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PubMed1984

Malignant hemangiopericytoma and other sarcomas with hemangiopericytoma-like pattern.

This clinicopathologic study concerns 19 cases of malignant hemangiopericytoma among 755 cases of soft tissue sarcomas. The age of the patients ranged from 18 to 76 years, with a median of 43 years. Tumors occurred on the trunk in 8, lower extremities in 5, the head in 3, and the retroperitoneum in 3. According to follow-up information, nine of the 19 patients had died. Histologically the tumor was characterized by its homogeneous vascular pattern, its uniform cell population and a wide range of cellular anaplasia. After extensive sampling of the tumors, a comparative light microscopy revealed differences in diagnostic histology between malignant hemangiopericytoma and other soft tissue sarcomas with a hemangiopericytoma-like vascular pattern. The frequency of appearance of such pericytoma pattern in different soft tissue sarcomas was as follows: 4/4 cases (100%) in extraskeletal mesenchymal chondrosarcoma, 11/14 (79%) in infantile fibrosarcoma, 27/45 (60%) in synovial sarcoma, 62/201 (30%) in malignant fibrous histiocytoma, 9/37 (25%) in malignant schwannoma and 6/72 (8%) in liposarcoma.

Adolescent↗
PubMed1992

Cytogenetic findings in liposarcoma correlate with histopathologic subtypes.

The cytogenetic findings in 31 liposarcomas from 26 patients are reported. Four other tumors did not grow. Three histologic types are represented in this analysis. The well-differentiated liposarcomas were characterized by telomeric associations, large marker chromosomes and ring chromosomes, and in some cases, double minutes. The pleomorphic liposarcomas contained very high clonal chromosomal numbers with near-tetraploid modes and numerous variable, often unidentifiable, chromosomal abnormalities. The myxoid liposarcomas were characterized primarily by a t(12;16)(q13;p11) as the sole abnormality or additional changes. These results indicate that cytogenetic findings may provide a new criterion, not only for establishing the diagnosis of liposarcoma, but also for differentiating confusing histologic types of liposarcoma and these lesions from other types of sarcomas.

Aged↗
PubMed2003

Protein gene product 9.5 (PGP 9.5) is not a specific marker of neural and nerve sheath tumors: an immunohistochemical study of 95 mesenchymal neoplasms.

In non-neoplastic tissues, the expression of protein gene product 9.5 (PGP 9.5), a member of the ubiquitin hydrolase family of proteins, is confined to neural and neuroendocrine cells. Although it has been claimed that PGP 9.5 is a specific marker of neural and/or nerve sheath differentiation in human tumors, careful review of the literature suggests that relatively few nonneural or nerve sheath tumors have been studied. Prompted by our recent observation of a PGP 9.5-positive malignant fibrous histiocytoma, we undertook a study of PGP 9.5 expression in a large group of well-characterized mesenchymal neoplasms. Sections from 95 mesenchymal tumors were retrieved from our archives and immunostained for PGP 9.5 using standard avidin-biotin complex technique and heat-induced epitope retrieval. Scoring was as follows: negative, 1+ (<10-25% of cells), 2+ (25-50% of cells), and 3+ (>50% of cells). Normal nerves and fibrous tissue were internal positive and negative controls, respectively. Positive immunostaining was seen in 80/95 (84%) of cases. Positive results by tumor subtype were as follows: (1) nerve sheath tumors: malignant peripheral nerve sheath tumor (7/10), neurofibromas (10/10), and perineuriomas (3/3); (2) (Myo) fibroblastic tumors: malignant fibrous histiocytoma (18/20), low-grade fibromyxoid sarcomas (8/9), fibromatoses (7/7), and desmoplastic fibroblastomas (2/2); (3) vascular tumors: angiosarcomas (4/4), hemangioendotheliomas (3/5), and hemangiomas (3/4); and (4) other non-nerve sheath tumors: pleomorphic liposarcoma (4/4), dermatofibrosarcoma protuberans (2/5), rhabdomyosarcomas (2/2), synovial sarcomas (8/8), melanomas (1/2). All positive cases were 2-3+ except 6 malignant peripheral nerve sheath tumor, 1 neurofibroma, 3 malignant fibrous histiocytoma, 2 low-grade fibromyxoid sarcoma, and 1 dermatofibrosarcoma protuberans. Positive staining was seen in normal smooth muscle and germinal centers in addition to nerves. We conclude that in this, the largest study to date of PGP 9.5 expression in mesenchymal neoplasms, we have found strong (2-3+) expression in the vast majority of nonneural or nerve sheath neoplasms studied. Although PGP 9.5 is a sensitive neural/nerve sheath marker, it is essentially totally nonspecific for diagnostic purposes. It is possible that our findings reflect cross-reactivity of the 13C4 clone with epitopes present on other ubiquitin hydrolases. Alternatively, PGP 9.5 expression may be aberrantly up-regulated in a variety of mesenchymal neoplasms.

