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PubMedDate not supplied

[Synovial sarcoma of the head and neck: a case report of parapharyngeal region and review of the literature].

Synovial sarcoma is a clinically and morphologically well defined entity that has been described extensively in Literature. It occurs primarily in the para-articular regions, usually in close association with tendon sheaths, bursae and joint capsules. On rare occasions it is also encountered in areas without any apparent relationship to synovial structures, as in the parapharyngeal region or the abdominal wall. It is considered the fourth most common type of sarcoma (7-10%) after malignant fibrous histiocytoma, liposarcoma and rhabdomyosarcoma. There are three histological variants: the classical biphasic, the monophasic fibrous type and the monophasic epithelial type (the biphasic and monophasic fibrous type are equally common). Clinical sign complaints are subtle and at times noted 20 years before diagnosis. The course of the disease is slow and insidious. The most typical presentation is that of a palpable deep-seated swelling or mass associated with pain or tenderness. Patients with synovial sarcoma in the head and neck (10%) tend to have difficulties in swallowing and breathing and not infrequently have alteration or loss of voice. Head and neck synovial sarcoma seem to originate from the paravertebral connective tissue spaces and manifest themselves as solitary retropharyngeal or parapharyngeal masses near the forking of the carotid. Additional cases in this general area have been reported in the soft palate, tongue, maxillofacial region, mandible corner, sternoclavicular region, scapular region and the cervical oesophagus. As in other types of sarcoma, the principal sites of metastases are the lung, but many make their appearance many years after the initial diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1999

H-ras and K-ras gene mutations in primary human soft tissue sarcoma: concomitant mutations of the ras genes.

ras gene mutations have been described with varying frequency in several types of human malignancies. To determine the incidence and type of ras mutations in human soft tissue tumors, we studied 45 sarcomas, including 27 malignant fibrous histiocytomas (MFHs), 10 liposarcomas, 2 rhabdomyosarcomas, and 6 leiomyosarcomas. Al of the tumors were investigated by direct sequence analysis with the automated DNA sequencing of polymerase chain reaction-amplified ras sequences. Twenty (44%) of the sarcomas examined harbored K-ras mutations, 18 (90%) of which were MFHs. All of the K-ras mutations were G-to-A transition mutations in the second position of codon 13 (glycine --> aspartic acid). Of the samples with K-ras activation, 7 (16% of the total of 45 tumors), including 6 MFHs and 1 leiomyosarcoma, also contained H-ras mutation. All of the tumors that showed H-ras alteration had G-to-T transversion mutations in the second base of codon 12 (glycine --> valine). These data possibly implicate that ras gene activation may be a relatively uncommon event in soft tissue tumors, with the exception of MFH. It is suggested that the oncogenic process underlying the development of tumors between these groups may be different and that ras gene mutations may play a role in the etiology and/or progression of MFH. It is noteworthy that when ras gene activation occurs in sarcoma, it predominantly affects the K-ras gene, particularly codon 13. Moreover, H-ras mutations in our samples were detected only in association with tumors that also displayed K-ras-mutated genes. This study demonstrates for the first time concomitant mutations in two different members of the ras gene family in sarcoma

Adolescent↗
PubMed1999

A comparative study of P53/MDM2 genes alterations and P53/MDM2 proteins immunoreactivity in soft-tissue sarcomas.

In the present study, the expression of P53 and MDM2 proteins were examined in 94 soft-tissue sarcomas (35 malignant fibrohistiocytomas, 15 neurosarcomas, 14 liposarcomas, 13 leiomyosarcomas, 11 fibrosarcomas and 6 dermatofibrosarcomas) by immunohistochemistry. The immunohistochemical findings were correlated with P53 mutation analysis using PCR-SSCP, PCR-HDF and direct sequencing, and MDM2 amplification studies by differential PCR. P53 immunopositivity was found in 25 out of 94 (26.6%) cases. Alterations of the P53 gene were detected in 12 (12.8%) tumors; eight of these tumors revealed P53 immunoreactivity. A high number of P53 positive and P53 mutated tumors were histologically defined as poorly differentiated G3 (64.0% and 75.0%, respectively). MDM2 immunopositivity was revealed in 36 out of 94 (38.3%) cases. MDM2 amplification occurred in 17 tumors (18.1%); only nine of these tumors exhibited MDM2 immunoreactivity. Overall, MDM2 positivity was not associated with MDM2 amplification in 27 out of 94 tumors (28.7%). There was no significant correlation between MDM2 overexpression and histological grade. However, when the samples were stratified by immunophenotype, the majority of tumors (52.5%) with isolated MDM2 overexpression (dissociated from P53 positivity) were defined histologically as low grade (G1 + G2). These results support the notion that besides P53 alterations, MDM2 gene deregulation seems to be an important event in sarcomas evolution. Additionally, the mechanism of MDM2-mediated degradation of P53 protein, without involving stabilization and inactivation of P53 gene, should be considered for better understanding of all features of tumor progression processes.

