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PubMed2000

Clinical value of [18-F]] fluorodeoxyglucose positron emission tomography imaging in soft tissue sarcomas.

OBJECTIVE: To evaluate positron emission tomography (PET) using 2-fluoro-2-deoxy-D-glucose (FDG) for clinical application in soft tissue sarcomas. SUMMARY AND BACKGROUND DATA: FDG PET is a promising noninvasive method for the preoperative assessment of soft tissue sarcomas and may complement radiologic tomography. METHODS: Data from 50 consecutive patients with 59 masses, either suspicious for primary or locally recurrent soft tissue sarcoma, were prospectively gathered. The semiquantitative FDG uptake (standardized uptake values [SUVs]) was calculated in tumor and normal tissue (muscle). Histopathology of surgical specimens and follow-up data were used as control criteria. RESULTS: In primary soft tissue sarcomas, PET displayed a sensitivity of 91% and a specificity of 88%. Local recurrence was detected with a sensitivity of 88% and a specificity of 92%. All intermediate-grade and high-grade soft tissue sarcomas (primary and locally recurrent) were visualized with a precise differentiation from muscle. Fifty percent of the low-grade sarcomas showed an FDG uptake equivalent to muscle (false-negative results in one primary and three recurrent soft tissue sarcomas). Benign soft tissue tumors (e.g., lipoma, leiomyoma, ganglion) did not accumulate FDG. Inflammation resulted in an increased FDG uptake. The semiquantitative FDG uptake (SUVs) correlated with tumor grade but not with size and histologic type. CONCLUSION: High-grade and intermediate-grade soft tissue sarcomas are amenable to PET imaging, whereas low-grade lesions may not be depicted. SUVs for FDG correlate with tumor grade in soft tissue sarcomas. Benign soft tissue tumors are differentiated from higher-grade soft tissue sarcomas. These data show that FDG-PET can complement preoperative radiologic assessment for soft tissue sarcomas and that FDG-PET is a powerful diagnostic tool for detecting high-grade and intermediate-grade local recurrence.

Adolescent↗
PubMed2000

Kaposi's sarcoma and central nervous system disease: a real association or an artifact of the control group? Terry Beirn Community Programs for Clinical Research on AIDS.

OBJECTIVES: To test the hypothesis that Kaposi's sarcoma (KS) protects against four central nervous system (CNS) diseases in HIV-1-infected individuals. STUDY POPULATION AND DESIGN: The study population of 9404 subjects included participants in Terry Beirn Community Programs for Clinical Research on AIDS (CPCRA) protocols who were enrolled between September 1990 and September 1998. This was an observational study. METHODS: Proportional hazards regression was used to estimate adjusted relative risks for predictors of four central nervous system diseases. Covariates included occurrence of Kaposi's sarcoma, occurrence of other opportunistic infections or malignancies, baseline CD4+ count, and other baseline characteristics. RESULTS: Among the 5944 participants without progression to AIDS at entry, 451 developed a CNS disease. The adjusted relative risk of any CNS disease for those who developed Kaposi's sarcoma versus those who did not develop any AIDS-defining event was 1.41 [95% confidence interval (CI), 0.98-2.03; P = 0.06]. In contrast, the adjusted relative risk of any CNS disease for those with Kaposi's sarcoma versus those with some other non-Kaposi's sarcoma AIDS-defining event was 0.37 (95% CI, 0.24-0.57; P < 0.0001). Among the 3460 participants with progression to AIDS at entry, the adjusted relative risk of any CNS disease for those with Kaposi's sarcoma versus those with some other non-Kaposi's sarcoma AIDS-defining event was 0.71 (95% CI, 0.40-1.25; P = 0.23). CONCLUSIONS: Our analyses indicate that the risk of CNS disease associated with Kaposi's sarcoma depends strongly on the reference or control group chosen. When compared to individuals with other non-Kaposi's sarcoma AIDS-defining diseases, Kaposi's sarcoma is associated with a lower risk of CNS disease in HIV-1 positive individuals. However, when compared to individuals with no AIDS-defining disease or with a similarly mild AIDS-defining disease such as invasive candidiasis, Kaposi's sarcoma is associated with an equivalent risk of CNS disease.