Biomarkers, Tumor↗
PubMed1986

New monoclonal antibodies specific for human sarcomas.

Monoclonal antibodies (MAbs) against sarcoma-associated cell membrane antigens were prepared by immunizing BALB/c mice with tumor cells from a human osteosarcoma, TPX, grown as a xenograft in athymic BALB/c nude mice. Spleen cells from immunized mice were hybridized with X-63 Ag. 8.653 mouse myeloma cells which yielded 260 growing hybridomas. Seven of these produced antibodies that bound to TPX cells and to cells from another osteosarcoma, but not to autologous skin fibroblasts. MAbs from 2 (TP-1 and TP-3) of these 7 clones did not cross-react with non-sarcomatous tumor cells or peripheral blood lymphocytes. Immunohistochemical studies on frozen tissue sections showed that the TP-1 (IgG-2a) and TP-3 (IgG-2b) antibodies had characteristic and identical specificity profiles. Binding of TP-1 (TP-3) was demonstrated to 15/15 (15/15) osteosarcomas, 3/3 (2/2) synovial sarcomas, 7/9 (6/8) malignant fibrous histiocytomas, 2/2 (1/1) malignant hemangiopericytomas, 1/2 (1/2) chondrosarcomas and 3/6 (1/3) unclassified sarcomas. The antibodies did not bind to any of 16 sarcomas belonging to other histological subtypes, including liposarcomas and leio- and rhabdomyosarcomas. Moreover, they failed to bind to sections of 66 different non-sarcomatous malignancies, or to any of a range of normal adult and fetal tissues, although some weak staining of proximal kidney tubules was seen. The restricted specificity of these antibodies to some major subtypes of human sarcomas makes them promising tools for identification and subclassification of sarcomas.

Animals↗
PubMed1990

Breast sarcoma. A clinicopathologic review of 25 cases.

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.

Adult↗
PubMed1987

Liposarcoma of the spermatic cord: case report and review of the literature.

This report describes a patient with liposarcoma of the spermatic cord. The clinical presentation, pathogenesis, and prognosis of this unusual sarcoma are reviewed. The low-grade malignant potential and irregular growth characteristics of this liposarcoma render preoperative diagnosis difficult. The role of radical orchiectomy, retroperitoneal lymph node dissection, radiation therapy, and chemotherapy in the treatment of spermatic cord sarcomas is discussed.

Genital Neoplasms, Male↗
PubMed2002

Soft tissue liposarcoma: histological subtypes, MRI and CT findings.

