Specificity of antibody induced in sarcoma patients immunized with allogeneic sarcoma cells.
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Absorption↗
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Results for “SARCOMA, LIPOSARCOMA”
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Amplification of chromosome 1 sequences in lipomatous tumors and other sarcomas.
Amplifications and gains involving 1q are common abnormalities in solid tumors. Recently, an amplicon originating from 1q21-23, containing the candidate oncogenes COAS1, COAS2 and COAS3 (Chromosome One Amplified Sequence) was identified. The presence, distribution and copy number level of extra COAS sequences were investigated in 48 bone and soft tissue tumor (BSTT) samples using metaphase FISH analysis. Amplification was seen in 27/48 (56%) samples. With few exceptions, all 3 genes were involved, but on average COAS2 exhibited higher copy numbers. The presence of extra COAS signals, irrespective of copy numbers, was found at similar frequencies in different histologic tumor subtypes. However, medium or high level amplification was common in lipomatous tumors but rare in other, nonlipomatous tumors (9/21 vs. 2/27 samples). The most common localization of extra COAS signals in lipomatous tumors was in supernumerary ring and giant marker chromosomes. Among nonlipomatous tumors, the distribution of extra COAS genes was more disperse, being located in various unidentified chromosomal structures, including double minutes, and only rarely in ring chromosomes. Because MDM2 is known to be amplified frequently in BSTTs, and in particular in atypical lipomatous tumors, cases with extra copies of COAS were studied also with an MDM2 probe. Twelve out of 18 lipomatous tumors had extra copies of both COAS and MDM2, and the 2 genes were found to be coamplified and interspersed exclusively in ring and giant marker chromosomes. Also 12 out of 18 nonlipomatous tumors exhibited simultaneous gain of COAS and MDM2, but colocalization in the same chromosome was less frequent. The role of the frequent coamplification of COAS, or some other yet unknown gene in the 1q21-23 region, and MDM2 remains to be elucidated.
Adult↗
Surgical management of primary retroperitoneal sarcoma.
BACKGROUND: Retroperitoneal sarcoma is a rare and challenging group of diseases for surgeons, characterized by extensive growth and high recurrent rate. We analyzed data from our patients to identify the factors of survival. METHODS: From 1971 to 1993, medical records of 40 patients with primary retroperitoneal sarcoma were reviewed. Clinical factors including age, sex, tumor size, histopathology, type of operation, disease-free interval, and number of operation were collected to correlate with patient's survival. RESULTS: Most patients presented with huge abdominal mass. There were 22 liposarcomas, 8 leiomyosarcomas, 5 malignant fibrous histiocytomas, 2 fibrosarcomas, 2 malignant mesenchymomas and one rhabdomyosarcoma. Twenty-eight patients received complete resection and 12 had incomplete resection. The group with complete resection showed a better survival than incomplete resection group (p = 0.0001). Nineteen patients with complete resection had tumor recurrence. The recurrent rate was 68%. Patients having been disease-free for more than 12 months showed to have better survival than those less than 12 months (p = 0.005). Aggressive surgical resection was done in case of tumor recurrence. Patients who received more than 2 (> or = 3) operations also showed a better survival than those with less than 2 operations (p = 0.0247). CONCLUSIONS: Complete surgical resection and aggressive repeated surgical intervention are the most effective treatment modality for better survival in patients with retroperitoneal sarcoma.
Adult↗
How would you manage recurrent liposarcoma of the chest wall?
Summary of the cases presented in full in the August issue (Eur J Surg Oncol 1995; 21: 424-426 Patient 1. A 46-year-old female presented with a huge benign lipoma of the left axilla/breast in 1977. Repeated excisions led to three recurrences, the first reported as an intramuscular lipoma, the latter two as well-differentiated liposarcoma. She presented in 1990 with a large recurrence partially fixed to the chest wall. Patient 2. A 58-year-old man with cardiorespiratory impairment due to long-standing valvular disease presented in 1982 with a large poorly differentiated sarcoma over the lower posterior rib cage. He was treated by wide local excision (not including the ribs), and cover with a latissimus dorsi flap to allow completion of a radical perioperative course of radiotherapy. Recurrences around the periphery of the excision occurred in 1984, 1985, 1986, 1987, each treated by relatively wide local excision. He presented again in 1989 with further peripheral recurrences attached to the rib cage, still without evidence of distant metastasis, at which stage a further plan of management was sought.
