after the completion of therapy. Progress in the molecular characteristics of these tumors should in the near future translate into molecularly based therapies that can be incorporated into standard treatment strategies.     

Soft tissue sarcomas of the extremity

In 1955, in a series of abstracts entitled "A Half Century of Effort to Control Cancer," Pack and Ariel wrote, "in each individual case, there is a constant battle of judgement between the wisdom of more radical amputation and the need for preservation of that important functional part." This dilemma remains with us today. In his paper on the histogenesis of tumors, Stout, relating to the philosophy of treatment, wrote that "the best chance of curing malignant tumors of the soft tissues lies in the hands of the therapist who makes the first attempt." Pack's words are as true today as they were in 1955, and although limb salvage is possible for many patients with soft tissue sarcomas, preservation of an extremity at the risk of patient survival is not a feasible option. Most efforts at less than amputative surgery must entail multimodality therapy. Although there are some reports of success with single-agent therapy, such as surgery alone, the majority of successful, nonamputative series include surgery with radiation and/or chemotherapy. It must be emphasized that an extremely important aspect of the treatment of these patients is the judgment and evaluation by the primary physician. Therefore it is extremely important that the primary physician be familiar with the many factors involved in prognosis, both in terms of local control and of systemic illness, in order for the patient to be informed of treatment options. If this is not possible, the patient should be referred to a center that is involved in active research protocols or treatments. Although rare, the soft tissue sarcomas remain among the most difficult to treat, even though over the years there has been significant progress in diagnosis, classification, and successful local control. Patient survival is based on a multitude of factors that include the histogenesis of the tumor, its grade, size, anatomical location, the surgical procedure performed, the use of preoperative or postoperative radiation, and the clinical stage of the disease--whether it is primary or metastatic. In time, it is probable that additional factors will be found.     

Soft tissue sarcomas involving the pelvis

BACKGROUND AND OBJECTIVES: Soft tissue sarcomas (STS) of the true pelvis are rare tumors and there is little information in the literature related to pelvic STS. The purposes of this review were to understand the anatomic extension of these tumors to better plan surgical treatment and to determine the outcome of these patients. METHODS: Eighteen consecutive patients presenting between 1987 and 1995 with soft tissue sarcomas involving the true pelvis were retrospectively reviewed at minimum follow-up of 18 months. Cross-sectional imaging was reviewed for each patient to determine the anatomical location of the lesions. RESULTS: The tumors were confined to the true pelvis in 4 patients, extended to the retroperitoneum in three cases, and extended to the thigh in 11 patients. Adjuvant radiation was administered to all but 2 patients who had received radiation to the region in the past and all patients underwent surgical resection (local resection in 13 patients and hindquarter amputation in 5 patients). Surgical resection had a high rate of morbidity and complications including positive resection margins in nine individuals. Of the 18 patients, 11 died at a mean time of 15.5 (2-58) months from surgery, 4 were alive with evidence of disease at a mean time of 44.3 (18-68) months, and 3 were alive with no evidence of disease at a mean time of 57 (43-71) months. CONCLUSIONS: Soft tissue sarcoma of the pelvis is fortunately a rare disease with a high risk of local and systemic disease progression despite treatment with irradiation and surgical resection.     

Menstrual and reproductive factors and risk of soft tissue sarcomas

BACKGROUND: Soft tissue sarcomas (STS) are a heterogeneous group of neoplasms whose etiology remains largely undefined. A role for female hormones in the development of STS has been suggested. To investigate this possibility, the authors analyzed data from a hospital-based case-control study conducted in Northern Italy between 1983 and 1998. METHODS: Cases were 104 women aged < 79 years with incident STS who were admitted to the cancer institutes and major teaching and general hospitals. Controls were 505 women admitted to the same network of hospitals for acute, nonneoplastic, nongynecologic, and nonimmune-related conditions. RESULTS: The multivariate odds ratio (OR) for women aged >/= 15 years compared with those aged < 12 years at menarche was 1.94 (95% confidence intervals [95% CI], 0.80-4.74). No association with STS risk was observed for menstrual cycle pattern, age at menopause, parity, and abortions. Late age at first pregnancy and birth were found to be related to an increased risk of STS, with an OR of 3.16 (95% CI, 0. 96-10.44) and 2.79 (95%% CI, 0.79-9.90) for women aged >/= 30 years at first pregnancy and birth compared with those aged < 20 years. The trend in risk was significant for age at first pregnancy. No relation with the risk of STS emerged for age at last birth and time since first or last birth. CONCLUSIONS: The risk of STS was found to be weakly related to late age at first pregnancy or birth, but not to other menstrual and reproductive factors.     