PURPOSE: Liposarcoma is the second most common malignancy of soft tissues. The commonly accepted classification of liposarcoma includes five basic histological categories: well-differentiated, myxoid, round cell, dedifferentiated and pleomorphic liposarcoma. The clinical behaviour of liposarcoma closely reflects its histological appearance, so that to identify the histological subtypes is very important for both prognosis and therapy. The aim of this study was to ascertain whether the histological features of liposarcoma subtypes can be correlated with MRI (Magnetic Resonance Imaging) and CT (Computed Tomography) findings by retrospectively evaluating nineteen cases of histologically-proved liposarcoma. MATERIALS AND METHODS: The MRI examinations performed over the past eight years on nineteen patients affected by liposarcoma were retrospectively reviewed. All patients underwent ultrasound and MRI examination; T2 and T1-weighted sequences were available in all cases and fat-saturated sequences in 4 cases; all patients were administered paramagnetic contrast material. CT scans were obtained in twelve patients. All patients had a biopsy, surgical resection and histology. RESULTS: The study group had 7 well-differentiated, 8 myxoid, 3 pleomorphic and 1 round cell liposarcoma. Well-differentiated liposarcomas had largely lipomatous appearance on both CT and MRI, typically with septa and areas showing high signal intensity on T2w MR images and low signal intensity on T1w images, and slightly hypodense compared to the muscle in CT, representing the sarcomatous areas. Myxoid liposarcomas were mildly heterogeneous with typical high signal intensity on T2w images and isointense to the muscle in T1w images, with lacy or linear septa of fatty tissue in six cases. The pleomorphic and round-cell subtypes demonstrated marked heterogeneity on MR images, with areas of necrosis and heterogeneous contrast enhancement, indistinguishable from other high-grade sarcomas. DISCUSSION AND CONCLUSIONS: Well-differentiated liposarcoma may be distinguished from other types of liposarcoma by its largely lipomatous appearance. Myxoid liposarcoma may be distinguished on the basis of its homogeneous or mildly heterogeneous structure due to the large amounts of the extracellular myxoid material that give it its typical MR appearance. Both well-differentiated and myxoid liposarcomas, the most common types accounting for about 50% of all liposarcomas, have a more favourable clinical behaviour than the other histological types. Differentiation of these from the other histological types of liposarcoma therefore has a high significance for prognosis and therapeutical approach. On the basis of our experience and of the literature, we believe that diagnostic imaging and in particular the MR examination may lead to a correct diagnosis.

Adult↗
PubMed1999

[Retroperitoneal mixoid liposarcoma: a difficult diagnosis with often unfavorable prognosis. Case report].

Liposarcoma is the most common adult soft tissue sarcoma. The hallmark is the immature fat cell or lipoblast. It occurs in late adult life, frequently in the lower extremities, in the retroperitoneal, perineal and mesenteric region. Retroperitoneal liposarcoma doesn't produce symptoms until it is very large. A review of the literature is made and the case of a 65-years old man, submitted to surgical treatment for a bulky retroperitoneal mixoid liposarcoma is reported.

Aged↗
PubMed1999

Prognostic factors in retroperitoneal sarcomas: ploidy of DNA as a predictor of clinical outcome.

BACKGROUND AND OBJECTIVES: Radical surgery is the best mode of treatment of retroperitoneal sarcomas (RS); however, common recurrences are unpredictable. METHODS: For the better understanding of outcomes and possibilities of treatment retrospective analysis of different factors, including DNA content, was performed based on 70 patients. RESULTS: Leiomyosarcoma and liposarcoma were most common histologic types of classified sarcomas. Different kinds of resection were successfully performed in 51 patients (73%) and 35 of their available DNA specimens were analyzed. The actuarial 5- and 10-year survival rates in the resection group were 53% and 40%, respectively, with the median survival of 57 months. Patients with diploid resected tumors had a better 10-year survival rate (58%), than those patients with aneuploid tumors (25%,)--P<0.005. Those patients with low-grade sarcomas had a significantly longer survival than those with high-grade sarcomas (10-year survival rate: 44% compared to 29%). In the univariate analysis, adjuvant therapy, type of histology, type of surgery, location of tumor, and S-phase fraction had no influence on survival. In the multivariate analysis (Cox), only ploidy was an independent prognostic variable. Relative risk of death was over three times higher for aneuploid than for diploid tumors. CONCLUSION: Tumor ploidy should be analyzed in every case of retroperitoneal sarcoma for better assessment of prognosis and possible indication for adjuvant therapy.

Adult↗
PubMed2006

Myxoid liposarcoma of the breast--report of a rare case.

Breast sarcomas are very rare. A 40 year old lady presented with a well-defined hard lump, 6 cm in size, in the left breast. Patient underwent simple mastectomy with axillary clearance following a diagnosis of phylloides tumour on fine needle aspiration cytology. Grossly, it was a circumscribed myxoid tumour. Histopathologically, it fulfilled all the criteria for a diagnosis of myxoid liposarcoma. The absence of epithelial component excluded phylloides tumour.

Adult↗
PubMed2003

Localized extremity soft tissue sarcoma: improved knowledge with unchanged survival over time.

