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PubMed2007

Adult human sarcomas. I. Basic science.

When connective tissue undergoes malignant transformation, glioblastomas and sarcomas arise. However, the ancient biochemical mechanisms, which are now operational in sarcomas distorted by mutations and gene fusions in misaligned chromosomes, were originally acquired by those cells that emerged during the Cambrian explosion. Preserved throughout evolution up to the genus Homo, these mechanisms dictate the apoptosis- and senescence-resistant immortality of malignant cells. A 'retroviral paradox' distinguishes human sarcomas from those of the animal world. In contrast to the retrovirally induced sarcomatous transformation of animal (avian, murine, feline and simian) cells, human sarcomas have so far failed to yield a causative retroviral isolate. However, the proto-oncogenes/oncogenes transduced from their host cells by retroviruses of animals are the same that are active in human sarcomas. Since the encoded oncoproteins arise after birth, they are recognized frequently by the immune system of the host. Immune lymphocytes that kill autologous sarcoma cells in vitro commonly fail to do so in vivo. Sarcoma vaccines generate immune T- and natural killer cell reactions; even when vaccinated patients do not show a clinical response, their tumors become more sensitive to chemotherapy. The aim of this review is to lay a solid molecular biological foundation for the conclusion that targeting the sarcoma oncogenes will result in regression of the disease.

B-Lymphocytes↗
PubMed1994

A comparison of the prognoses for deep and subcutaneous sarcomas of the extremities.

We evaluated 172 patients who had a soft-tissue sarcoma of the extremity in order to determine whether the prognosis for a subcutaneous sarcoma was better than that for a deep sarcoma. At a median of thirty-six months after the biopsy or definitive operation at our hospital, six of the fifty-two patients who had had a subcutaneous sarcoma had died and one had had a local recurrence; in contrast, forty of the 120 patients who had had a deep sarcoma had died and eight had had a local recurrence. Twenty-five (48 percent) of the subcutaneous sarcomas were malignant fibrous histiocytomas, and thirty-eight (73 percent) were small (five centimeters or less in the largest dimension). The three-year estimates of disease-free survival were 85 percent for the patients who had a subcutaneous sarcoma and 54 per cent for those who had a deep sarcoma (p = 0.002). Although the survival estimates remained significantly different when the groups were matched for histological diagnosis and for intracompartmental location (p = 0.0001 and 0.0006, respectively), they were not significantly different when the groups were matched for the size of the tumor (p = 0.42). A Cox proportional-hazards model confirmed that a tumor size of more than five centimeters and the histological grade are the most significant prognostic factors (p = 0.0007 and p = 0.004, respectively): a tumor size of more than five centimeters was associated with a relative risk of 3.5 (95 per cent confidence interval, 1.7 to 7.3), and a higher histological grade was associated with a relative risk of 4.0 (95 per cent confidence interval, 1.6 to 10.3). Subcutaneous location, when considered separately, was not a significant prognostic factor (p = 0.45). The data indicate that a tumor size of more than five centimeters is a more important prognostic indicator than histological diagnosis, depth, or intracompartmental location.

Adolescent↗
PubMed1996

Residual disease following unplanned excision of soft-tissue sarcoma of an extremity.