Adult↗
PubMed2005

Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana.

BACKGROUND AND OBJECTIVES: Retroperitoneal sarcomas are rare malignant tumors with an aggressive course of disease and high local recurrence rate. Local disease is the main cause of death. A retrospective study was undertaken to evaluate the results of aggressive surgical treatment in a series of patients of primary and recurrent retroperitoneal sarcomas. PATIENTS AND METHODS: From 1975 to 2000, 155 patients with retroperitoneal tumors were operated on at the Institute of Oncology, Ljubljana. Patients with fibromatosis and children less than 16 years were excluded from further calculation. Out of the rest 139 patients, 102 patients had localized retroperitoneal sarcoma, and 37 patients had retroperitoneal sarcoma with distant metastases Leiomyosarcoma was the most common type of histology, followed by liposarcoma and schwannoma. Their referral status was as follow: 56 had primary sarcoma, 20 residual sarcoma after operation elsewhere and 26 already recurrent sarcoma. Our treatment approach was aggressive. We removed surgically primary tumor, recurrent sarcoma and metastases, whenever possible. To this end, we performed, 235 operations for retroperitoneal sarcomas on 139 patients (up to 8 operations on 1 patient). RESULTS: Complete resection was done in 97 of 102 patients with localized sarcoma (resectability rate 95%); in 55 patients, R0 resection was made, in 42 patients, R1 resection, in 3 patients, debulking procedure, and in 2 patients biopsy alone. In 118, patients at least one organ was resected completely or partially en bloc with tumor. The 5-, 10-, and 15-year survivals of patients with localized sarcoma were 52%, 36%, and 22%, respectively. The 5-year survival of patients with metastases was 12% (P = 0.0002), the 5- and 10-year survival rates of patients with R0 resection were 75% and 64%, respectively, and of those with R1 resection, 25% and 8%, respectively (0 < 0.00001). High tumor grade was associated with poor survival. The 5-year local recurrence rate of patients with primary sarcoma and of those with locally recurrent sarcoma was 37% and 71%, respectively (P = 0.04). After the first local recurrence, the 5- and 10-year survival rates were 42% and 26%, after the second local recurrence 45% and 22%, after the third local recurrence 43% and 11%, and after the fourth local recurrence 51% and 17%, respectively. CONCLUSION: Complete surgical resection without microscopic residuum and contamination is likely to offer the best chances for long-term survival. Until there are no other treatment modalities, aggressive surgery for recurrent sarcoma is recommended.

Adolescent↗
PubMedDate not supplied

Isolation and analysis of lectin-reactive sarcoma-associated membrane glycoproteins.

Sarcoma and normal tissue plasma membrane lectin-reactive glycoproteins were analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. Two peanut agglutinin-reactive N-acetylgalactosamine-containing glycoproteins of 1.05 x 10(6) and 1.25 x 10(5) Da and one lentil agglutinin-reactive mannose/N-acetylglucosamine(-fucose)/sialic acid-containing glycoprotein of 1.7 x 10(5) Da (Gp170) were detected in osteosarcoma and malignant fibrous histiocytoma (MFH), respectively. However, these glycoproteins were not detected in normal tissue plasma membranes. Concanavalin A, wheat germ and Ulex europaeus Type I agglutinins did not reveal any unique sarcoma-associated membrane glycoproteins. Preliminary studies on monoclonal antibodies (mAbs) generated against Gp170 (mAb 64-35-84) and against lentil-reactive glycoproteins from MFH (mAbs 67-34 and 67-117) revealed high specific binding to a number of membranes isolated from MFH and osteosarcoma tissues, with no crossreactivity to normal human tissues tested (liver, spleen and skin). Detailed analysis of mAb 67-102, which was generated against lentil-reactive glycoproteins isolated from MFH plasma membranes, exhibited significant binding to membranes isolated from osteosarcoma, liposarcoma and MFH; moderate binding to synovial sarcoma, aggressive fibromatosis and fibrosarcoma; and minimal to no binding to other soft tissue sarcoma plasma membranes. No binding was observed to twenty normal tissue specimens, with the exception of low positive binding to two of five fat and two of three colon specimens.