AIDS Dementia Complex↗
PubMed2000

Intrathoracic Kaposi's sarcoma in women with AIDS.

STUDY OBJECTIVE: To describe the radiographic features of intrathoracic Kaposi's sarcoma in women with AIDS. SUBJECTS AND METHODS: From 1987 to 1998, we identified seven women with biopsy-proven (n = 4) or autopsy-proven (n = 3) pulmonary Kaposi's sarcoma. Charts were reviewed for HIV risk factors, cutaneous and/or oropharyngeal Kaposi's sarcoma, CD4 cell count, and differential diagnosis of pulmonary disease prior to the diagnosis of pulmonary Kaposi's sarcoma. Chest radiographs (n = 6), chest CT scans (n = 3), and reports of unavailable chest radiograph (n = 1) closest to the time of diagnosis of pulmonary Kaposi's sarcoma were reviewed for the following: nodular and peribronchovascular opacities; thickened interlobular septa; pleural effusions; lymphadenopathy; and radiographic stage. RESULTS: Mean patient age was 33 years (range, 27 to 42 years). HIV risk factors were IV drug use (n = 2), heterosexual contact (n = 3), and both (n = 2). All patients had prior opportunistic infections. The median CD4 cell count was 18 /microL (mean, 63/microL; range, 5 to 210/microL). Cutaneous Kaposi's sarcoma was diagnosed prior to pulmonary Kaposi's sarcoma in four patients, subsequently in two patients, and not identified in one patient. Oropharyngeal Kaposi's sarcoma was diagnosed prior to pulmonary Kaposi's sarcoma in three patients. Only infection was considered in the differential diagnosis of the patients' pulmonary disease in five patients. One patient presented with acute hemoptysis and died, and one patient recently received a diagnosis of pulmonary Karposi's sarcoma at another hospital. Chest radiographic findings were the following: nodular opacities in five of seven patients (71%); peribronchovascular opacities in six of seven patients (86%); thickened interlobular septa in two of seven patients (29%); pleural effusion in three of seven patients (43%); and lymphadenopathy in two of seven patients (29%). Five of seven patients (71%) were determined to be in radiographic stage 3, one patient in stage 1, and one patient in stage 2. CT demonstrated additional lymphadenopathy in three of three patients, thickened interlobular septa in two of three patients, and pleural effusion in one of three patients, but it did not change the staging of disease in any patient. CONCLUSION: Pulmonary Kaposi's sarcoma can cause diffuse lung disease in women with AIDS. The disease is usually mistaken clinically for pulmonary infection.

Acquired Immunodeficiency Syndrome↗
PubMed1991

[Radiation induced sarcoma of the shoulder girdle].

A well-known complication after irradiation of tissue is development of postradiation sarcomas, and the shoulder girdle is in this connexion a frequent location, because it relatively often is exposured to x-rays. During the period 1956 to 1989 121 patients with sarcomas located to the shoulder girdle were referred to the Sarcoma centre in Arhus. Of these, six were postradiation sarcomas. The indication for the initial irradiation was in two cases cancer of the breast, in one malignant lymfogranulomatosis, in one a metastasis from malignant melanoma and finally two cases of peritendinitis humeroscapularis. In average 15 years (7-26 years) elapsed from irradiation to the diagnosis of the sarcomas. There were four bone sarcomas, two located in the clavicles and 2 in the humeri. Of these, three were osteogenic sarcomas and one a malignant fibrous histiocytoma. There were two soft tissue sarcomas, both located subcutaneously with involvement of deep fascia and muscle. Both tumors were extraskeletal osteogenic sarcomas. Three patients died of tumor on an average after 11 months. Two died without tumor from other causes, and one patient is alive without tumor 11 years after the treatment. If a patients presents with pain at the side of prior radiation, the diagnosis postradiation sarcoma must be considered and the patient referred to the Sarcoma centre. Radiation therapy should not be used in patients with benign lesions.

Adult↗
PubMed1996

Interphase cytogenetic study of endometrial stromal sarcoma by chromosome in situ hybridization.