Soft tissue sarcomas of adults: state of the translational science.

Sarcomas--like leukemias, which are also mesodermal malignancies--carry biological significance disproportionate to their clinical frequency. Identification of mutations and translocations associated with these tumors has illuminated aberrant signaling pathways that cause these diseases, determine their behavior, and are therapeutic targets. Activated receptor-associated tyrosine kinase c-kit, mutated in most gastrointestinal stromal tumors, has proven a clinically effective target for enzyme inhibition. A translocation involving a single gene family, consisting of EWS and related genes, has been identified in five different sarcomas, and its chimeric protein products could prove similarly amenable to inhibitors. Resolution of the histopathological complexity is being aided by data from molecular and chromosomal characterization. Improvements in imaging, definition of prognostic factors, and surgical and radiotherapeutic treatment have resulted in improved local control. Continued progress will depend on further adapting the rapidly evolving technologies of genomics and proteomics. It will also depend upon accurate histopathological diagnosis based on validated reagents and consistent methodologies applied to adequate tissue samples derived from patients with complete clinical data. Finally, multicenter, coordinated trials, such as those that occurred with assessment of imatinib mesylate in metastatic gastrointestinal stromal tumors, will assure the most rapid reductions in morbidity and mortality.     

Soft tissue sarcomas of the hand and foot

A retrospective study was made of 82 soft tissue sarcomas of the hand and foot. Prognosis was related to histopathologic grade of malignancy and success or failure of local tumor control. After treatment with curative intent, low-grade tumors gave a 5-year survival rate of 90%, compared to 63% for high-grade tumors. Simple excision or limited amputation of the digit, hand, or foot yielded a 5-year survival rate of 68% (15/22) and a local recurrence rate of 32%. Amputation below the elbow or knee or higher resulted in a similar survival of 68% (19/28) but no local recurrences. All patients who developed local recurrences died, except for two with low-grade tumors and one patient lost to follow-up. These observations indicate the need for systemic chemotherapy to prevent death from metastasis, and for effective local tumor control. Conventional radiation therapy may be difficult to apply at these sites. Innovative local treatment approaches to preserve limb function without jeopardizing tumor control merit study.     

Soft tissue sarcomas in Iceland 1955-1988. Analysis of survival and prognostic factors

From 1955 to 1988 a total of 129 cases (69 males and 60 females) of soft tissue sarcomas were diagnosed in Iceland, four at autopsy. The median age was 55 years (0-91). All the cases have been reviewed clinically and histopathologically and graded on both a three- and a four-point scale. The average age-standardized incidence was 1.8/100,000 for males and 1.6 for females. The tumour was most often localized in the thigh and retroperitoneal space. The most common histologic subtypes were malignant fibrous histiocytoma (22.5%), liposarcoma (18.6%) and leiomyosarcoma (16.3%). The 5- and 10-year survival rates (n = 125) were 38% and 29% respectively. Cox's multivariate analysis was performed on the following prognostic factors: age, sex, tumour localization, histopathologic subtype, tumour size, malignancy grade and year of diagnosis. The strongest prognostic factor was malignancy grade (IV vs I; p less than 0.001 and RR = 5.35 and III vs I; p = 0.017 and RR = 2.01) followed by tumour size (pT2 vs pT1; p less than 0.001 and RR = 3.09 and pT3 vs pT1; p = 0.002 and RR = 3.40) and year of diagnosis (p = 0.003 and RR = 0.96; corresponding to a 54% reduction in mortality risk during a 20-year period).     

Low-grade soft tissue sarcomas of the extremities. Analysis of risk factors for metastasis

Of 130 patients with low-grade localized soft tissue sarcomas of the extremities treated at Memorial Hospital between 1968 and 1978, 18 (14%) developed metastases. The risk of metastasis could not be predicted by the clinical or pathologic size, tumor depth, proximal location, recurrence before presentation or subsequent to treatment at our institution, the type of surgical and adjuvant therapies, and the adequacy of surgical margins. The median age of patients with metastases was 10 years older than the other patients (57.5 years and 47.5 years, respectively). Of the more common sarcomas in this study, malignant peripheral nerve tumors and malignant fibrous histiocytomas had the highest prevalence of metastasis (27% (three of 11) and 17% (three of 18), respectively). Although patients with low-grade extremity sarcomas treated during the first 5 years of this review had an increased rate of metastasis, no other patient, tumor, or treatment variable examined significantly differed between the two time periods. Although grade alone is a good predictor of the risk of metastasis from soft tissue sarcomas of the extremity, 14% of patients in this series with low-grade histology developed metastases. Further review of larger numbers of these tumors should be undertaken in an effort to more definitively identify risk factors predictive of metastatic potential in low-grade sarcomas.     