Combined Modality Therapy↗
Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk.
OBJECTIVE: To determine the prognostic significance of histologic subtype in a large series of patients with primary liposarcoma (LS) and to construct a LS-specific postoperative nomogram for disease-specific survival (DSS). SUMMARY BACKGROUND DATA: Nomograms, used to define and predict outcome following operative intervention, may contain variables not conventionally used in standard staging systems. A 12-year DSS postoperative nomogram for all sarcomas has already been established. METHODS: From a single-institution prospective sarcoma database, patients with primary extremity, truncal, or retroperitoneal LS treated between 1982 and 2005 were identified. Histology was reviewed by a sarcoma pathologist and divided into 5 subtypes. A nomogram predictive of 5- and 12-year DSS was developed. RESULTS: Of 801 patients with primary LS resected with curative intent, 369 (46%) presented with well-differentiated, 143 (18%) dedifferentiated, 144 (18%) myxoid, 81 (10%) round cell, and 64 (8%) pleomorphic histology. The median tumor burden was 15 cm (range, 1-139 cm). At last follow-up, 560 patients were alive with a median follow-up time of 45 months (range, 1-264 months) and 51 months for surviving patients. The 5- and 12-year DSS rates were 83% (95% confidence interval [CI], 80%-86%) and 72% (95% CI, 67%-77%), respectively. The nomogram was drawn on the basis of a Cox regression model. The independent predictors of DSS were age, presentation status, histologic variant, primary site, tumor burden, and gross margin status. The nomogram was internally validated using bootstrapping and shown to have excellent calibration. The concordance index was 0.827 compared with 0.776 for the general sarcoma postoperative nomogram for 12-year DSS. CONCLUSION: The LS-specific nomogram based on histologic subtype provides more accurate survival predictions for patients with primary LS than the previously established generic sarcoma nomogram. DSS nomograms aid in more accurate counseling of patients, identification of patients appropriate for adjuvant therapy, and stratification of patients for clinical trials and molecular analysis.
Adolescent↗
A novel effector domain from the RNA-binding protein TLS or EWS is required for oncogenic transformation by CHOP.
In human myxoid liposarcoma, a chromosomal rearrangement leads to fusion of the growth-arresting and DNA-damage-inducible transcription factor CHOP (GADD153) to a peptide fragment encoded by the TLS gene. We have found that wild-type TLS and a closely related sarcoma-associated protein, EWS, are both abundant nuclear proteins that associate in vivo with products of RNA polymerase II transcription. This association leads to the formation of a ternary complex with other heterogeneous RNA-binding proteins (hnRNPs), such as A1 and C1/C2. An NIH-3T3-based transformation assay was used to study the oncogenic role of the sarcoma-associated domain of these RNA-binding proteins. Transduction of the TLS-CHOP oncogene into cells by means of a retroviral expression vector leads to loss of contact inhibition, acquisition of the ability to grow as colonies in soft agar, and tumor formation in nude mice. Mutations that interfere with the function of the leucine zipper dimerization domain or the adjacent basic region of CHOP abolish transformation. The essential role of the TLS component was revealed by the inability of truncated forms to fully transform cells. Domain swap between TLS- and EWS-associated oncogenes demonstrated that the component contributed by the RNA-binding proteins are functionally interchangeable, whereas the transcription factor component specifies tumor phenotype. The sarcoma-associated component of TLS and EWS contribute a strong transcriptional activation domain to the fusion proteins; however, transforming activity cannot be fully substituted by fusion of CHOP to other strong trans-activators. The juxtaposition of a novel effector domain from sarcoma-associated RNA-binding proteins to the targeting domain of transcription factors such as CHOP leads to the creation of a potent oncogene.