Endometrial stromal neoplasms include a heterogenous group of tumors with different clinical behavior and response to treatment. Cytogenetic study of such tumors has been scanty. The aim of this study was to analyze and compare the chromosome composition in low-grade and high-grade endometrial stromal sarcoma using the technique of chromosome in situ hybridization. Eight cases of low-grade stromal sarcoma and three cases of high-grade stromal sarcoma were studied. Biotinylated DNA probes specific for the regions of chromosomes X, 11, 12, and 17 were used on formalin-fixed paraffin embedded material from these tumors. The in situ hybridization signals were visualized by immunoperoxidase technique. Four of the eight low-grade stromal sarcomas retained normal disomy of the chromosomes studied. The other four low-grade sarcomas showed a gain of from one to three chromosomes, whereas all of the three high-grade sarcomas showed polysomies in all of the four chromosomes being studied. No loss of chromosomes was detected. One case of high-grade sarcoma contained coexisting areas of low-grade sarcoma. Although aneusomy was found in the high-grade portion, disomy was noted in the low-grade areas. In the stromal sarcomas studied, there was no definite correlation between the presence of chromosome polysomies and the clinical progress of the tumors. This is the first interphase cytogenetic study of uterine stromal sarcoma, and the results support the concept that complex numerical chromosome abnormalities evolve during anaplastic transformation of endometrial stromal sarcoma.

Adult↗
PubMed1976

Type C particle-positive and type C particle-negative rat cell lines: characterization of the coding capacity of endogenous sarcoma virus-specific RNA.

Various rat cell lines have been analyzed for expression of endogenous RNA homologous either to RT21C, a typical rat type C virus, or to Kirsten sarcoma virus. Cells have been found that express either (i) high levels of RNA homologous to RT21C rat type C virus and low levels of RNA homologous to Kirsten sarcoma virus (RT21Chigh,sarclow) or (ii) high levels of RNA homologous to Kirsten sarcoma virus and low levels of RNA homologous to typical rat type C virus (sarchigh, RT21Clow). The properties of these two classes of cell lines have been compared. Each type of cell contains an equal amount of the expressed RNA on polysomes. Cell lines that are RT21Chigh produce abundant rat p30 nad p12 structural proteins and release rat type C particles containing viral RNA and reverse transcriptase into supernatant fluids from these cultures. Cell lines that are sarchigh,RTC21Clow have no detectable rat viral p12 protein and no p30 protein immunoreactive in even broad interspecies radioimmunoassays, and do not release type C particles into the supernatant from the cultures. When the particle-negative cell lines are superinfected with heterologous mouse or wooly type C viruses or are producing typical rat type C virus particles, the endogenous sarcoma virus-specific RNA is secreted from these cells. The sarcoma virus-specific RNA can be transcribed in complementary DNA in the endogenous reverse transcriptase reactions carried out in vitro with such virus preparations. However, exposure of cells that are permissive to the helper virus with the particles containing sarcoma virus-specific RNA has not yet resulted in cell transformation or in the synthesis of these RNA sequences. The results suggest: (i) that the first step in the genesis of sarcoma viruses involves the packaging of this expressed sarcoma virus-specific RNA in helper viral particles; (ii) that efficient transmission of the sarcoma virus-specific RNA requires additional events; and (iii) that the formation of a stable sarcoma virus by recombination between the helper viral genome and part of the rescued sarcoma virus-specific RNA is much less common event than the rescue process itself.

Base Sequence↗
PubMed2002

Endogenous gonadal hormone exposure and bone sarcoma risk.

Although experimental and clinical evidence suggest that endogenous sex hormones influence bone sarcoma genesis, the hypothesis has not been adequately tested in an appropriate animal model. We conducted a historical cohort study of Rottweiler dogs because they frequently undergo elective gonadectomy and spontaneously develop appendicular bone sarcomas, which mimic the biological behavior of the osteosarcomas that affect children and adolescents. Data were collected by questionnaire from owners of 683 Rottweiler dogs living in North America. To determine whether there was an association between endogenous sex hormones and risk of bone sarcoma, relative risk (RR) of incidence rates and hazard ratios for bone sarcoma were calculated for dogs subdivided on the basis of lifetime gonadal hormone exposure. Bone sarcoma was diagnosed in 12.6% of dogs in this cohort during 71,004 dog-months follow-up. Risk for bone sarcoma was significantly influenced by age at gonadectomy. Male and female dogs that underwent gonadectomy before 1 year of age had an approximate one in four lifetime risk for bone sarcoma and were significantly more likely to develop bone sarcoma than dogs that were sexually intact [RR +/-95% CI = 3.8 (1.5-9.2) for males; RR +/-95% CI = 3.1 (1.1-8.3) for females]. Chi(2) test for trend showed a highly significant inverse dose-response relationship between duration of lifetime gonadal exposure and incidence rate of bone sarcoma (P = 0.008 for males, P = 0.006 for females). This association was independent of adult height or body weight. We conclude that the subset of Rottweiler dogs that undergo early gonadectomy represent a unique, highly accessible target population to further study the gene:environment interactions that determine bone sarcoma risk and to test whether interventions can inhibit the spontaneous development of bone sarcoma.