Sixty-five patients who had been referred to our unit for additional management after an unplanned excision of a soft-tissue sarcoma of an extremity at another institution were studied retrospectively to determine the prevalence of residual tumor and to identify factors that predict which patients will have a tumor following such an excision. Unplanned excision was defined as excisional biopsy or unplanned resection of the lesion without benefit of preoperative imaging and without regard for the necessity to resect the lesion with a margin of normal tissue. In each patient, histological evaluation of the specimen removed at the unplanned excision had demonstrated positive resection margins, but postoperative physical examination on our unit revealed no gross evidence of residual tumor and no tumor was identified on cross-sectional imaging of the local site. Patients who had evidence of residual disease on physical examination or on imaging were thought to have definite evidence of sarcoma at the site of the operative wound and were therefore excluded from the study. After multidisciplinary consultation, all patients had a repeat resection at our cancer center. Extensive pathological sampling of the specimen from this second procedure was carried out, with sections obtained at mean intervals of 1.2 +/- 0.7 centimeters. Nodules initially thought to indicate disease were identified grossly in twenty-seven (42 percent) of the sixty-five patients, but histological evaluation confirmed the presence of tumor in only sixteen (59 percent). Histological evidence of sarcoma was identified in seven additional patients in whom gross nodules were not apparent in the specimen. Thus, sarcoma was identified in a total of twenty-three (35 percent) of the sixty-five patients. The mean duration of follow-up was forty-six months (range, twenty-four to eighty months; median, thirty-nine months). The margins of the second resection were positive in nine (39 percent) of the twenty-three patients who had residual sarcoma. Five (22 percent) of the twenty-three had a local recurrence. Four of the five patients who had a local recurrence had positive margins on repeat resection. This rate of local recurrence (five of twenty-three patients) was significantly higher than that in the remainder of our patients who had a soft-tissue sarcoma of an extremity (sixteen [7 percent] of 227) (p = 0.03). There was no association between the detection of sarcoma at the second procedure and the initial size or grade of the tumor, the use of irradiation preoperatively, or the interval between the initial, unplanned excision and referral to our cancer center. These data indicate that it is not possible to predict which patients will have residual tumor at the site of the operative wound. Therefore, it is prudent to advise repeat excision for all patients who have had an unplanned excision of a soft-tissue sarcoma of an extremity. Unplanned excision complicates decision-making in the treatment of this disease and should be avoided.

Biopsy↗
PubMed2005

Recent developments in salvage chemotherapy for patients with metastatic soft tissue sarcoma.

The number of effective cytotoxic agents for the treatment of patients with metastatic adult soft tissue sarcoma is limited, especially when patients have failed anthracycline- and ifosfamide-based chemotherapy. For the subgroup of patients with inoperable gastrointestinal stromal tumour (GIST), progress has been made via the rapid development and approval of the targeted therapy imatinib. Small round cell tumours (SRCTs), such as Ewing's sarcoma/primitive neuroectodermal tumour, desmoplastic SRCT and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensive, neoadjuvant protocols regardless of size or overt metastatic disease. Most other high-grade (grading >I), so-called 'adult type', soft tissue sarcomas such as fibrosarcoma, liposarcoma, pleomorphic and synovial sarcomas are treated with an anthracycline-based regimen with or without ifosfamide as front-line therapy. In relapsed 'adult type' soft tissue sarcomas, trofosfamide, gemcitabine and trabectedin (ecteinascidin 743) appear to be drugs associated with some activity and an acceptable toxicity profile. A high activity has been reported for the taxanes, in particular for paclitaxel, in vascular sarcomas located in the scalp or face and in Kaposi's sarcoma. It is interesting to note that the different drugs have particular effects in distinct subtypes of soft tissue sarcoma; however, it should be taken into account that the number of patients included in the phase II trials is limited. The role of the newer agents (e.g. epothilones, brostallicin) is currently undefinable. Targeted therapy inhibiting vascular endothelial growth factor receptor, epidermal growth factor receptor, RAF kinase, c-KIT or platelet-derived growth factor receptors will continue to be tested in GIST patients refractory to imatinib and in other sarcoma histologies.

Animals↗
PubMed2002

Detection and induction of CTLs specific for SYT-SSX-derived peptides in HLA-A24(+) patients with synovial sarcoma.

To investigate the immunogenic property of peptides derived from the synovial sarcoma-specific SYT-SSX fusion gene, we synthesized four peptides according to the binding motif for HLA-A24. The peptides, SS391 (PYGYDQIMPK) and SS393 (GYDQIMPKK), were derived from the breakpoint of SYT-SSX, and SS449a (AWTHRLRER) and SS449b (AWTHRLRERK) were from the SSX region. These peptides were tested for their reactivity with CTL precursors (CTLps) in 16 synovial sarcoma patients using HLA-A24/SYT-SSX peptide tetramers and also for induction of specific CTLs from four HLA-A24(+) synovial sarcoma patients. Tetramer analysis indicated that the increased CTLp frequency to the SYT-SSX was associated with pulmonary metastasis in synovial sarcoma patients (p < 0.03). CTLs were induced from PBLs of two synovial sarcoma patients using the peptide mixture of SS391 and SS393, which lysed HLA-A24(+) synovial sarcoma cells expressing SYT-SSX as well as the peptide-pulsed target cells in an HLA class I-restricted manner. These findings suggest that aberrantly expressed SYT-SSX gene products have primed SYT-SSX-specific CTLps in vivo and increased their frequency in synovial sarcoma patients. The identification of SYT-SSX peptides may offer an opportunity to design peptide-based immunotherapeutic approaches for HLA-A24(+) patients with synovial sarcoma.