PURPOSE: The objective of this study was to define whether survival of patients with extremity soft tissue sarcoma (STS), stratified for known risk factors, has improved over the last 20 years. PATIENTS AND METHODS: From January 1982 to December 2001, 1,706 patients with primary and recurrent STS of the extremities were treated at our institution and were prospectively followed. From this cohort, we selected 1,261 patients who underwent complete macroscopic resection and had one of the following histopathologies: fibrosarcoma, liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, or synovial sarcoma. Median follow-up was 55 months. Patient, tumor, and treatment factors were analyzed as prognostic factors. RESULTS: The 5-year disease-specific actuarial survival was 79% (78% for patients treated from 1982 to 1986, 79% for patients treated from 1986 to 1991, 79% for patients treated from 1992 to 1996, and 85% for patients treated from 1997 to 2001; P = not significant). For high-risk patients (high-grade, > 10 cm, deep tumors; n = 247), 5-year disease-specific survival was 51% (50% for patients treated from 1982 to 1986, 45% for patients treated from 1986 to 1991, 52% for patients treated from 1992 to 1996, and 61% for patients treated from 1997 to 2001; P = not significant). Tumor depth, size, grade, microscopic margin status, patient age, presentation status (primary tumor versus local recurrence), location (proximal versus distal), and certain histopathologic subtypes were significant prognostic factors for disease-specific survival on multivariate analysis; however, time period of treatment was not. CONCLUSION: Prognosis of patients with extremity STS, stratified for known risk factors, has not improved over the last 20 years, indicating that current therapy has reached the limits of efficacy.

Adult↗
PubMed1978

Spermatic cord sarcoma in adults.

The clinical courses are presented for 6 patients with sarcoma of the spermatic cord seen between 1967 and 1976. There were 3 fibrosarcomas, 2 liposarcomas and 1 malignant fibrous histiocytoma, making the total reported cases of spermatic cord sarcoma 212. Management of spermatic cord sarcoma is discussed.

Adult↗
PubMed1995

The emerging molecular genetics of sarcoma translocations.

Many types of sarcomas are characterized by specific chromosomal translocations which are likely to be of etiologic significance. The recent elucidation of the molecular structure of the several of these translocations has revealed some striking similarities. Nearly all appear to result in the production of novel, tumor-specific chimeric transcription factors. Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). The observation that the different translocation partners of the EWS gene are specifically associated with several distinct types of primitive sarcomas suggests a model in which the translocation partner supplying the DNA-binding domain confers the target specificity of the transcriptional activation mediated by these chimeric proteins, whereas the partner supplying the N-terminal domain and promoter region determines their transactivation potential and expression level. Further analysis of the normal functions and expression patterns of these genes should yield insights into the histogenesis of these different tumor types and into normal tissue development and differentiation. Clinically, our new understanding of the molecular structure of these translocations opens new avenues for molecular diagnosis and investigative therapeutics.

Base Sequence↗
PubMedDate not supplied

Chromosome-12 copy number alterations and MDM2, CDK4 and TP53 expression in soft tissue liposarcoma.

BACKGROUND: Liposarcoma is a heterogeneous group of soft tissue sarcomas in which definitive prognostic parameters need to be identified. MATERIALS AND METHODS: The series included 33 consecutive soft tissue (well-differentiated, WDLPS, n=19; and dedifferentiated, DDLPS, n=14) liposarcoma. Clinicopathological variables included age, gender, body location, degree of dedifferentiation and mitotic count. The rrolecular analysis included MDM2, CDK4 and TP53 expressions and chromosome-12 copy number alterations. RESULTS: Centrally located (retroperitoneal, abdominal cavity or groin region) WDLPS had more dedifferentiation (p=0.001). Patients with DDLPS and a high mitotic rate died (p=0.070) or experienced recurrencies (p=0.029) more frequently. Co-expression of MDM2/CDK4 (p=0.001) and TP53 accumulation (p=0.017) related to dedifferentiation but not to recurrence or death, both in WDLPS and DDLPS. DDLPS had higher centromeric chromosome-12 copy number than WDLPS (p=0.013), but this was unrelated to recurrence or death. CONCLUSION: Central location is a risk factor in WDLP. Co-expression of MDM2/CDK4/TP53 and chromosome-12 alterations characterize DDLPS suggesting a link with dedifferentiation.

Adult↗
PubMed1991

Liposarcoma of the esophagus.

Liposarcoma is one of the most common soft tissue sarcomas found in adults, yet it rarely develops in the alimentary tract. This report describes the pathological features and management of a liposarcoma of the esophagus.

Aged↗