Animals↗
PubMed1995

Angiomatoid features in fibrohistiocytic sarcomas. Immunohistochemical, ultrastructural, and clinical distinction from vascular neoplasms.

The typical example of malignant fibrous histiocytoma (MFH) or dermatofibrosarcoma protruberans (DFSP) does not require ancillary studies for diagnosis. However, hemorrhage with cystic change consisting of blood-filled spaces may closely mimic a vascular neoplasm. Eight fibrohistiocytic sarcomas exhibiting these angiomatoid features, initially mistaken for vascular neoplasms, were identified from personal consultation files and review of 157 consecutive sarcomas (1985 through 1993) at the University of California-(Davis) Medical Center. They included five MFH giant-cell-type sarcomas, two MFH angiomatoid-type sarcomas, and one DFSP. Immunohistochemical analysis of paraffin-embedded material showed vimentin diffuse positive, CD68 (KP-1) diffuse positive, and factor VIII negative in all eight sarcomas; actin HHF-45 focal positive in six, diffuse positive in one, and negative in one sarcoma; desmin focal positive in two and negative in six sarcomas; and S100 protein, cytokeratin AE1:AE3, cytokeratin 10.11, and EMA negative in all eight sarcomas. Electron microscopy of three tumors exhibited neoplastic cells with fibroblastic, myofibroblastic, and histiocytic features. Weibel-Palade bodies or neolumens diagnostic of vascular differentiation were absent. The clinical characteristics and behavior of these sarcomas reflect entities in the spectrum of fibrohistiocytic lineage (MFH subtypes and DFSP) rather than vascular neoplasms. Patients with deep, large, giant-cell-type MFHs did poorly (two of four patients died from disease at 8 and 25 months). Both patients with angiomatoid MFHs showed local recurrences from large incompletely excised head and neck lesions. One died of disease at 21 months and the other is free of disease 12 months following excision of a local metastasis to the opposite side of the neck. The patient with DFSP had an 18-cm locally recurrent scalp tumor that extended into bone. Immunohistochemical and ultrastructural confirmation of fibroblastic, myofibroblastic, and histiocytic lineage and exclusion of vascular differentiation help to establish the correct diagnosis in these fibrohistiocytic sarcomas with angiomatoid features. The clinicopathologic features of these eight cases reaffirm the practical utility of MFH and DFSP as diagnostic entities in the spectrum of fibrohistiocytic sarcomas.

Adult↗
PubMed1988

The capacity of avian retrovirus-induced sarcomas to expand by infectious virus production.

Sarcomas arising in FP chickens infected with subgroup B avian sarcoma virus at increasing times posthatch were compared for their capacity to mediate recruitment of new sarcoma cells by infectious virus production. This comparison was premised on the observation that primary sarcoma growth is much slower when older chickens are infected with avian sarcoma virus. As measured in a transplantation assay, no age-related diminution in the recruitment capacity of sarcoma tissue from virus-infected transplant donors was observed, despite the marked age-related differences in their patterns of primary sarcoma growth. These observations establish that a diminished recruitment capacity is not required for slow primary sarcoma growth. The capacity to induce the formation of sarcomas at sites distal to the primary sarcoma was correlated with a strong recruitment capacity.

Animals↗
PubMedDate not supplied

Prognostic importance of vascular endothelial growth factor and its receptors in the uterine sarcoma.