Adolescent↗
PubMed1999

Different patterns of angiogenesis in sarcomas and carcinomas.

Solid tumors depend on angiogenesis for growth and metastasis. It has been shown that blood vessel density, as determined by counting the number of capillaries in clustered bursts, is a significant prognostic factor in carcinomas. It is unclear, however, whether vessel density is a prognostic factor in sarcomas. In this study, we examined angiogenesis in sarcomas of various grades and compared their vascular patterns to those of carcinomas. Microvessels were identified by von Willebrand factor staining. The matrix of multiple sarcoma and breast carcinoma specimens were extracted and subjected to Western analysis of various angiogenic factors and inhibitors. Metalloproteinase inhibitor presence was also determined by in situ hybridization. In breast carcinomas, capillaries were clustered in bursts within the stroma of the tumor, whereas the sarcoma capillaries were homogeneously distributed in the tumor stroma. Random blood vessel density per high power field in sarcomas did not correlate with patient prognosis. The matrix of sarcomas and carcinomas contained both angiogenic stimulators and inhibitors. Tissue inhibitor of metalloproteinase-1 was found predominantly in fibroblasts and myofibroblasts in the matrix of carcinoma specimens. The difference in the pattern of angiogenesis in sarcomas and carcinomas may be attributable to the presence of fibroblasts and myofibroblasts in carcinomas, resulting in the compartmentalization of bursts of angiogenic factors. The homogeneous appearance of vessel density in sarcomas observed in the present study would be the consequence of the influence of a single compartment.

Angiogenesis Inhibitors↗
PubMed2000

Induction of cell death by basic fibroblast growth factor in Ewing's sarcoma.

Ewing's sarcoma is thought to arise after developmental arrest of primitive neural cells during embryogenesis. Because basic fibroblast growth factor (bFGF) has a critical role in the regulation of cell survival, proliferation, and differentiation during embryogenesis, we have tested the hypothesis that bFGF and FGF receptors may contribute to the development of Ewing's sarcoma and may provide a mechanism for the modulation of their behavior. All four of the Ewing's sarcoma cell lines examined expressed bFGF and FGF receptors, which were detected by immunofluorescence and Western blotting. bFGF-induced a significant dose-dependent decrease in Ewing's sarcoma cell proliferation on plastic and reduced anchorage-independent growth in soft agar. Unexpectedly, this decrease in cell number reflected bFGF-induced apoptosis and necrosis, as demonstrated by electron microscopy, binding of annexin V, and staining with acridine orange. Induction of cell death was dependent on dosage of, and period of exposure to, bFGF. bFGF did not induce differentiation of Ewing's sarcoma cells in either the presence or the absence of serum or nerve growth factor. Treatment of NuNu mice with bFGF decreased growth of the highly tumorigenic Ewing's sarcoma cell lines. Histologically tumors grown in the NuNu mice treated with bFGF were less cellular than those in control mice, and showed an increased level of apoptotic nuclei. This is in contrast to the mitogenic effect bFGF has in most other cancer cells. In summary, bFGF decreases Ewing's sarcoma growth in vitro and in vivo by the induction of cell death. This novel observation may provide a new therapeutic strategy for Ewing's sarcomas.

Animals↗
PubMed2001

[Radiotherapy in the treatment of uterine sarcomas].

