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PubMed1994

Epithelioid sarcoma of the orbit.

BACKGROUND: Epithelioid sarcoma is a rare and aggressive malignancy commonly occurring in the tendon sheaths of the distal extremities of young adults. This article presents the clinical and pathologic details of two patients with epithelioid sarcoma in the orbit, the first to be reported as occurring in this location. METHODS: The clinical history, results of physical and radiologic examination, surgical procedures, pathologic specimens, and clinical course of each patient were reviewed and recorded. RESULTS: Biopsies were performed of the masses that were found in the superotemporal orbit of both patients. Results of pathologic examination in each patient caused considerable difficulty, and the correct diagnosis was reached only after extensive consultation. The first patient underwent wide soft tissue and bone exenteration and is alive without recurrence at 3 years. The second patient had multiple recurrences and died of recurrent and metastatic disease at 29 months. CONCLUSIONS: Epithelioid sarcoma must be added to the differential diagnosis of orbital sarcomas. The histology is nodular with a pseudogranulomatous appearance. Based on our limited experience, epithelioid sarcoma of the orbit may have a course similar to that which occurs elsewhere in the body, requiring aggressive surgical treatment at the outset.

Adolescent↗

PubMed2002

Complex t(X;18)(p11.2;q11.2) with a pericentric inversion of the X chromosome in an adolescent boy with synovial sarcoma.

Synovial sarcoma is the most common nonrhabdomyosarcomatous soft-tissue sarcoma in children and young adults. It is characterized by the common t(X;18)(p11.2;q11.2) that results in the fusion of SYT on chromosome 18 to one of two closely related and adjacent genes on the X chromosome, SSX1 or SSX2. Here we describe a poorly differentiated, monophasic synovial sarcoma in a 17-year-old adolescent boy. Hyperdiploidy, a t(X;18)(q13;q11), and other structural abnormalities were detected by conventional cytogenetic analysis. Fluorescence in situ hybridization with the PAC probe RP3-519N18, which is specific for the Xp11 region, resulted in a signal on the der(Xq), a finding consistent with a pericentric inversion of the X chromosome that resulted in a t(X;18)(p11.2;q11.2)inv(X)(p11.2q13). Real-time polymerase chain reaction using primer sets specific for SYT-SSX1 and SYT-SSX2 confirmed the presence of an SYT-SSX1 fusion transcript. Our finding of this unique and complex translocation in synovial sarcoma demonstrates the utility of molecular methods in confirming the diagnosis of synovial sarcoma.

Adolescent↗

PubMed1996

Epithelioid sarcoma with an 18q aberration.

Epithelioid sarcoma is a peculiar soft-tissue neoplasm of uncertain origin, which is characterized by an epithelioid morphology of tumor cells coexpressing epithelial (keratin) and nonepithelial (vimentin) antigens. We herein report a new cytogenetic abnormality with der(22)t(18;22)(q11;p11.2) in a case of epithelioid sarcoma that occurred in the elbow of a 75-year-old man. Histologically, the tumor demonstrated a multinodular proliferation of epithelioid cells, with positive immunostaining for keratin, epithelial membrane antigen (EMA), and vimentin. Cultured tumor cells obtained from fresh surgical materials were frozen in plastic ampules and stocked in a liquid nitrogen freezer. Six years after surgery, the cells were recovered from the freezer and utilized for both morphologic and cytogenetic analyses. These cultured cells both before and after the freezing exhibited essentially the same epithelioid morphology and immunophenotypes as those of the original tumor. A chromosome analysis, together with fluorescence in situ hybridization (FISH), demonstrated a 61-67 modal population, and a characteristic clonal abnormality with der(22)t(18;22)(q11;p11.2). Other clonal abnormalities included numerical (-3, -4, +7, -13, -14, -16, -18, +20, -22) and structural (8p+, 9p+, 12p+, i(21q)) aberrations. Some variant clones also demonstrated i(18q). Since the breakpoint at 18q11 is similar to that reported in synovial sarcoma, this finding may support the presence of a histogenetic relationship between epithelioid sarcoma and synovial sarcoma. Our study thus indicates that the storage of frozen cells is useful for both morphologic and cytogenetic analyses of soft tissue tumors.

Aged↗

PubMed1999

Role of radiotherapy in sarcoma of the breast--a retrospective review of the M.D. Anderson experience.