3T3 Cells↗
Isolated pancreatic metastasis of extremity myxoid liposarcoma: Report of a case.
Myxoid liposarcoma (ML) has a high predilection for extrapulmonary sites of metastases, including intra-abdominal metastases, but pancreatic involvement is extremely rare. Here, we report the case of a 66-year-old male patient, who underwent pancreaticoduodenectomy for isolated pancreatic metastasis of ML of the left lower extremity that had been excised 6 years before. Completion pancreatectomy was necessitated afterwards for a delayed haemorrage associated with pancreatic fistula. Currently the patient is alive with no evidence of disease. Highly selected patients with isolated pancreatic metastasis of soft tissue sarcoma may benefit from a curative surgical resection.
Aged↗
Responses of tumors induced in inbred guinea pig strain JY=1 and strain Hartley/F to BCG.
A transplantable fibrosarcoma induced in inbred JY-1 guinea pig strain by 3-methylcholanthrene (MCA) and designated J4, an allotransplantable subline of J4 (JH4) which was obtained by the transplantation of J4 into the inbred Hartley/F guinea pig strain and maintained by passages in this strain, and a syngeneic liposarcoma H10 induced in a Hartley/F guinea pig by MCA were tested for their immunotherapeutic response with BCG. The growth of J4 and H10 tumors was suppressed in most of the animals when tumor cells were mixed with BCG before being injected sc into BCG-immune or BCG-nonimmune recipients. The growth of the JH4 tumor was suppressed at the sites of injection with a mixture of tumor cells and BCG in BCG-immune recipients but not in nonimmune animals. All guinea pigs surviving the injection of a tumor cell-BCG mixture resisted a second tumor cell challenge. When subcutaneous sarcomas grew to about 8-15 mm in diameter, BCG was injected into the tumors. The growth of JH4 tumor was not influenced by the injection in either BCG-immune or BCG-nonimmune animals, while the regression of the established J4 transplants was produced in 2 of 3 nonimmune recipients. The growth of the H10 tumor was not inhibited with an intratumor injection into nonimmune guinea pigs, while the H10 tumor regressed in BCG-immune animals for 4-5 weeks after intratumor injection and thereafter grew progressively. Skin reactions in animals that received repeated intradermal injections of the tumor cells and BCG were tested with 10(6) viable tumor cells as eliciting antigens. Typical delayed-type hypersensitivity reactions that were specific to the homologous antigens were observed. The possible reasons for the different responses to BCG among the guinea pig tumors, including line-10 hepatocarcinoma in strain-2 guinea pigs, were discussed.
Animals↗
Tumors of the lower extremities.
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Adolescent↗
[Isolation, analysis and radioactive labeling of nucleic acids from rat tumors induced by polyoma virus].
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Adenocarcinoma↗
Low grade fibromyxoid sarcoma: report of a case with fine needle aspiration cytology and histologic correlation.
BACKGROUND: Low grade fibromyxoid sarcoma has been fully described histologically; however, the fine needle aspiration (FNA) cytologic findings are scantily defined, and the distinction from other benign and malignant soft tissue tumors can be difficult. CASE: We examined FNA cytologic material from a slowly growing, large chest wall mass in a 28-year-old woman. The surgical specimen was processed for routine histology and immunohistochemical studies. The cytologic smears were adequately cellular, showing spindly cells with uniform, elongated nuclei; small, inconspicuous nucleoli; and scanty, wispy cytoplasm associated with myxoid material. No significant nuclear pleomorphism or mitoses were noted. The excised tumor was well circumscribed, focally infiltrating the surrounding muscles. The cut surface was variable, featuring fibrous, solid, fleshy and myxoid areas. Microscopically, the solid, fibrous areas displayed increased cellularity with storiform, intersecting and parallel patterns. In the myxoid areas the cells grew in a haphazard fashion and appeared floating in abundant mucoid matrix associated with a capillary vascular network similar to the chicken-wire pattern seen in cases of myxoid liposarcoma. The tumor cells were spindly, with fusiform, uniform nuclei. Focal, moderate nuclear pleomorphism was noted. The mitotic index was low. The tumor cells were positive for vimentin, alpha-1-antitrypsin and lysozyme and negative for S-100, actin, desmin and CD34. CONCLUSION: Although low grade fibromyxoid sarcoma is a rare neoplasm, it should be recognized and distinguished from other soft tissue tumors because of its low malignant potential. The definitive FNA cytologic diagnosis can be challenging but is possible if the tumor is adequately sampled, with multiple passes from different areas. Clinical and radiologic correlations are of great help. All spindle cell tumors with myxoid changes, such as myxoid liposarcoma, myxofibrosarcoma, cellular myxoma, myxoid leiomyosarcoma and peripheral nerve sheath tumors, should be considered in the differential diagnosis. In contrast to the cytologic features, the histologic findings are characteristic and well established.