OBJECTIVE: To study radiotherapy in the treatment of uterine sarcomas and response to radiation in primary or recurrent uterine sarcomas. METHODS: A total of 102 uterine sarcomas treated in the Cancer Hospital from May 1960 to June 1999. A retrospective review of 45 patients receiving surgery alone, 29 patients for postoperative radiotherapy, 4 radiotherapy alone, 24 recurrent sarcomas receiving radiotherapy. RESULTS: The 5-year overall survival for 45 patients treated with surgery alone and 29 patients managed by postoperative radiotherapy were 56.7% and 58.6% respectively (P > 0.05). The addition of radiotherapy significantly reduced the local recurrence rate from 42.2% to 20.7% (P < 0.05). The tumor was disappeared completely by treatment of radiotherapy alone in 3 patients of primary uterine cervix sarcomas. 1 patient was free of disease at 36 months. Other 2 patients died 9 and 11 months later because of distant metastases. The complete and partial response rate of radiotherapy was 50.0% for recurrent tumors. The radiotherapeutic sensibility of endometrial stromal sarcomas, mixed mesodermal sarcomas and leiomyosarcomas was downward tendency in turn. CONCLUSIONS: The postoperation adjuvant radiotherapy reduced the pelvic recurrent significantly. Radiotherapy should be used in the treatment of uterine sarcomas as an important part of combination therapy.

Female↗
PubMed1976

Sarcomas of the vagina.

The clinical and patholgic features of 16 cases of vaginal sarcoma are assessed. There were 5 embryonal rhabdomyosarcomas, 5 leiomyosarcomas, 2 reticulum cell sarcomas, 1 malignant schwannoma, 1 müllerian stromal sarcoma, and 2 pleomorphic sarcomas that could not be classified as to cell type. The rhabdomyosarcoma occurred exclusively in infants and children, and all 5 patients died of their disease with locally infiltrative recurrences and/or disseminated metastases. The commonest sarcoma in adults was leiomyosarcoma, there being 5 examples in the series. Three of the 4 patients with survival information were clinically free of disease 2 years, 4 years, and 8 years after diagnosis and treatment. Two patients with reticulum cell sarcoma succumbed to their disease within a few months. Local recurrences were common for all types of sarcoma in this series, suggesting that more aggressive initial treatment is mandatory if recurrences are to be prevented and the survival rates improved. In general, the vaginal sarcomas in adults pursued a less aggressive course than the rhabdomyosarcomas of infancy and childhood.

Adult↗
PubMed2004

Synovial sarcoma: the importance of size and location for survival.

A retrospective analysis was done on 135 consecutive patients with extremity and truncal synovial sarcomas seen at our institutions between 1961 and 1996. Ninety-nine patients (73%) presented with a newly diagnosed localized primary sarcoma, nine patients (7%) presented with a locally recurrent sarcoma previously treated at another institution, and 27 patients (20%) presented with metastatic disease. The median followup for surviving patients was 78 months (range, 20-420 months). The overall survival for patients with localized primary and locally recurrent synovial sarcoma was 51% at 10 years. Patients with localized synovial sarcomas less than 5 cm in longest diameter had a survival at 10 years of 88% compared with a 10-year survival of 38% and 8% for patients with sarcomas 5 to 10 cm and greater than 10 cm in longest diameter, respectively. Distal location (hands and feet) and histologic grade also were statistically significant predictors of patient survival for localized synovial sarcoma on univariate analysis. On multivariate analysis size greater than 10 cm and size 5 to 10 cm were associated with an 18-fold and a 3.1-fold increased risk of death compared with tumors smaller than 5 cm. Grade and location were not found to be independent predictors of survival on multivariate analysis. Primary tumor size and initial status at presentation were prognostic for survival and patients with synovial sarcomas larger than 5 cm or locally recurrent at presentation should be considered for more aggressive surgical and adjunctive radiotherapy or chemotherapy.

Adolescent↗
PubMed1992

A possible role for interferon-alpha and activated natural killer cells in remission of AIDS-related Kaposi's sarcoma: in vitro studies.