Soft tissue sarcomas in skin: presentations and management

Soft tissue sarcomas are a rare but heterogeneous family of malignant tumors that are predominantly found deep to the integumentary layer. Only a small number of these primary mesenchymal tumors actually originate from the dermal layers. A systematic approach to the evaluation and workup of these neoplasms can prevent inappropriate management. After staging evaluation, most of these tumors are primarily managed with en-bloc surgical resection. Other adjuvant therapies routinely employed include chemotherapy and radiation therapy. Proper treatment typically involves participation of a multidisciplinary care team for optimal outcome. General principles and treatment strategies will be discussed along with a review of the more common cutaneous manifestations of sarcoma.     

Soft tissue sarcomas: Preoperative versus postoperative radiotherapy

External beam radiation may be given either before or after excision of a primary soft tissue sarcoma. This study was undertaken to determine whether or not the timing of radiotherapy was associated with any difference in either local control, survival, or incidence of complications. The files of 112 patients with a primary, nonmetastatic, extremity soft tissue sarcoma, treated with limb salvage surgery and irradiation were evaluated. Data regarding tumor stage, grade, site, surgical margin, dosage and timing of radiotherapy, treatment complications, disease relapse, and relapse-free survival (RFS) were analyzed. Kaplan-Meier lifetable analysis was used to determine survival estimates. There was no significant difference in the 5-year RFS between patients receiving radiotherapy (RT) preoperatively versus postoperatively; 56 ± 15% and 67 ± 12% (P = 0.12, Mantel-Cox), respectively. There was no significant difference in the overall survival between patients receiving RT preoperatively versus postoperatively; 75 ± 15% and 79 ± 11% (P = 0.94), respectively. Actuarial local control at 5 years for preoperative versus postoperative RT patients was not statistically different; 83 ± 12% versus 91 ± 8% (P = 0.41), respectively. Wound complications were more frequent in preoperative RT patients (31%) compared to postoperative RT patients (8%) (P = 0.0014, chi-square). Preoperative irradiation was not associated with any benefit in terms of relapse-free survival, overall survival or actuarial local control in this series. A higher incidence of major wound complications was found among patients treated with preoperative irradiation. We recommend that patients with a resectable extremity soft tissue sarcoma be treated with postoperative irradiation, reserving preoperative irradiation for those situations in which either the tumor is initially thought to be unresectable or the original tumor boundaries are obscured. © 1996 Wiley-Liss, Inc.     

Soft tissue sarcomas in infancy and childhood

Thirty-one soft tissue sarcomas in children were studied. The majority of these tumors occurred between the ages of 6 and 10; there were 18 boys and 13 girls. Three main histologic types were encountered, 14 children had fibrosarcoma, 13 rhabdomyosarcoma, 2 had solitary malignant schwannoma, and in 2 the histogenetic origin could not be determined. Head and neck region was found to be the most common anatomical site, and 16 of 31 patients had their primary tumors in this region. In the rhabdomyosarcoma group of the 11 eligible patients, 6 lived free of disease for 5 years; in the fibrosarcoma group of the 12 eligible patients, 6 lived for 5 years or more. Both children with solitary malignant schwannoma are living. It is proposed that rhabdomyosarcoma in children be treated by combining irradiation, chemotherapy, and surgery while fibrosarcoma should be treated by excision alone. Furthermore, it is also proposed that, when the histogenetic type of a tumor can not be determined, these children be treated as having rhabdomyosarcoma.     

Soft tissue sarcomas of the adult head and neck

From 1969 to 1983, 53 adult patients with head and neck soft tissue sarcomas were evaluated and treated by the Division of Surgical Oncology at the University of Illinois. The most common anatomic location was the neck (36%), and these patients had the highest 5-year disease-free survival rate (67%). Fibrosarcoma was the most common histologic type (26%); patients with aggressive fibromatosis had the longest mean survival time (93 months). The mean overall survival time was 58.7 months, and the disease-free 2-year, 5-year, and 10-year survival rates were 68%, 54%, and 28%, respectively. Wide excision was the treatment of choice, with adjuvant radiotherapy or chemotherapy, or both, used in selected patients. In all of the long-term survivors, the tumors were either well-differentiated or less than or equal to 5.0 cm in diameter. It is apparent that aggressive therapy of such tumors can provide good long-term results.