Adult↗
Improved survival following complete excision of retroperitoneal sarcomas.
Charts and slides of 47 patients with primary retroperitoneal sarcomas (excluding pediatric rhabdomyosarcoma) were reviewed to determine clinical presentation, histologic features, extent of surgical resection, operative morbidity and mortality, use of radiation and/or chemotherapy, and survival data. Most patients presented with pain and a palpable mass. Leiomyosarcomas and liposarcomas were the most common tumors. Eighteen of the 47 patients (38%) had complete tumor excision; 68% required resection of adjacent organs. Operative morbidity was 33% with no mortality. After complete resection, the disease-free 5-year survival was 50% and the overall survival was 70% at 5 years; 10-year disease-free survival was 25% with an overall 58% survival at 10 years. Eleven patients (61%) developed recurrent disease with a median interval of 5 years following complete excision. Six patients received adjuvant radiation and/or chemotherapy with four remaining disease-free from 46 to 61 months. Eighteen patients underwent partial excision of tumor and 11 patients underwent biopsy only; these groups had similar survival curves with only 4% alive at 5 years. Their operative morbidity was 18% and mortality was 7%; median time to clinical evidence of tumor progression was 12 months. Sixty per cent of these patients received therapeutic radiation and/or chemotherapy, but their survival was the same as those undergoing surgery alone. These data emphasize the importance of an aggressive surgical approach in the treatment of retroperitoneal sarcomas. Complete tumor resection and total excision of recurrences will allow many patients long-term survival.
Adolescent↗
Electron microscopy of adipose tissue tumors: comparative features of hibernomas, myxoid and pleomorphic liposarcomas.
Two hibernomas, 16 myxoid, and eight pleomorphic liposarcomas were studied by transmission electron microscopy. Neoplastic cells in hibernomas and the type I cells--adipocytes--in myxoid liposarcomas consistently display abundant lipid droplet populations and prominent basal lamina deposition. Despite some variability in their distribution, these features clearly pertain to the mature and/or maturing characteristics of brown and white adipose cells. On the other hand, the more primitive type II cells of myxoid liposarcomas display highly variable, and occasionally absent, lipid droplet populations and basal lamina deposition. Moreover, the type II cells may also display other features such as prominent cytoplasmic filaments and rough endoplasmic reticulum that, while not characteristic of typical adipose cells, are still consistent with the more primitive mesenchymal cells from which they are assumed to derive. The cells of pleomorphic liposarcomas exhibit a variable lipid droplet population and apparently total absence of basal lamina deposition. Moreover, they often display atypical cell junctions that are neither characteristic for adipose cells nor for the "primitive reticular cells" from which adipocytes presumably derive. These observations would suggest that some "high grade" sarcomas may no be truly "undifferentiated"; rather, they may often display structural characteristics indicative of multiple as well as variable lines of differentiation. Therefore, classifications of such tumors based on quantitatively limited observations may not truly reflect their evident complexities.
Adipose Tissue↗
Expression of Ki-1 antigen (CD30) in mesenchymal tumors.