The effect of consensus interferon-alpha on the growth of AIDS-related Kaposi's sarcoma-derived cells was studied. Interferon caused a low but significant in vitro inhibition of cell growth. Whereas Kaposi's sarcoma cells were resistant to the cytotoxic effect of natural killer (NK) cells, treatment of NK cells with either interferon or interleukin-2 activated the cell-mediated cytotoxic response. Pretreatment of the Kaposi's sarcoma cells with interferon reduced their sensitivity to interferon-primed natural killer cell or lymphokine (interleukin-2)-activated killer (LAK) cell cytotoxicity. The resistance of Kaposi's sarcoma cells to NK cell-mediated cytotoxicity did not reside in conjugate formation but was due to an inability of Kaposi's sarcoma cells to induce NK cytotoxic factor. Although interferon reduces the sensitivity of Kaposi's sarcoma cells to interferon-primed NK cell and LAK cell activities, the level of natural killing susceptibility remained significantly higher than the poor sensitivity of Kaposi's sarcoma cells to unprimed NK cell activity. Thus, the potential antitumor effect of interferon against Kaposi's sarcoma cells could be mediated both directly (antiproliferative) and/or indirectly by activation of NK cells.

Acquired Immunodeficiency Syndrome↗
PubMed2005

[Prostate sarcoma: a report of 14 cases].

OBJECTIVE: To discuss the diagnosis and effective treatment of prostate sarcoma. METHODS: We analysed the clinical materials of fourteen patients with prostate sarcoma treated in our hospital from Jan. 1991 to Jun. 2004. RESULTS: Prostate sarcoma accounted for 3.21% of all the prostatic malignant tumors treated in our hospital during that period. The average age was 39.5 years old (ranging from 17 to 62). Twelve cases came to our hospital because of dysuria. Large soft prostate tumors were found in all the patients in physical examination. The serum AKP, ACP and PSA levels were normal in all the 10 patients who had received the test. Pathological test revealed: 7 cases leiomyosarcoma, 1 case rhabdomyosarcoma, 4 cases fibrosarcoma, 2 cases spindle cell sarcoma. Different kinds of prostate sarcoma each his its own immuno-histochemical staining features. Two cases were at Ghavimi Stage I, 5 at Stage II, 3 at Stage III, and 4 at Stage IV. All the patients received surgery, chemotherapy and/or radiotherapy. One case failed to be followed up, 11 died 2 approximately 12 months after diagnosis. Two patients are still alive, 1 for 18 months without recurrence, and the other for through with relapse. CONCLUSION: Dysuria is always the first symptom of prostate sarcoma. DRE test may suggest prostate sarcoma but needle biopsy contributes to a definite diagnosis. The immuno-histochemical dyeing helps to differentiate the disease. The prognosis of prostate sarcoma is very poor and the main treatment is surgery followed by chemotherapy and/or radiotherapy.

Adolescent↗
PubMed2006

[Clinicopathologic and immunophenotypic analysis of myeloid sarcoma].

OBJECTIVE: To study the clinicopathologic features of myeloid sarcoma and to evaluate the role of immunohistochemical study in diagnosis of this entity. METHODS: Eighty-two cases of myeloid sarcoma were retrieved from the archives of Department of Pathology, West China Hospital of Sichuan University during the period from January, 1990 to February, 2005. The morphologic features were reviewed and classified according to the 2001 WHO classification for hematopoietic and lymphoid tissue tumors. Immunohistochemical study using a panel of 11 antibodies was performed on 73 cases. The survival data were collected and analyzed by SPSS 10.0. RESULTS: The median age of patients was 35.5 years. The male-to-female ratio was 1.4:1. The sites of occurrence included lymph node (43.1%), skin (16.7%), nose (7.8%), soft tissue (7.8%) and bone (6.9%). Fifty-one cases (62.2%) represented myeloid sarcoma associated with an underlying myeloproliferative disorder and 25 cases (30.5%) represented solitary myeloid sarcoma. As for the morphology, 79 cases (96.3%) were granulocytic sarcoma, including 41 cases (51.9%) blastic type, 25 cases (31.6%) immature type and 13 cases (16.5%) differentiated type. The other 3 cases (3.7%) were monoblastic sarcoma. Immature eosinophils were found in 51 cases (64.6%) of granulocytic sarcoma, among which 13 cases (31.7%) were of blastic type. Immunohistochemical study showed that 95.9% cases (70/73) were positive for myeloperoxidase, 95.5% (63/66) for lysozyme, 95.2% (60/63) for CD68 (KP1), 90.8% (59/65) for leukocyte common antigen, 85.7% (54/63) for CD43, 77.8% (49/63) for CD117, 58.7% (37/63) for CD99, 54.0% (34/63) for CD15, 22.2% (14/63) for CD34, and 4.7% (3/64) for CD68 (PG-M1). Proliferation index, as demonstrated by Ki-67 positivity, was 0.49+/-0.22. Follow-up data was obtained in 59 of the 82 patients. The two- and five-year survival rates were 36.1% and 17.3% respectively. No significant prognostic factors were found in the survival analysis. CONCLUSIONS: Myeloid sarcoma may precede, develop in a background of myeloproliferative disorder or even after remission of the disease. The presence of immature eosinophils is an important morphologic clue and immunohistochemical study plays an essential role in arriving at a correct diagnosis. Immunopositivity for myeloperoxidase is specific for granulocytic differentiation, while CD68 (PG-M1)-positivity suggests monocytic differentiation. Detailed clinicopathologic correlation is also helpful.