Vascular endothelial growth factor (VEGF) and its receptors play an important role in tumor progression; however, there is no report regarding this factor in uterine sarcoma. Thirty-nine patients with uterine sarcoma, 14 carcinosarcomas, 4 endometrial stromal sarcomas, and 21 leiomyosarcomas, were studied. By immunohistochemical staining, VEGF was not detected in normal uterine smooth muscle, but VEGF receptor-1 (flt-1) and VEGF receptor-2 (flk-1) were observed in 14 and 4 of 14 normal smooth muscles, respectively. Of 39 sarcomas, 25 expressed VEGF, and 38 and 34 sarcomas expressed flt-1 and flk-1 at various intensities, respectively. The staining intensity of VEGF, flt-1, and flk-1 was significantly higher in sarcoma than in normal uterine smooth muscle, but that of phospho-flt-1 (p-flt-1) was significantly lower in sarcoma than in normal uterine smooth muscle. When sarcomas were divided into two groups according to staining intensity, a significant difference in survival curves was observed in only p-flt-1 of leiomyosarcoma (P= 0.008), and in all sarcomas, a lower survival curve was also observed in the high staining intensity group than in the low staining intensity group, although there was no significant difference (P= 0.102). In conclusion, VEGF and its receptors are suggested to be involved in progression of uterine sarcoma, but only the p-flt-1 level significantly affected the survival of leiomyosarcoma patients.

Adult↗
PubMed1978

Ultrastructural study of 28 cases of Ewing's sarcoma: typical and atypical forms.

The characteristics of 35 round cell sarcomas of bones are analyzed by optical and electron microscopical means, 28 cases of Ewing's sarcoma and 7 cases of malignant lymphomas (reticulum cell sarcoma) being distinguished. The existence of a morphological diversity within the Ewing's sarcoma group allows the authors to differentiate a conventional form of Ewing's sarcoma (21 cases) consisting of principal blastemic cells and secondary degenerative ones (dark cells) from an atypical variant (7 cases of atypical Ewin's sarcoma) whose structure adopts either an immature mesenchymal or histiocytic appearance. The ultrastructure of the atypical Ewing's cells demonstrates variability in size and in the nucleus, which is grooved and possesses prominent nucleoli. In the cytoplasm, in addition to the existence of a variable amount of glycogen, more highly differentiated structures appear when a comparison is made with the principal cells of the conventional Ewing's sarcoma. There exist filaments and mitochondria complexes, desmosomic-like junctions and profiles of endoplasmic reticulum. No transitional forms between the conventional and the atypical variants of Ewing's sarcoma have been observed. According to this analysis, when compared to other bone malignancies, the presence of glycogen as a means for identifying Ewing's sarcoma seems to be of restricted value. No clinical or anatomical differences could be established between either variant of Ewing's sarcoma.

Adolescent↗
PubMed1981

Growth pattern and differentiation of human soft tissue sarcomas in nude mice.

Tumor suspensions prepared from 62 human soft tissue sarcomas were injected into the peritoneal cavity and subcutis of 124 nude mice. The sarcomas included were ten malignant fibrous histiocytomas, 15 fibrosarcomas, six tendosynovial sarcomas, five liposarcomas, nine myosarcomas, six angiosarcomas, six malignant schwannomas, and five miscellaneous sarcomas. Sixty-two percent of the sarcomas showed progressive growth in nude mice either subcutaneously (46%), intraperitoneally (14%), or at both sites (40%). Fifty-one percent of the primary sarcomas, 71% of the recurrent tumors, and 78% of the sarcomas that were metastatic grew in the animals. Sarcomas that were grafted into the subcutis appeared as solitary nodules and showed a more accelerated growth as well as better differentiation than those that were injected into the peritoneal cavity. The intraperitoneal tumors were multifocal and dedifferentiated growths. It appears that simultaneous subcutaneous and intraperitoneal grafting of human sarcomas into nude mice offers a model to study the histogenesis and morphologic variations of soft tissue sarcomas.

Animals↗
PubMed1991

Prognostic value of histopathology in Ewing's sarcoma. Long-term follow-up of distal extremity primary tumors.

