PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “soft tissue sarcomas”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 127 records · Page 7Linked to original sources

[Soft tissue sarcoma in adulthood].

Soft tissue sarcomas (STS) represent a heterogenous group of malignant tumors arising in mesenchymal tissue and in the autonomal and peripheral nervous systems. Only 1% of all malignancies in adults are STS. Most of them are localized at the extremities, but they also occur in the abdomen and the thorax as well as at the abdominal and chest wall. They are usually surrounded by a pseudocapsule which contains tumor cells and they can exhibit a discontinuous growth pattern. Macroscopically undetectable branches might grow along given anatomical structures. Thus the whole sarcoma-related anatomic compartment should be judged as tumor-contaminated. The high rate of local failure is often caused by insufficiently extended primary resections. Lymph node metastases are rare. The main prognostic factors are histological grading, tumor size and surgical radicality. Diagnosis of STS is often made at a rather late state of tumor progression often too late for curative therapy. Early histological diagnosis is thus of great importance. The operation aims at the removal of the whole tumor bearing anatomic compartment. Even wide excisions of the sarcoma surrounded by 3 cm of tumor free tissue will lead to at least 60% local recurrencies. Excisions along the pseudocapsule (enucleation) will most likely leave parts of the tumor behind. Insufficient surgical radicality cannot be compensated for by adjuvant therapies. The resection should be carried out without compromises.

Adult↗

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.

Adolescent↗

Optimizing radiation therapy and post-treatment function in the management of extremity soft tissue sarcoma.

When treating soft tissue sarcomas (STS) of the extremities, the major therapeutic goals are survival, local tumor control, optimal function, and minimal morbidity. Surgical resection of the primary tumor is the essential component of treatment for virtually all patients. However, local control by surgery alone is poor for the majority of patients with extremity lesions unless the procedure removes large volumes of grossly normal tissue (ie, widely negative margins are attained, because sarcomas tend to infiltrate normal tissue adjacent to the evident lesion). Thus, removal of the gross lesion by a simple excision alone is followed by local recurrence in 60% to 90% of patients. Radical resections reduce the local recurrence rate to 10% to 30%, but may compromise limb function. The combination of function-sparing surgery and radiation achieves better outcomes than either treatment alone for nearly all patients with STS. Because both surgical and radiation technique are critically important for optimizing local control of tumor and functional outcome, it is important to manage these patients in dedicated multispecialty clinics comprised of physicians with expertise in sarcomas, including orthopedic and general oncologic surgeons, radiation oncologists, medical oncologists, sarcoma pathologists, and bone and soft tissue diagnostic radiologists. Radiation therapy can be given by external beam radiation therapy (EBRT) or brachytherapy (BRT) or combination thereof. External beam radiation can be given either preoperatively or postoperatively. The clinical considerations and the outcome data that must be considered in choosing the most appropriate treatment technique for the individual patient are discussed.

Brachytherapy↗

The IVADo regimen--a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study of behalf of the European pediatric Soft tissue sarcoma Study Group.

BACKGROUND: The role of doxorubicin (Doxo) as part of multidrug regimens used to treat children with soft tissue sarcoma (STS) is controversial. To evaluate the feasibility of combining Doxo with the well established ifosfamide, vincristine, and actinomycin D (IVA) regimen, the Italian STS Committee performed a pilot study on a series of children with metastatic STS. METHODS: Between July 2002 and February 2004, 29 evaluable patients were enrolled in this study; 19 patients had rhabdomyosarcoma, 5 patients had peripheral neuroectodermal tumor, and 5 patients had other types of STS. The IVA-Doxo (IVADo) regimen included ifosfamide 3 g/m(2) on Days 1 and 2, vincristine 1.5 mg/m(2) on Day 1, actinomycin D 1.5 mg/m(2) on Day 1, and Doxo 30 mg/m(2) on Days 1 and 2. Three courses of IVADo were to be administered in the initial part of treatment and analyzed for toxicity and tumor response. RESULTS: Overall, 92 cycles were delivered. Major regimen-related toxicity was myelosuppression, with Grade 4 neutropenia in 67% of cycles and fever and neutropenia in 37% of cycles. Nonhematologic toxicity included Grade 3-4 mucositis (6.5% of cycles), constipation (9.7%), and peripheral neuropathy (6.5%). Other manifestations of major toxicity were venoocclusive disease and seizures, which occurred in one patient each. All but 1 patient with a malignant schwannoma showed some degree of tumor volume reduction; however, considering only complete and partial remissions, the response rate was 76% (+/- 7.9%). CONCLUSIONS: The intensive IVADo regimen was effective against pediatric STS with acceptable toxicity. This combination will be investigated in high-risk patients with rhabdomyosarcoma in a randomized trial launched by the European pediatric Soft tissue sarcoma Study Group.