12E7 Antigen↗
PubMed1991

Soft tissue sarcomas of the extremity and retroperitoneum: advances in management.

The treatment of soft tissue sarcomas continues to pose significant challenges. The past decade has seen a dramatic increase in multimodality therapy for these tumors, with a resultant decrease in the number of amputations performed for extremity lesions. Even with combined modality treatment, however, the prognosis remains grim for most patients with retroperitoneal sarcomas, in which delay in diagnosis and tumor proximity to vital structures hinder the chances for cure. Improved protocols for the treatment of retroperitoneal sarcomas must be developed if we are to match the successes achieved with extremity tumors. As efforts to decrease local recurrence of extremity sarcomas have been largely successful, a new challenge to decrease the frequency of systemic metastases has emerged. Although aggressive surgical approaches to recurrent and metastatic sarcomas can result in long-term survival in some cases, better treatments are needed for those patients whose resections fail or those who are not candidates for resection. Combined modality treatments and new modalities, such as adoptive immunotherapy, need to be carefully evaluated in this group of patients. Enormous strides have been made in the treatment of osteogenic sarcomas and some pediatric soft tissue sarcomas. Additional research into basic tumor biology, as well as major intergroup studies of therapy, will be necessary if we are to duplicate those achievements in the more refractory adult soft tissue sarcomas.

Combined Modality Therapy↗
PubMed1990

Treatment of patients with sarcoma of the uterus.

In the present series of 156 patients with sarcomas of the uterus the therapeutic modalities employed were evaluated. The 5-year survival rate of 51.3% was obtained for this series including 41.2% for leiomyosarcomas, 57.1% for endometrial stromal sarcomas, 50.0% for mixed mesodermal tumors and 40.0% for carcinosarcomas. The prognostic significance of the extent of disease, histological type and the age of patients is emphasised. Clinical features of uterine sarcomas specific for different histological types were shown: leiomyosarcomas were characterized by a rapid tumor growth while uterine bleeding in the background of neuroendocrine disturbances similar to those observed in endometrial carcinomas were related to endometrial stromal sarcomas and mixed mesodermal tumors. In respect to early diagnosis of sarcomas an ultrasonic pelvic examination and studies of aspirate specimens taken from the uterine cavity are recommended to be performed for the groups at high risk. An individual treatment policy was developed for each sarcoma patient depending on the histological type, the stage of disease and general condition of the patient. Surgical approach varied ranging from a total hysterectomy and adnexectomy, done for leiomyosarcomas, to an extended hysterectomy and pelvic lymphadenectomy for mixed mesodermal tumors and endometrial stromal sarcomas. Postoperative distant and/or endovaginal radiation are indicated for all histological types of sarcomas excepting leiomyosarcomas. Application of adjuvant chemotherapy with carminomycin or adriamycin improves the results of treatment.

Carcinosarcoma↗
PubMed1990

The association of selected cancers with service in the US military in Vietnam. II. Soft-tissue and other sarcomas. The Selected Cancers Cooperative Study Group.