BACKGROUND: The role of adjuvant radiotherapy for sarcoma of the breast, based on local extension of disease and patterns of failure, remains undefined because of the rarity of the disease presentation. METHODS: Fifty-nine cases of soft tissue sarcoma of the breast were retrospectively reviewed. Cystosarcoma phylloides was excluded from analysis. Surgical intervention consisted of segmental resection (n = 16) or mastectomy (n = 38); five patients underwent excisional biopsy. Adjuvant radiotherapy was administered in four patients following segmental resection and in 13 patients after mastectomy. Doses totaled 50 Gy in the majority of patients, and conventional criteria and radiotherapy techniques for adjuvant breast irradiation were used. RESULTS: None of the dissected axillary nodes contained metastatic tumor on pathologic review. Patterns of failure were evaluated. Tumor size (P < 0.03) and surgical margins (P < 0.002) were predictive of local failure (LF). Due to limited patient numbers, no statistical significance was identified with any treatment modality. Following mastectomy alone, LF occurred in 13 patients (34%) versus the 13% rate of LF with mastectomy and radiotherapy (P = NS). Distant metastases developed concurrently with the two local failures in the group that underwent mastectomy and radiation. After segmental mastectomy, LF occurred in 3 cases (25%) concurrent with distant metastases: no LF were noted after segmental mastectomy and radiation (P = 0.27). For all treatment groups, local recurrences were characterized as multiple and involved the chest wall. Local failure occurred in 60% of patients with positive surgical margins who did not receive adjuvant irradiation. Irrespective of surgical margins, over 75% of local recurrences developed among patients treated by surgery alone. CONCLUSIONS: The role of radiotherapy for breast sarcoma remains undefined due to the rarity of this disease presentation. This retrospective review failed to demonstrate a statistical benefit for the administration of adjuvant irradiation in sarcoma of the breast, probably because of limited patient numbers. Because large tumor size and positive surgical margins incur a higher risk for LF, radiotherapy is probably indicated in these cases. Axillary dissection obligates the radiotherapist to treat the axilla in order to include all tissues in the surgical bed, and should be avoided to reduce potential treatment related morbidity. Established therapeutic principles and techniques used for both soft tissue sarcoma and breast cancer should continue to be applied.

Adolescent↗

PubMed1999

Extraskeletal Ewing's sarcoma in a kidney transplant patient.

Organ transplant recipients are prone to develop a variety of malignancies, most of which are encountered uncommonly in the general population. Approximately 5% to 7% of these malignancies are sarcomas, of which most are Kaposi's sarcomas. Ewing's sarcoma is an extremely uncommon tumor in organ transplant recipients, and only one case of skeletal Ewing's sarcoma has been reported in the transplant literature. We present a case of extraskeletal Ewing's sarcoma (EES) in a renal transplant patient.

Abdominal Muscles↗

PubMed1990

An immunohistological comparison of primary lung carcinosarcoma and sarcoma.

We wished to assess the antigenic expression of primary lung tumors diagnosed as either carcinosarcoma or sarcoma in order to determine whether this information would be useful in distinguishing the two. We therefore immunohistochemically analyzed six pulmonary carcinosarcomas and five primary lung sarcomas for the presence of carcinoembryonic antigen (CEA), S100 protein, cytokeratin and vimentin using commercially available monoclonal and polyclonal antibodies on formalin fixed tissues. Six of six carcinosarcomas stained positively for cytokeratin while none of the sarcomas stained. In three carcinosarcomas both the carcinomatous and sarcomatous areas were positive while in three only the carcinomatous areas were positive. CEA staining was present in five carcinosarcomas and absent in all the sarcomas. CEA positivity was strong and not confined to those tumors with obvious gland formation. Staining for S100 protein was positive in two carcinosarcomas but only in those areas showing chondroid differentiation. Immunohistochemical staining for vimentin using two different monoclonal antibodies gave inconsistent results. We conclude that in differentiating between a carcinosarcoma and a sarcoma of the lung, immunohistochemical staining for both cytokeratin and CEA are useful with cytokeratin marginally preferable. The data indicate that carcinosarcoma of the lung, like that of the upper aerodigestive tract, expresses antigens suggesting both epithelial and mesenchymal differentiation.

Antigens, Neoplasm↗

PubMed1996

Corticomedullary tumors of the adrenal glands. Report of two cases. Association of corticomedullary tumor with spindle cell sarcoma.