The pathologic material from 56 patients diagnosed initially as Ewing's sarcoma of the distal extremity and treated on National Cancer Institute protocols between 1968 and 1984 was reviewed and correlated with clinical outcome. Histologically, the tumors were categorized, based on recent pathologic criteria, into three diagnostic groups: (1) typical Ewing's sarcoma, (2) atypical Ewing's sarcoma, and (3) other (predominantly peripheral neuroepithelioma [PN]). Thirty-two patients (57%) had typical Ewing's, 13 (23%) were atypical, and 11 (20%) were in the "other" diagnostic category (seven [13%] PN, two primitive rhabdomyosarcoma, one primitive sarcoma of bone, and one synovial cell sarcoma). No cases of metastatic neuroblastoma, osteosarcoma, or lymphoma were found. Forty-five patients had localized disease at diagnosis; 11 had metastases. Patients with typical Ewing's sarcoma were less likely to have metastatic disease at the time of diagnosis. Only two of 32 patients with typical Ewing's sarcoma had metastases compared with nine of 24 patients in the two other groups. The pattern of relapse was also different in these other groups compared with typical Ewing's patients; five patients developed lymph node metastases and two patients developed brain metastases. Although the presence of metastatic disease at diagnosis was a strong negative prognostic factor, our histologic grouping was independently prognostic of clinical outcome in patients with localized disease. Patients with typical osseous Ewing's sarcoma had an improved overall survival (P2 = 0.03) and patients with other tumors (neither typical nor atypical Ewing's sarcoma) had a poorer disease-free survival (P2 = 0.02). Since no generally accepted histopathologic prognostic criteria exist for Ewing's sarcoma, the potential value of our proposed classification should be evaluated in a larger retrospective and a prospective study.

Adolescent↗
PubMed1991

Sarcomas of soft tissue and bone.

Sarcomas arise primarily from mesenchymal structures at any site in the body, even within visceral stroma and neurovascular bundles. Sarcomas have been associated with prior radiation therapy, toxic exposures, and genetic conditions and soft tissue sarcomas have been distinguished from bone sarcomas. For localized soft tissue sarcoma, tumor grade is the most important prognostic variable. Low-grade tumors are generally cured by wide surgical excision, but there is a significant rate of both local recurrence and development of distant metastasis in high-grade lesions. The treatment of soft tissue sarcoma histologic subtypes is generally similar grade-for-grade, with the exception of rhabomyosarcoma, Kaposi's sarcoma, and mesothelioma. Tumor location strongly influences resectability. Radiation therapy has been used successfully in conjunction with conservative surgery to improve local control rates for soft tissue sarcomas, particularly in extremity lesions. Currently, adjuvant chemotherapy remains unproven for most adult soft tissue sarcomas, but is established in the treatment of rhabdomyosarcomas, osteosarcomas, and Ewing's sarcomas.

Age Factors↗
PubMed2003

The influence of anatomic location on outcome in patients with soft tissue sarcoma of the extremity.

BACKGROUND: The anatomic location of an extremity soft tissue sarcoma may influence the patient's presentation, management, and local and systemic recurrence rates. The objective of this study was to compare the presentation, management, and outcome of patients with soft tissue sarcomas of the upper extremity and the lower extremity. METHODS: Prospectively collected data from patients who underwent limb-sparing surgery for extremity soft tissue sarcoma between January, 1986 and April, 1997 were analyzed. Local recurrence free rates and metastasis free rates were calculated using the method of Kaplan and Meier. Univariate and multivariate analyses of potential predictive factors were evaluated with the log-rank test and the Cox proportional hazards model. RESULTS: Of 480 eligible patients, 48 patients (10.0%) had a local recurrence, and 131 patients (27.3%) developed distant metastasis. The median follow-up of survivors was 4.8 years (range, 0.1-12.9 years). Patients with upper extremity tumors had smaller lesions (6.0 cm vs. 9.3 cm; P < 0.001), more often underwent unplanned excision before referral (89 patients [64.0%] vs. 160 patients [46.9%]; P < 0.001), and less often received radiotherapy (98 patients [70.5%] vs. 289 patients [84.8%]; P < 0.001). The 5-year local recurrence free rate was 82% for patients with sarcomas of the upper extremity and 93% for patients with sarcomas of the lower extremity (P = 0.002). The 5-year metastasis free rate was 82% for patients with sarcomas of the upper extremity and 69% for patients with sarcomas of the lower extremity (P = 0.013). CONCLUSIONS: Local recurrence was more frequent in patients who had sarcomas of the upper extremity compared with patients who had sarcomas of the lower extremity. Factors that contributed to this difference included histologic type, the use of radiotherapy, and local anatomy. Metastasis was more frequent among patients with sarcomas of the lower extremity, because those tumors tended to be large and deeper compared with upper extremity tumors.