As part of a series of investigations into the health of Vietnam veterans, we conducted a population-based, case-control study of soft-tissue and other sarcomas between 1984 and 1988. All men born between 1929 and 1953 and diagnosed in an area covered by eight cancer registries were considered eligible. Controls were selected by random-digit dialing. Analyses of 342 men with pathologically confirmed sarcoma and 1776 controls showed that Vietnam veterans had a relative risk of 1.0 for sarcoma in comparison with men who did not serve in Vietnam (95% confidence interval, 0.6 to 1.6). Restriction of the analysis to the 254 men with soft-tissue sarcoma yielded a relative risk of 0.9 (95% confidence interval, 0.5 to 1.6). Several attributes of military service in Vietnam (eg, branch, duration of service, military region, and other characteristics that may have been associated with the use of Agent Orange) were examined, and none was associated with an increased risk for the development of sarcoma. Furthermore, no morphologic type of sarcoma was overrepresented among Vietnam veterans. Results were unchanged if Vietnam veterans were compared with (1) other veterans or (2) men who never served in the military. This study, which had 97% power to detect a relative risk of 2.0 for all sarcomas, provides no evidence that the risk for the development of soft-tissue or other sarcomas is increased among veterans 15 to 25 years following service in Vietnam.

Adult↗
PubMed1989

Clear cell sarcoma. An immunohistochemical analysis of six cases and comparison with other epithelioid neoplasms of soft tissue.

Clear cell sarcoma (CCS) is an uncommon, clinicopathologically distinct neoplasm that typically arises in association with tendons and aponeuroses. It shares several histologic and ultrastructural features with malignant melanoma. Clear cell sarcoma occasionally may be confused with other tumors of soft tissue that have a predominantly epithelioid appearance, including epithelioid leiomyosarcoma, epithelioid neurofibrosarcoma, synovial sarcoma, and epithelioid sarcoma. To assess the potential contribution of immunohistochemistry to this differential diagnosis, a panel of immunostains was applied to examples of each of these neoplasms. All six CCSs contained vimentin, and five were reactive with the melanoma-specific monoclonal antibody HMB-45. In addition, five CCSs expressed neuron-specific enolase, four cases displayed S100 protein, and four examples contained LN3 antigen. Synaptophysin and Leu-7 antigen were present in one case each. Cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, and leukocyte common antigen were invariably absent. No other primary epithelioid neoplasm of soft tissue reacted with HMB-45. Clear cell sarcoma could be separated from epithelioid leiomyosarcoma by the presence of desmin and muscle-specific actin in the latter neoplasm. Similarly, both synovial sarcoma and epithelioid sarcoma differed from CCS by their expression of cytokeratin and epithelial membrane antigen. Clear cell sarcoma and malignant melanoma were immunohistochemically indistinguishable, supporting the concept that they share a common pattern of differentiation.

Adolescent↗
PubMed1984

Synovial sarcoma--a misnomer.

For an evaluation of the putative histogenetic relationship of synovia and synovial sarcomas, normal synovia, villonodular synovitis, and synovial sarcomas were compared for their patterns of expression of intermediate filaments of keratin and vimentin type and epithelial membrane antigen and for lectin binding sites. The lining cells in both normal synovia and villonodular synovitis reacted with anti-vimentin antibodies, but not with antibodies to different types of keratins or epithelial membrane antigen. The cleft-lining cells in synovial sarcoma, on the other hand, showed only keratin positivity, and epithelial membrane antigen could also be detected in these cells. Nonneoplastic synovial lining cells bound peanut agglutinin (PNA), Ricinus communis agglutinin (RCA), soybean agglutinin (SBA), concanavalin A (Con A), and wheat germ agglutinin (WGA) conjugates, but not Ulex europaeus I lectin (UEA I). In contrast, the epithelial-like cleft lining cells in synovial sarcomas showed an apical cytoplasmic binding of PNA, UEA I, RCA, and SBA, and binding of WGA to the whole cytoplasm but did not bind Con A. The distinct differences between synovial lining cells and synovial sarcoma cells speak against synovial cell features in synovial sarcoma. These results indicate that synovial sarcoma is a carcinosarcomalike tumor with true epithelial differentiation, and the term "synovial sarcoma" apparently is a misnomer that should be abandoned.

Antibodies↗