We describe two cases of corticomedullary tumors of the adrenal gland. The patients suffered from Cushings syndrome and paroxysmal hypertension. The corticomedullary tumors consisted of benign looking cortical adenoma cells growing on the background of the pheochromocytoma cells. We further present the ultrastructural and immunohistochemical features of these tumors. Focally a spindle cell sarcoma arising from the corticomedullary tumor was found in one case. The spindle cell sarcoma was immunohistochemically negative with antibodies to chromogranin, synaptophysin, cytokeratin and S-100 protein. Ultrastructurally the sarcoma was composed of undifferentiated primitive cells poorly endowed with cytoplasmic organelles. Focal transitions of the pheochromocytoma into the spindle cell sarcoma were seen. It is hypothesized that the spindle cell sarcoma was arising from the pheochromocytoma component of the corticomedullary tumor.

Adrenal Cortex Neoplasms↗

PubMed1998

Expression of p21WAF1/CIP1 in soft tissue sarcomas: a comparative immunohistochemical study with p53 and Ki-67.

The p53 gene controls the cell cycle by transactivating p21WAF1/CIP1, a cyclin dependent kinase (cdk) inhibitor. By inhibiting cdks, p21WAF1/CIP1 regulates the cell cycle by blocking the G1 to S phase transition. In this study, we analyzed the immunohistochemical expression of p21WAF1/CIP1 in 66 soft tissue sarcomas and its relationship to p53 and the cell cycle proliferation antigen Ki-67. Expression of p21WAF1/CIP1, was detected in 76% of the tumors and p53 in 26%. All malignant schwannomas, synovial sarcomas, leiomyosarcomas and gastrointestinal stromal tumors expressed p21WAF1/CIP1. The majority of angiosarcomas, dermatofibrosarcomas, and fibrosarcomas showed low expression or were negative for p21WAF1/CIP1. Ewing's sarcomas, liposarcomas, and malignant fibrous histiocytomas were heterogeneous in their expression of p21WAF1/CIP1. Combining p53 and p21WAF1/CIP1 staining, the following four patterns were observed: 23% of the tumors showed the p53+/p21+ pattern; 53% showed the p53-/p21+ pattern; 3% showed the p53+/p21- pattern and 21% were negative for both p53 and p21WAF1/CIP1. There was no correlation between Ki-67 and p21WAF1/CIP1 or p53 staining. Our results show that soft tissue sarcomas, independent of their histologic subtype, frequently express p21WAF1/CIP1 which is probably important in their tumorigenesis. Additionally, p21WAF1/CIP1 may play a role in determining the efficacy of various cell cycle-directed therapies in soft tissue sarcomas.

Cyclin-Dependent Kinase Inhibitor p21↗

PubMed2001

The American Brachytherapy Society recommendations for brachytherapy of soft tissue sarcomas.

PURPOSE: This report presents the American Brachytherapy Society (ABS) guidelines for the use of brachytherapy for patients with soft tissue sarcoma. METHODS AND MATERIALS: Members of the ABS with expertise in soft tissue sarcoma formulated brachytherapy guidelines based upon their clinical experience and a review of the literature. The Board of Directors of the ABS approved the final report. RESULTS: Brachytherapy used alone or in combination with external beam irradiation is an established means of safely providing adjuvant local treatment after resection for soft tissue sarcomas in adults and in children. Brachytherapy options include low dose rate techniques with iridium 192 or iodine 125, fractionated high dose rate brachytherapy, or intraoperative high dose rate therapy. Recommendations are made for patient selection, techniques, dose rates, and dosages. Complications and possible interventions to minimize their occurrence and severity are reviewed. CONCLUSION: Brachytherapy represents an effective means of enhancing the therapeutic ratio, offering both biologic and dosimetric advantage in the treatment of patients with soft tissue sarcoma. The treatment approach used depends upon the institution, physician expertise, and the clinical situation. Guidelines are established for the use of brachytherapy in the treatment of soft tissue sarcomas in adults and in children. Practitioners and cooperative groups are encouraged to use these guidelines to formulate their treatment and dose-reporting policies. These guidelines will be modified, as further clinical results become available.

Adult↗

PubMed1999

Radiation therapy for consolidation of metastatic or recurrent sarcomas in children treated with intensive chemotherapy and stem cell rescue. A feasibility study.