Adult↗
PubMed1994

Myogenic regulatory protein expression in adult soft tissue sarcomas. A sensitive and specific marker of skeletal muscle differentiation.

Myogenic regulatory protein (MyoD1) is a DNA-binding nuclear protein that initiates myogenesis in mesenchymal stem cells. Its expression has proved a very sensitive marker of myogenic differentiation in malignant tumors of childhood. In this study, the reliability of MyoD1 expression as marker of skeletal muscle differentiation has been tested in 38 cases of adult-type sarcomas. Frozen sections were stained with the monoclonal antibody 5.8A (MAb 5.8A) developed against recombinant wild-type murine MyoD1. Routinely processed formalin-fixed sections from the same cases were reviewed and immunostained with a panel of antibodies commonly used to detect muscle differentiation: myoglobin, fast-myosin, desmin, muscle-specific actin, alpha-smooth muscle actin. Four cases positively stained with MAb 5.8A: three were high-grade spindle cell sarcomas, the fourth case was an alveolar soft-part sarcoma. Only tumors in which skeletal muscle histogenesis was inferred on the basis of histology and/or immunohistochemistry with conventional muscle markers stained positively for MAb 5.8A. All the other tumors tested, including leiomyosarcomas (11), liposarcomas, (10, including four dedifferentiated liposarcomas), fibrosarcomas (three), malignant fibrous histiocytomas (two), synovial sarcomas (two), and malignant peripheral nerve sheath tumors (two), were negative for MAb 5.8A. The high level of sensitivity and specificity of MyoD1 expression indicates the value of this marker in the diagnosis of soft tissue sarcomas.

Adolescent↗
PubMed2006

The myxoid/round cell liposarcoma fusion oncogene FUS-DDIT3 and the normal DDIT3 induce a liposarcoma phenotype in transfected human fibrosarcoma cells.

Myxoid/round cell liposarcoma (MLS/RCLS) is the most common subtype of liposarcoma. Most MLS/RCLS carry a t(12;16) translocation, resulting in a FUS-DDIT3 fusion gene. We investigated the role of the FUS-DDIT3 fusion in the development of MLS/RCLS in FUS-DDIT3- and DDIT3-transfected human HT1080 sarcoma cells. Cells expressing FUS-DDIT3 and DDIT3 grew as liposarcomas in severe combined immunodeficient mice and exhibited a capillary network morphology that was similar to networks of MLS/RCLS. Microarray-based comparison of HT1080, the transfected cells, and an MLS/RCLS-derived cell line showed that the FUS-DDIT3- and DDIT3-transfected variants shifted toward an MLS/RCLS-like expression pattern. DDIT3-transfected cells responded in vitro to adipogenic factors by accumulation of fat and transformation to a lipoblast-like morphology. In conclusion, because the fusion oncogene FUS-DDIT3 and the normal DDIT3 induce a liposarcoma phenotype when expressed in a primitive sarcoma cell line, MLS/RCLS may develop from cell types other than preadipocytes. This may explain the preferential occurrence of MLS/RCLS in nonadipose tissues. In addition, development of lipoblasts and the typical MLS/RCLS capillary network could be an effect of the DDIT3 transcription factor partner of the fusion oncogene.

Adipogenesis↗
PubMed1997

Relevance of cytogenetic and fluorescent in situ hybridization analyses in the clinical assessment of soft tissue sarcoma.

Soft tissue sarcomas are a heterogeneous group of malignant tumors displaying a wide range of clinical presentations, morphological features, and biological behaviors. These characteristics and the recent development of differentiated treatment regimens for the different types of soft tissue sarcomas call for refined histological classification using additional ancillary approaches such as cytogenetic and molecular genetic analyses. We coupled classical cytogenetics and fluorescent in situ hybridization (FISH) on both metaphases and interphase nuclei to show the feasibility of this approach to characterize tumor type-specific chromosome rearrangements in soft tissue sarcomas of different histotype. In 35 cases analyzed, we detected the presence of specific chromosome rearrangements such as t(X;18) in synovial sarcoma (SS), t(12;16) in myxoid liposarcoma (MLS), t(11;22) in peripheral primitive neuroectodermal tumors (pPNET), t(2;13) in alveolar rhabdomyosarcoma (ARMS) and ring chromosomes in dermatofibrosarcoma protuberans (DFSP). In several cases, the presence of these cytogenetic rearrangements was of help for a differential diagnosis. The FISH analysis using painting probes not only confirmed the cytogenetic results but also allowed the identification of tumor-specific chromosome changes in those cases presenting low mitotic index or with poor quality chromosomes. Moreover, in the absence of analysable metaphases, FISH was successfully performed on interphase nuclei. Taken together, these results indicate both the diagnostic and clinical relevance of a molecular cytogenetic analysis in the study of soft tissue sarcomas.