Adolescent↗

Evaluation of P-glycoprotein expression in soft tissue sarcomas of the extremities.

Soft tissue sarcomas comprise a heterogeneous group of mesenchymal tumors accounting for less than one-percent of adult neoplasms. In the last few years, the use of adjuvant chemotherapy has been proposed for the treatment of these lesions in order to obtain a better systemic control, but its usefulness is still controversial. In this study, we evaluated whether P-glycoprotein, a membrane protein strictly associated with multidrug resistance, is overexpressed in soft tissue sarcomas. By using human multidrug resistant sarcoma cell lines as controls, we analyzed P-glycoprotein expression in 34 primary and in 23 relapsed soft tissue sarcomas of the extremities. Overexpression of P-glycoprotein was found in 6 out of 34 primaries (18%) and in 8 out of 23 relapses (35%). In particular, in malignant fibrous histiocytoma, the most frequent soft tissue sarcoma of adults, P-glycoprotein overexpression was found in 23% of primary untreated cases, in agreement with the reported relapse rate of this tumor after surgery and chemotherapy. These data suggest that, in soft tissue sarcomas, overexpression of P-glycoprotein may be of prognostic value and that the assessment of P-glycoprotein expression may be useful for the design of chemotherapy protocols.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86.

PURPOSE: The goal of the second German Soft Tissue Sarcoma Study CWS-86 (1985 to 1990) was to improve the prognosis in children and adolescents with soft tissue sarcoma by means of a clinical trial comprising intensive chemotherapy and risk-adapted local therapy. PATIENTS AND METHODS: There were 372 eligible patients. A staging system based on the postsurgical extent of disease was used. Chemotherapy consisted of vincristine, dactinomycin, doxorubicin, and ifosfamide. Radiotherapy was administered early at 10 to 13 weeks simultaneously with the second chemotherapy cycle (32 Gy or 54. 4 Gy). The single dose was reduced to 1.6 Gy and given twice daily (accelerated hyperfractionation). RESULTS: The event-free survival (EFS) and overall survival rates at 5 years were 59% +/- 3% and 69% +/- 3%, respectively. The 5-year EFS rate according to stage was as follows: stage I, 83% +/- 5%; stage II, 69% +/- 6%; stage III, 57% +/- 4%; and stage IV, 19% +/- 6%. The outcome for patients with stage III disease who required radiotherapy was much better in the CWS-86 study compared with the CWS-81 study (5-year EFS, 60% +/- 5% v 44% +/- 6%; P =.053). The most common treatment failure was isolated local relapse, with 14% of patients relapsing at the primary tumor site. CONCLUSION: The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

Adolescent↗

Preoperative multimodality treatment for soft tissue sarcomas.

BACKGROUND: Frequently, soft tissue sarcomas (STS) do not present until they are large, thus making local control difficult. Consequently, different methods of preoperative cytoreduction should be evaluated. METHODS: This study evaluated a 10-day preoperative regimen of intraarterial doxorubicin (10 mg/m2/d), with concomitant radiation therapy (25 Gy), administered to 55 adult patients with either Stage T2 (n = 41) or distal Stage T1 (n = 14) STS. Seven of the tumors were low grade. All patients were treated by the Division of Surgical Oncology at the University of Illinois between 1978-1991. RESULTS: This regimen was successful and obviated the need for amputation in 47 patients who underwent wide soft tissue excision, an initial limb-salvage rate of 87%. Complications related to the therapy occurred in 26% of patients, which resulted in additional operative procedures in 7%. The mean follow-up time was 94 months. The 5-year overall survival rate was 69%, with a disease-free survival rate of 51%. Local control was unsuccessful in 15% of the patients. Three additional patients required amputation for recurrent disease (n = 2) or complications of therapy (n = 1), resulting in an ultimate limb-salvage rate of 81%. CONCLUSIONS: The long-term results of this preoperative protocol for adults with limb-threatening STS appears to justify the utilization of a multimodality approach. This preoperative regimen was useful in minimizing limb loss in patients with limb-threatening STS. However, intraarterial doxorubicin administration is associated with significant morbidity, and its role in multimodality treatment of STS requires further clarification.

Adolescent↗

Does a radiation dose-response relation exist concerning survival of patients who have soft-tissue sarcomas of the extremities? Radiation dose-response relation for soft-tissue sarcomas.