PURPOSE: To assess the role of consolidative radiation therapy (CRT) in conjunction with myeloablative therapy with or without total body irradiation (TBI) in children and young adults with metastatic or recurrent sarcoma. METHODS AND MATERIALS: Twenty-one pediatric sarcoma patients with metastatic (10) or recurrent (11) disease were entered on a prospective feasibility study of intensive myeloablative therapy with or without TBI. Median patient age was 17.8 years (range, 9.4-24.7 years). Primary histologies included Ewing's (12), PNET (3), and other soft tissue sarcomas (6). Twenty patients received induction chemotherapy. Myeloablative therapy consisted of TBI in 11 patients with either high dose melphalan/etoposide (9) or high dose cytoxan/thiotepa (2). TBI consisted of 12 Gy in 2 Gy fractions delivered twice daily over 3 days. Ten patients received high dose chemotherapy alone, either with thiotepa/carboplatinum/etoposide (8) or cytoxan/carboplatinum (2). Myeloablative therapy was followed by autologous stem cell rescue (ASCR) 24 to 48 hours after completing chemotherapy. Fourteen patients (67%) received CRT either prior to (5) or following (9) myeloablative therapy. Median CRT dose was 37.2 Gy (range, 20-60). Fifty-one disease sites were present prior to myeloablative therapy. Twelve (24%) were bulky (> 8 cm) and 18 (35%) underwent surgical debulking. The median follow-up of surviving patients was 15 months (range, 8-20) with 25% of patients having been followed for more than 20 months. RESULTS: The 3-year actuarial disease-free (DFS) and overall survival (OS) rates for the entire group were 36% and 27%, respectively. Following myeloablative treatment, responses were: 11 complete, 6 partial, 1 stable, and 3 progressive disease. Sixteen patients (71%) have relapsed. The most common site of relapse was the lung (13). Of the 51 disease sites present prior to myeloablative therapy, 36 sites (71%) were amenable to CRT. Nonamenable sites were: multiple lung metastases (13) and bone marrow (2). Twenty-six amenable sites (51%) received CRT either prior to (14) or following (12) ASCR. Amenable sites treated with CRT had a better 3-year actuarial local control (80 vs 37%) (p = 0.0065) than amenable sites not treated with CRT. Factors associated with improved disease-free survival (DFS) in univariate analysis were induction chemotherapy response (p = 0.002) and extent of surgical resection (p = 0.045). There was a trend toward improved DFS on univariate analysis with the use of TBI as part of myeloablative therapy (p = 0.07). The one factor associated with improved OS on univariate analysis was induction chemotherapy response (p = 0.007). Multivariate analysis revealed that induction chemotherapy response is the only factor that remains significant for DFS (p = 0.032) as well as for OS (p = 0.017). Patients with complete response to induction therapy had 40% probability of survival versus all other patients who had 10% survival (p = 0.05). CONCLUSION: Consolidative radiotherapy is feasible in primary metastatic or recurrent pediatric sarcoma patients treated with myeloablative therapy with or without TBI. CRT to sites amenable to irradiation provided an improved 3-year actuarial local control than that seen in sites amenable to CRT that did not undergo radiotherapy. There was a trend for improved DFS with the use of TBI. Improved DFS and OS can be predicted by response to induction therapy. This intensive regimen may improve the cure rate of advanced pediatric sarcomas in select patients.

Adolescent↗

PubMed1998

Primary aortic intimal sarcoma of the endothelial cell type with long-term survival.

Primary tumors of the aorta are rare and are difficult to diagnose preoperatively. These tumors are malignant and ultimately fatal. A patient had initial evidence of aortoiliac obstructive disease. Aortic endarterectomy produced an excellent postoperative clinical result until the pathology report from the endarterectomy specimen revealed aortic sarcoma. The patient refused a second operation for en bloc aortic resection. One year later with recurrent symptoms, the patient underwent en bloc resection of the aorta and common iliac vessels until clear margins were obtained. The tumor was found to be intimal aortic sarcoma of endothelial cell origin. The only metastasis was to bone, and it was controlled with radiation and chemotherapy. The patient lived a total of 8 years from the time of initial diagnosis. Abdominal aortic intimal sarcoma of the endothelial cell type is a rare variety of aortic sarcoma that can be managed successfully if diagnosed early. This specific type of tumor has a longer survival period and better prognosis than other types of aortic sarcoma when managed with aggressive surgical resection, radiation, and chemotherapy.