Biomarkers, Tumor↗
PubMed2006

Aberrant expression of the Rb pathway proteins in soft tissue sarcomas.

Cell cycle regulation depends on a fine balance between cyclins, cyclin-dependent kinases (CDKs), and cyclin-dependent kinase inhibitors (CKIs) that block the cycle progression. Alterations of the cell cycle regulators are a common feature of many malignant tumors, and some have been shown to have prognostic significance. In this study, 152 cases of different types of soft tissue sarcomas were evaluated for alterations of cell cycle regulator proteins that control the cell cycle progression from G1 to S phase and govern the Rb pathway. Immunohistochemical stains for proteins Rb, E2F1, cyclin D1, CDK4, CDK6, p16, and p27 were carried out on tissue microarrays. The relationship between the expression of these proteins and the histologic grade of the sarcomas was assessed. Altered expression for Rb and p16 proteins was identified in 67.8% and 65.1% of the cases, respectively. Overexpression of E2F1, cyclin D1, CDK4, and CDK6 was detected in 50.7%, 24.3%, 92.1%, and 10.5%, respectively. Overexpression of E2F1 was associated with altered expression of Rb protein. Overexpression of cyclin D1, CDK4, and CDK6 showed an association with normal Rb expression. CDK6 expression revealed a positive correlation with the histologic grade of the sarcoma, and p27 expression was inversely correlated with sarcoma grade. These results suggest that alterations of the Rb pathway proteins are common in soft tissue sarcomas and may participate in their tumorigenesis. CDK6 and p27 showed correlation with the histologic grade of the sarcomas, suggesting that these proteins could be used as prognostic markers.

Biomarkers, Tumor↗
PubMed1997

Adult genitourinary sarcomas: a report of seventeen cases and review of the literature.

Seventeen adult urologic sarcomas treated at the Oncology Hospital of Ankara in the last ten-year period are described. There were six cases of paratesticular rhabdomyosarcoma (RMS), four cases of liposarcoma (LS), three cases of leiomyosarcoma (LMS); two cases of Kaposi sarcoma limited to glans penis, and only one primary malignant fibrous histiocytoma of the kidney. We report our ten years' experience with adult genitourinary sarcomas. The necessity for a uniform staging system and adequate multidisciplinary therapy are discussed.

Adolescent↗
PubMed1974

Simultaneous occurrence of sarcomas in a husband and wife.

The simultaneous occurrence of sarcomas in a husband and wife is documented. The wife first presented with an undifferentiated uterine sarcoma, and within six months of the onset of her disease her husband developed an anaplastic liposarcoma. This finding is considered in the light of recent evidence suggesting a possible viral etiology for human sarcomas.

Aged↗
PubMed1994

MDM2 gene amplification and transcript levels in human sarcomas: relationship to TP53 gene status.

BACKGROUND: Alterations of the TP53 tumor suppressor gene appear to be implicated in the tumorigenesis and progression of several types of human cancer, including different histologic subtypes of sarcomas. The MDM2 (murine double minute-2) gene encodes a nuclear phosphoprotein that may interact with both mutant and wild-type p53 proteins, thereby inhibiting p53-mediated transactivation in a dose-dependent manner. Recently it has been suggested that mdm2 and p53 proteins are components of an autoregulatory loop in which the MDM2 gene is transactivated by p53. PURPOSE: Our purpose was to examine the frequency of MDM2 amplifications in larger panels of sarcomas, determine if the mRNA level could be elevated in tumors without concomitant gene amplification, and relate MDM2 findings to the TP53 status of the tumors. METHODS: Sarcoma tissue of different histologic subtypes was obtained from 68 patients at the time of surgery and from 26 human xenografts in nude mice. In addition, two human sarcoma cell lines (OSA and U2OS) were studied. Genomic DNA from tumor tissue, in vitro cell lines, and peripheral blood cells were isolated by Southern-blot analysis methods to determine MDM2 gene amplification. Tumor DNA was analyzed for possible TP53 gene mutations in exons 5, 7, and 8 by constant denaturing gel electrophoresis. To determine the MDM2 and TP53 mRNA levels, Northern-blot analysis was performed. RESULTS: Amplification of the MDM2 gene was detected in 10 tumors (10.3%). Whereas MDM2 amplification and/or over-expression were found only in two (U2OS and OSA cell lines) of 18 osteosarcomas, one of 20 malignant fibrous histiocytomas (MFHs), and in none of 14 leiomyosarcomas, such alterations were observed in two of two fibrosarcomas, three of six malignant schwannomas, three of 19 liposarcomas, and in the one hemangiopericytoma examined. MDM2 overexpression was found in all nine examined cases with and in three tumors without amplification. TP53 mutations were detected in 12 cases (five osteosarcomas, four MFHs, and three leiomyosarcomas), of which none showed amplification, but one had increased levels of MDM2 mRNA. None of the fibrosarcomas, malignant schwannomas, and liposarcomas examined had mutated TP53. The six sarcomas that showed high TP53 mRNA expression in the absence of gene mutation also had elevated levels of MDM2 mRNA. CONCLUSIONS: The present data provide further indications that increased MDM2 expression level, caused by gene amplification or altered regulation of transcription, is involved in tumor progression of some, but not all, sarcoma subtypes.