This study analyzes a single-institution experience by evaluating the impact on survival of increasing total dose of adjunctive irradiation in patients who had extremity soft-tissue sarcoma (ESTS). A retrospective review of the tumor registry at a university medical center from January 1984 through December 1992 yielded a total of 59 surgical patients of ESTS. With follow-up ranging from 30 to 135 months (median, 65 months), the 2-, 5-, and 8-year overall and disease-free survival for all patients was 86%, 71%, 58% and 76%, 70%, 56%, respectively. Multivariate analyses using the Cox proportional hazards model revealed that total radiation dose (p = 0.02), American Joint Committee on Cancer stage (p = 0.04), and tumor size (p = 0.006) were all significant prognostic factors of overall survival; however, only tumor size was predictive of disease-free survival (p = 0.02). When the effect of tumor size and disease stage were controlled in the Cox model, a dose-response curve between increasing total radiation dose and improved overall patient survival was indicated. This study demonstrates the significance of tumor size on predicting both overall and disease-free survival in patients who have soft-tissue sarcomas of the extremity. It also suggests, however, that a radiation dose-response relation may exist for overall survival. Future investigations should consider evaluating the minimal total radiation dose needed to optimize patient survival after limb-sparing surgery.

Adolescent↗

High-dose epirubicin is not an alternative to standard-dose doxorubicin in the treatment of advanced soft tissue sarcomas. A study of the EORTC soft tissue and bone sarcoma group.

The activity and toxicity of single-agent standard-dose doxorubicin were compared with that of two schedules of high-dose epirubicin. A total of 334 chemonaive patients with histologically confirmed advanced soft-tissue sarcomas received (A) doxorubicin 75 mg m(-2) on day 1 (112 patients), (B) epirubicin 150 mg m(-2) on day 1 (111 patients) or (C) epirubicin 50 mg m(-2) day(-1) on days 1, 2 and 3 (111 patients); all given as bolus injection at 3-week intervals. A median of four treatment cycles was given. Median age was 52 years (19-70 years) and performance score 1 (0-2). Of 314 evaluable patients, 45 (14%) had an objective tumour response (eight complete response, 35 partial response). There were no differences among the three groups. Median time to progression for groups A, B and C was 16, 14 and 12 weeks, and median survival 45, 47 and 45 weeks respectively. Neither progression-free (P = 0.93) nor overall survival (P = 0.89) differed among the three groups. After the first cycle of therapy, two patients died of infection and one owing to cardiovascular disease, all on epirubicin. Both dose schedules of epirubicin were more myelotoxic than doxorubicin. Cardiotoxicity (> or = grade 3) occurred in 1%, 0% and 2% respectively. Regardless of the schedule, high-dose epirubicin is not a preferred alternative to standard-dose doxorubicin in the treatment of patients with advanced soft-tissue sarcomas.

Adult↗

High prognostic significance of Mdm2/p53 co-overexpression in soft tissue sarcomas of the extremities.

Soft tissue sarcomas are a heterogeneous group of neoplasms with various histological subtypes. Up to now, no individual causal molecular markers for prognosis and therapeutic success have been identified. A tumorigenic connection between the oncogene product Mdm2 and tumor suppressor p53 is generally accepted, but their possible clinical relevance has not yet been investigated sufficiently in soft tissue sarcoma. In 86 primary soft tissue sarcoma of the extremities (RO-resected, T1/2 N0 M0), Mdm2 and p53 overexpression were investigated by immunohistochemistry. The results were adjusted to clinico-pathological characteristics and evaluated for their prognostic relevance by multivariate analysis. In Cox's multivariate analysis with stratification of Mdm2 to p53 results, we determined four groups which had different prognostic values for relapse-free and overall survival (Mdm2-/p53- < Mdm2-/p53+ < Mdm2+/p53- < Mdm2+/p53+). The most striking finding was a relative risk (rr) for overall survival of 18.77 (P=0.006) for patients with Mdm2/p53 co-overexpression (n=40). It is noticeably higher than the additive risk from both factors. Coincident Mdm2/p53 overexpression is an independent molecular marker with the highest prognostic relevance described for soft tissue sarcoma. Thus, a high risk sarcoma group has been defined which we believe requires alternative therapeutic approaches.

Adult↗

Role of chemotherapy in patients with soft tissue sarcomas.