Aged↗

PubMed2003

An audit of the management of soft tissue sarcoma within a health region in the UK.

AIMS: Soft tissue sarcomas are rare and heterogeneous tumours only occasionally seen by most individual clinicians. Early recognition, appropriate referral and timely investigations markedly improve outcomes. Our aim was to retrospectively assess the referral patterns, investigation, surgery and outcomes of patients with soft tissue sarcoma in the Trent region of the UK. METHODS: Two hundred and four patients with soft tissue sarcoma registered with the Trent Cancer Registry in 1995-1997 were first studied. Clinical details, tumour characteristics, presentation, management and follow-up were recorded from the case notes and analysed.A further group of 40 patients referred to a single cancer centre in 1999 were audited to establish whether there had been any improvements/changes over the 2 years, since the completion of the first audit. RESULTS: In the first audit, 49.5% were first referred to general surgeons and 16% to orthopaedic surgeons. Only 15% of patients fit for surgery were referred to a surgeon with a specialist sarcoma interest prior to definitive exploration. Of the deep tumours, 64% had a preoperative biopsy and only 68% had a scan before biopsy or definitive surgery. Tumour grade, completeness of excision and site of the sarcoma influenced survival. The second audit performed on patients treated in 1999 showed only a marginal improvement in management when compared to the first audit.

Abdominal Neoplasms↗

PubMed2005

[Intimal sarcoma of the inferior vena cava].

INTRODUCTION: Primary sarcomas of the great vessels, that is, the aorta, pulmonary artery, and inferior vena cava, are rare. They can be classified according to the location of the sarcoma in the vessel wall and by their gross appearance. Most often they are leiomyosarcomas or fibrosarcomas. CASE: We report here a case of an intimal sarcoma of the inferior vena cava. Histological and immunohistochemical findings confirmed the diagnosis for this 17-year-old girl and distinguished it from leiomyosarcoma and angiosarcoma, both of which have better prognoses. DISCUSSION: Intimal sarcoma of the inferior vena cava is rare and difficult to diagnose before surgery or biopsy. Histologically, it is a poorly differentiated tumor with the worst prognosis among the primary vascular sarcomas. Pathologic findings and immunohistochemical staining are useful for a positive diagnosis.

Adolescent↗

PubMed2004

Familial Kaposi's sarcoma: a cluster of five Israeli cases.

AIM: Familial cases of Kaposi's sarcoma (KS) are rare, and have all been described in patients with the classical variant of the disease. The predisposition of classical Kaposi's sarcoma among Jews is well known. We herein describe five families, all Jews, in which two members have Kaposi's sarcoma. To our knowledge, this has been the largest reported series of familial Kaposi's sarcoma. PATIENTS AND METHODS: The clinical course, management and response to therapy were described and compared with other published cases. RESULTS: No similarity was found in any of the families in time and age of onset of the disease, or in the severity and course of the disease among the members of the same family. There was a high incidence of second neoplasms among these familial cases. CONCLUSIONS: We discuss the potential implications of second neoplasms based on our understanding of the pathogenesis of the disease, as well as the influence or predisposal of some genetic mechanisms to the development of Kaposi's sarcoma.

Age of Onset↗

PubMed2001

Survival of children with soft-tissue sarcoma in Europe since 1978: results from the EUROCARE study.

Soft-tissue sarcomas account for 5--8% of all childhood cancers in European countries. In the EUROCARE childhood cancer study, there were 2163 registrations from 17 countries for soft-tissue sarcomas in patients aged 0--14 years during 1978--1989. Of this total, three-quarters were contributed by childhood cancer registries in Germany and the UK. Age-standardised 5-year survival rates of children diagnosed during 1985--1989 were 65% (95% confidence interval (CI) 56--72) for rhabdomyosarcoma, 68% (95% CI 58--77) for fibrosarcoma, 78% (95% CI: 64--87) for other specified soft-tissue sarcomas except Kaposi's and 51% (95% CI 37--65) for 'unspecified' soft-tissue sarcomas. Survival rates increased steadily throughout the 12-year study period for all soft-tissue sarcomas combined, but the increase took place predominantly in the early 1980s for rhabdomyosarcoma. Improvements in survival which had previously been reported from individual countries and in clinical series are confirmed as having taken place throughout much of Europe on a population basis. In a supplementary analysis, there was little indication of a further improvement during 1990--1992.