Adolescent↗
PubMed2005

National survival trends of young adults with sarcoma: lack of progress is associated with lack of clinical trial participation.

BACKGROUND: Young adults with cancer in the U.S. have had less improvement in survival than either younger patients or older patients. The authors attempted to determine whether similar deficits have occurred in young adults with sarcomas and, if so, then why. METHODS: In 38,144 young adults with sarcoma who were diagnosed during 1975-1998 and were followed by the National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results Program, the average annual percent change in 5-year survival was derived as a function of patient age. National sarcoma treatment trial data were obtained on 3242 patients who were entered onto NCI-sponsored trials during 1997-2002. RESULTS: 1) For patients with bone and soft-tissue sarcomas, except Kaposi sarcoma (KS), the least survival improvement occurred between age 15 years and age 45 years. For patients with KS, the pattern was reversed, with the greatest survival increase occurring among patients ages 30-44 years. 2) The lowest participation rate in NCI-sponsored sarcoma treatment trials occurred in patients ages 20-44 years. For patients with KS, the highest accrual rate occurred in patients ages 35-44 years. 3) The age-dependent survival improvement and clinical-trial accrual patterns were correlated directly (soft-tissue sarcomas, P < 0.005; bone sarcomas, P < 0.05; KS, P = 0.06), regardless of whether the accrual profile demonstrated a decline or a peak (KS) during early adulthood. CONCLUSIONS: The lack of survival prolongation in patients age 15-44 years in the U.S. with non-KS sarcomas may have been a result of their relative lack of participation in clinical trials. If this is true, then reversing the shortfall in survival among young adults with sarcomas, as was accomplished among patients with KS, should benefit from increased clinical trial availability, access, and participation.

Adolescent↗
PubMed2005

Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.

BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period. METHODS: Fifty-two patients age 8-18 years (median 8 years) were observed (1979-2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible. RESULTS: The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns-NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor. CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.

Adolescent↗
PubMed1980

Changes in tumor-specific antigen expression during passage in vitro and in vivo of newly derived methylcholanthrene-induced sarcomas of BALB/C mice.

The characteristics of tumor antigens of the transplantation type, TATA, were studied in a series of methylcholanthrene-induced sarcomas in pedigreed BALB/cAN mice. Each of the six sarcomas exhibited unique TATA when assayed for tumor rejection in syngeneic hosts. In three instances, however, in sarcomas CI-I, CII-5 and CII-7, TATA activity was lost during early in vivo passages. This activity was reestablished invivo from the in vitro-passaged lines in CI-I and CII-7 and these sarcomas along with CI-3, CI-4 and CII-10 maintained a stable TATA phenotype throughout the 50 transplant passages; TATA was lost permanently, however, in CII-5. These results indicate a dimorphic nature of early-passage neoplasms. No cross-reacting antigens were detected among these sarcomas, including also Meth A, a sarcoma well characterized as to its membrane antigens; nor was any evidence obtained for the existence of alien (inappropriate) H-2 antigens employing tumor rejection assays in F1 hybrids of BALB/c with strains bearing several haplotypes and also with studies of H-2 serology. In three sarcomas, some evidence was obtained for the existence of alloantigens of the non-H-2 type since it was found that (BALB/c X DBA/2)F1 hybrids could not be immunized by respective sarcomas. These findings suggest the existence on these sarcomas of a tumor antigen that is expressed normally in DBA/2 mice, although no definitive evidence has been obtained. Assays for MuLV, for the viral structural proteins gp70 and p30 and for reverse transcriptase showed that three sarcomas were positive and three others, as well as Meth A, were negative. MuLV and its antigens had no influence on tumor rejection activity nor could the cross-reactivity observed in the radioisotopic footpad assay (IFP) be related to MuLV.

Animals↗