The management of soft tissue sarcomas has been highlighted in the last few years by the responsiveness of gastrointestinal stromal tumors to imatinib (Gleevec, Novartis). In this article, the use of chemotherapeutic agents in the management of this and some of the 50 or more subtypes of sarcomas are discussed, and a brief review of the use of chemotherapy in the adjuvant or neoadjuvant setting for people with large extremity sarcomas is provided. Doxorubicin and ifosfamide (Mitoxana, Bristol-Myers Squibb) remain the best individual drugs for sarcomas overall, although dacarbazine and gemcitabine (Gemzar, Eli Lilly) with or without a taxane has activity in at least a subset of sarcomas. The data regarding adjuvant chemotherapy for extremity soft tissue sarcomas is still quite mixed, with little if any overall survival advantage found to support its incorporation into disease management. The finding of tyrosine kinase inhibitors such as imatinib with demonstrated activity in gastrointestinal stromal tumors and dermatofibrosarcoma protuberans, as well as the finding of new agents such as ecteinascidin-743 (Yondelis, PharmaMar) with at least some activity against soft tissue sarcomas, reinforces the idea that we should target individual subtypes of sarcoma, just as treatment varies by subtype for the hematological malignancies.

Chemotherapy, Adjuvant↗

Pathology of soft tissue sarcomas. Selected current issues.

Soft tissue sarcomas are a heterogeneous group of malignant tumors with a wide range of clinical presentation, morphologic features and biologic behavior. During the past two decades, many acceptable new entities have been proposed, and the diagnosis and classification of these tumors have been modified. This paper reviews several current issues that are considered worthy of note for the histologic diagnosis of soft tissue sarcomas.

Humans↗

Limb-sparing therapy for soft tissue sarcomas.

Effective therapy for soft tissue sarcoma of the extremities continues in its lengthy evolution from mandatory amputation or radical compartment resection to multimodality limb salvage procedures. This current approach typically includes preoperative neoadjuvant systemic chemotherapy, followed by negative margin surgical resection in conjunction with radiotherapy. The scope and functional outcome of these resections has been markedly enhanced by the application of microvascular-dependent free tissue transfer techniques. Because of the rarity of this tumor system, problems in soft tissue sarcoma receive proportionally less attention. Nonetheless, the past year has witnessed several important advances in this field, as is discussed.

Amputation, Surgical↗

[Intraoperative radiotherapy as part of the treatment concept of soft tissue sarcomas].

The therapy of soft tissue sarcomas is not uniform. The goal of treatment for soft tissue sarcomas is surgical resection with negative microscopic margins. Other therapy options are pre-, intra-, or postoperative radiotherapy as well as neoadjuvantor adjuvant chemotherapy with different drugs, systemic or under local perfusion of the limb. If a margin-free surgical resection is not possible, intraoperative radiation therapy (IORT) offers the possibility for treatment of sites at high risk for local recurrence. The goal of this collaborating study of the Orthopedic University Hospital of Heidelberg and the department of Radio-Oncology of the University Hospital of Heidelberg is to observe the influence of IORT on the rate of local recurrence, complications, metastases, and survival in another wise unchanged tumor management. From 1960 to 1998,78 patients were treated for soft tissue sarcomas; 26 of 78 patients underwent intraoperative radiation and 52 patients were treated without intraoperative radiation. We found a recurrence rate of 15% in the IORT group and 29% in the non-IORT group. No difference for survival or distant recurrence was found, also no difference in early postoperative complication rate, but a higher rate of late complications after IORT (34% vs 7%) was remarkable. We think intraoperative radiation allows better local control and for that reason IORT is integrated into our therapeutic concept. Its influence on survival and distant metastases needs further investigations.

Adolescent↗

Delay in referral to a specialist soft-tissue sarcoma unit.

BACKGROUND: Soft-tissue sarcoma is a rare neoplasm which can occur almost anywhere in the body. There are no specific symptoms and signs and the disease can mimic more common benign and malignant conditions. It is not surprising, therefore, that diagnosis and referral to a specialist unit is often delayed. METHODS: A prospective audit was undertaken in 216 patients referred to a specialist unit with suspected or proven soft-tissue sarcoma over a 12 month period. All patients were interviewed to determine the chronology of symptoms and signs, and the referral and diagnostic pathway. Delay in referral was defined as three months or greater from first presentation to a doctor to review at our unit. RESULTS: There were 159 patients with previously untreated soft-tissue sarcomas. Thirty-one patients had delays in referral, and the overall median delay was 14 months (range 4-96 months). The delay was caused by the general practitioner in 13 patients, the patient (after general practitioner referral) on two occasions, by the hospital or referring specialist in seven patients, and was multifactorial in nine patients. CONCLUSIONS: A fifth of patients with soft-tissue sarcoma encountered important delays in referral to this specialist unit. This duration of delay is likely to have had a detrimental effect on treatment options and outcomes, including survival in some patients.