Adolescent↗

PubMed1993

Increased incidence of sarcoma in patients treated for testicular seminoma.

In a nationwide cancer registry analysis of second primary malignancies in 6187 men with testicular cancer in the period 1943-1987, 13 sarcomas were found, yielding a 4-fold increase of the relative risk (RR). The majority of sarcomas occurred in men with seminoma, and the increased incidence was seen irrespectively of time since the diagnosis of testicular cancer. The interval between the testicular cancer and the sarcoma varied from 5-34 years. After investigation of the hospital records and re-examination of the histological specimens, 3 patients were excluded. In spite of this, the RR was still considerably increased (at least 3-fold). Seven of the 10 sarcomas were found to be located within the field of the radiation treatment administered and three at the periphery. The absolute number of these secondary sarcomas is low, but the risk of developing such neoplasms and other malignancies should, even so, be kept in mind in the follow-up of testicular cancer patients.

Adult↗

PubMed1999

Quantitative methodologies for selection of patients with recurrent abdominopelvic sarcoma for treatment.

Peritoneal sarcomatosis is a major cause of surgical treatment failure in patients with abdominal or pelvic sarcoma. In the past, patients with this condition have had a lethal outcome. In this study, 43 consecutive patients with recurrent sarcomatosis were studied in order to evaluate an aggressive reoperative approach. In all patients, the goal of surgery was to resect all recurrent sarcoma in the abdomen and pelvis. In 30 patients in whom sarcomatosis was demonstrated and in whom a complete cytoreduction could be performed, resection was associated with peri-operative intraperitoneal chemotherapy with doxorubicin or cisplatin plus doxorubicin. Using a standardised and quantitative methodology to assess local-regional recurrence and dissemination on peritoneal surfaces, the clinical features that may affect prognosis were tabulated and analysed statistically. The median survival of these 43 patients was 20 months. Clinical features that had a significant impact on survival were involvement of less than six abdominopelvic regions (P = 0.0009), an increase in the involvement of abdominopelvic regions of less than four regions (P = 0.0007), involvement of less than 10 anatomic sites (P = 0.0002), complete cytoreduction with tumour reduced to nodules < 2.5 mm (P = 0.005) and a Peritoneal Cancer Index less than 13 (P = 0.01). Histological type and grade of recurrent sarcoma were not correlated with prognosis. In the multivariate analysis, an increase in abdominopelvic regions by four or more showed a risk ratio of 18.5. The involvement of 10 or more anatomic sites showed a risk ratio of 5.9. These data suggest that selected patients with recurrent sarcoma should be considered for further treatment and that the results of aggressive reoperative surgery and peri-operative intraperitoneal chemotherapy are greatly dependent on the volume and distribution of disease, determined at the initiation of therapy. Because of the great likelihood of local-regional treatment failure, the use of peri-operative intraperitoneal chemotherapy in a randomised study in patients with primary abdominopelvic sarcoma should be considered.

Abdominal Neoplasms↗

PubMed1999

Descriptive epidemiology of soft tissue sarcomas in Vaud, Switzerland.

Trends in incidence and survival from soft tissue sarcomas (STS) were analysed for the period 1974-1994 using data from the Cancer Registry of the Swiss canton of Vaud. A total of 645 cases were registered. The most common histotypes were fibrosarcoma (0.82/100,000 males, 0.86/100,000 females, world standard, 1990-1994), leiomyosarcoma (0.90/100,000 males, 1.28/100,000 females, 1990-1994), and Kaposi's sarcoma (3.10/100,000 males in 1990-1994). Overall incidence rates for STS increased from 2.68/100,000 males in 1974-1979 to 6.86 in 1990-1994, and from 3.61 to 4.27 in females. However, after excluding Kaposi's sarcoma, no consistent trend over time was observed, peak rates (approximately 4.40/100,000) being registered in the late 1980s for both sexes, with some levelling off thereafter. Five-year relative survival was 17% for Kaposi's sarcomas, and 51% for other STS (all STSs, 45%). These data indicate that there has been no major new risk factor for STS of such a relevance to modify appreciably the overall rates on a population level, except from the impact of the AIDS epidemic for Kaposi's sarcoma.

Adult↗

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