Animals↗
PubMed2000

A reverse transcriptase-polymerase chain reaction assay in the diagnosis of soft tissue sarcomas.

BACKGROUND: Many types of sarcomas are characterized by specific chromosomal translocations that result in the production of novel chimeric genes. Detection of these fusion genes could be a sensitive molecular diagnostic assay. However, to the authors' knowledge there have been few systemic comparisons between the current histopathologic diagnosis and the presence or absence of particular fusion genes in patients with adult soft tissue sarcomas (STSs). METHODS: Total RNA was extracted from 75 cases of STS and analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR) assay for the detection of a variety of fusion transcripts. The results of the molecular assay were compared with standard histopathologic diagnoses. RESULTS: Of the 18 tumors diagnosed as synovial sarcoma, 17 (94%) expressed SYT-SSX chimeric transcripts. All nine myxoid liposarcomas were positive for FUS-CHOP fusion transcripts. Of the four cases of Ewing sarcoma, two had an EWS-FLI1 fusion transcript and one had an EWS-ERG fusion transcript. A clear cell sarcoma had a EWS-ATF1 fusion transcript. None of 19 cases of malignant fibrous histiocytoma nor 3 leiomyosarcomas contained a fusion transcript. Three cases with an initial diagnosis other than synovial sarcoma expressed a SYT-SSX fusion transcript. A review of the slides and additional examination showed that a diagnosis of synovial sarcoma was appropriate for these cases. There was a trend for biphasic synovial sarcoma to contain the SYT-SSX1 fusion. CONCLUSIONS: The authors believe RT-PCR assay for the detection of a specific fusion gene provides a useful tool for confirmation of the diagnosis of adult STS in diagnostically difficult cases and in retrospective studies.

DNA Primers↗
PubMedDate not supplied

From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation.

Liposarcoma is the second most common type of soft-tissue sarcoma, accounting for 10%-35% of these lesions. The World Health Organization has categorized soft-tissue liposarcomas into five distinct histologic subtypes: well differentiated, dedifferentiated, myxoid, pleomorphic, and mixed type. Well-differentiated liposarcomas frequently demonstrate a diagnostic appearance on computed tomographic (CT) or magnetic resonance (MR) images, with a largely lipomatous mass (>75% of the lesion) and nonlipomatous components in thick septa or focal nodules. The CT or MR imaging finding of a nodular dominant focus (>1 cm in size) of nonlipomatous tissue in a well-differentiated liposarcoma suggests dedifferentiated liposarcoma, and biopsy should be directed at the nonadipose component. The high water content of myxoid liposarcoma seen at pathologic analysis and constituting the majority of the lesion is reflected at sonography, CT, and MR imaging. However, the detection of a small amount of adipose tissue in the septa or as small nodular foci superimposed on the background of myxoid tissue allows prospective diagnosis in 78%-95% of myxoid liposarcomas. Pleomorphic liposarcomas are high-grade sarcomatous lesions and typically appear as heterogeneous soft-tissue masses, although small amounts of fat are seen on MR images in 62%-75% of cases, findings that suggest the diagnosis. Mixed-type liposarcomas have features representing a combination of the other subtypes. Primary liposarcoma of bone is exceedingly rare and usually demonstrates aggressive nonspecific features, although fat may be seen. Understanding and recognition of the spectrum of appearances of the various types of musculoskeletal liposarcoma, which reflect their underlying pathologic characteristics, improves radiologic assessment and is vital for optimal patient management.

Adolescent↗