Adult↗

Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: a study by the EORTC Soft Tissue and Bone Sarcoma Group.

CAELYX/DOXIL, pegylated liposomal doxorubicin, has shown antitumour activity and reduced toxicity compared with standard doxorubicin in other tumour types. In this prospective randomised trial, 94 eligible patients with advanced soft-tissue sarcoma (STS) were treated, 50 with CAELYX (50 mg/m(2) by a 1 h intravenous (i.v.) infusion every 4 weeks) and 44 with doxorubicin (75 mg/m(2) by an i.v. bolus every 3 weeks). Histological subtypes were evenly matched, 33% were leiomyosarcoma (CAELYX: 18; doxorubicin: 13). Primary disease sites were well matched. CAELYX was significantly less myelosuppressive, only 3 (6%) patients had grade 3 and 4 neutropenia, versus 33 (77%) on doxorubicin; febrile neutropenia occurred in 7 (16%) patients given doxorubicin, but only 1 (2%) given CAELYX. 37 (86%) patients on doxorubicin had grade 2-3 alopecia, but only 3 (6%) on CAELYX, and the major toxicity with CAELYX was to the skin. Palmar-plantar erythrodysesthesia with CAELYX was grade 1: 4 (8%) patients, grade 2: 11 (22%) patients, grade 3: 9 (18%) patients and grade 4: 1 (2%) patient. Other non-haematological grade 3 and 4 toxicities were rare. Confirmed responses were observed with both agents: CAELYX: complete response (CR) 1 (uterine), partial response (PR) 4 (response rate (RR) 10%); and doxorubicin: CR 1, PR 3 (RR of 9%); with the best response being stable disease (NC) in 16 and 18 patients, respectively. The reason for the low response rate is unknown, but it may be due partly to a high proportion of gastrointestinal stromal tumours. In conclusion, CAELYX has equivalent activity to doxorubicin in STS with an improved toxicity profile and should be considered for further investigation in combination with other agents such as ifosfamide.

Adult↗

Head and neck and intra-oral soft tissue sarcomas.

Intra-oral soft tissue sarcoma is a rare disease. We reviewed a tumour registry over a 45 year period, between January 1951 and January 1997, and identified a total of 11,250 head and neck cancers in British Columbia. Of the head and neck cancers, there were 139 cases (1.24%) of sarcoma; and of these, there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The initial presentation of intra-oral soft tissue sarcoma is most often as an asymptomatic mass and discomfort is reported by less than one half of the patients. The commonest lesion identified was rhabdomyosarcoma (RMS). In this review, we identified 2 unique cases, 1 case of intra-oral hemangiopericytoma and 1 case of oral carcinosarcoma. The prognosis remains poor. Treatment includes wide surgical excision which may be supplemented with radiotherapy and chemotherapy.

Adolescent↗

Soft tissue sarcomas in infants younger than 1 year of age: a report of the German Soft Tissue Sarcoma Study Group (CWS-81).

Of 357 patients with soft tissue sarcomas (STSs) who were registered between 1981 and 1986 in the CWS-81 study, 21 (5.8%) were younger than 1 year of age. Six cases were probably of congenital origin since they were diagnosed in the 1st month of life. There was no difference in clinical grouping (staging and primary site) between infants and older patients. The major histological subsets comprised 12 patients with embryonal rhabdomyosarcoma (57% vs. 44% in older patients) and five patients with fibrosarcoma (23% vs. 2% in older patients). Of 21 patients, 17 received chemotherapy. The response rate (tumor reduction more than 1/3) to exclusive cytostatics therapy within 7-9 weeks, of ten evaluable patients, was 100% (vs. 86% in older patients). Eight of the responders achieved complete remission after 16 weeks of therapy and are in continuous complete remission (CCR). Most patients received 20-50% of the recommended chemotherapy dose for older children, which was calculated using body surface area. No fatal or life-threatening complications were observed. The event-free survival rate for children younger than 1 year of age with rhabdomyosarcoma was 70% vs. 47% in older children (no significant difference). Now, 2-5 years after therapy all five fibrosarcoma patients are alive and free of disease. Our data indicate that children under 1 year of age with rhabdomyosarcoma have a slightly better prognosis than older children, when therapy modalities are adapted to age.

Age Factors↗