Expression of CD30(Ki-1) antigen has long been considered to be restricted to activated lymphocytes and related tumors. However, expression of this antigen has also been detected in embryonal carcinomas, in nonembryonal carcinomas, in malignant melanomas, and even in some myeloid cell lines and macrophages at late stages of differentiation. In this study, using monoclonal antibody Ki-1, expression of CD30 antigen was immunohistochemically examined in frozen sections of 28 benign and 63 malignant mesenchymal tumors. The authors found CD30 expressed in two of four leiomyomas, seven of 11 leiomysarcomas, one of six rhabdomyosarcomas, two of two aggressive fibromatoses, one of three fibrosarcomas, two of four synovial sarcomas, one giant cell tumors of tendon sheaths, all five malignant fibrous histiocytomas, all three osteosarcomas, one of three Ewing's sarcomas, in a tumor cell subpopulation of two of ten malignant schwannomas, and in the Schwann cell compartment of one of two ganglioneuromas tested. Furthermore, CD30 was consistently expressed in the myoepithelial compartment of 13 fibroadenomas. However, all five lipomas, all seven liposarcomas, all three neuroblastomas, both ganglioneuroblastomas, both chondrosarcomas, and tumors of disputed origin tested were consistently CD30 negative. These findings indicate that, outside the lymphatic system, CD30 antigen is not restricted to epithelial neoplasms but may also be present in tumors of mesenchymal origin. The authors conclude that CD30 antigen, although having limited utility in the differential diagnosis of tumors of questionable histogenesis, may eventually define relevant subgroups within the main tumor categories.
Antibodies, Monoclonal↗
Soft Tissue Masses: The Underutilization of Sonography.
For the evaluation of soft tissue masses, sonography (US) has proved to be very accurate in confirming the presence or absence of a lesion, with a very high negative predictive value. Many soft tissue masses result from trauma, inflammation, infection, or cystic changes and are not true neoplasms. In the proper clinical context, US can diagnose a muscular tear, hernia, myositis ossificans, or rhabdomyolysis. Retained foreign bodies are readily identified and localized with US. US differentiates between cellulitis and abscess and can diagnose masses resulting from tendinitis, tenosynovitis, or bursitis. It is the modality of choice for diagnosing cysts, including intact or ruptured BakerÕs cysts and ganglion cysts in the distal extremities. Among benign neoplasms, lipomas and hemangiomas display a wide spectrum of echogenicity. US can diagnose nerve sheath tumors by demonstrating the connection between the mass and the normal nerve. Except for some well-differentiated liposarcomas, which may appear echogenic, the vast majority of malignant tumors in the soft tissues are hypoechoic. Real-time US is ideal for guiding large-core needle biopsy of soft tissue sarcomas. US is extremely sensitive in detecting early recurrences after surgical excision, which are readily confirmed by US-guided fine-needle aspiration. Any nonpalpable mass visualized by US can be conveniently localized pre- or intraoperatively with US guidance. Provided the examination is done by a well-trained operator using state-of-the-art equipment, the cost-effectiveness of US justifies its use as a first-line examination technique in many situations involving soft tissues, with magnetic resonance imaging being the problem-solving tool and staging procedure.
Journal Article↗
Neoplasm of mixed mesenchymal and neuroepithelial origin: liposarcomatous meningioma combined with gliomas.
A case is reported of an intracranial neoplasm of mixed mesenchymal and neuroepithelial (glial) origin occurring in the parieto-parasagittal region of the brain of a 19 year old man. A meningioma with liposarcomatous and psammomatous components comprised the mesenchymal part of the combined tumour. The neuroepithelial portion was composed of mixed ependymoma and astrocytoma. The combined neoplasm had the gross appearances and several microscopic features of giant-celled glioblastoma, giant cell sarcoma, and monstrocellular sarcoma. The findings suggest that these tumours have a mixed mesenchymal and neuroepithelial origin.
Adult↗
Surgical resection for isolated cardiac metastases.
We performed what we believe to be the second successful resection of metastatic liposarcoma to the heart using cardiopulmonary bypass. Analysis of ten previous resections of cardiac metastases from a variety of tumors from distant primary sites revealed survivals from four months to two years. When patients can tolerate a major operative procedure, resection of solitary cardiac metastases can be successful if the primary tumor is well controlled. Patients with sarcomas and with long disease-free intervals should strongly be considered for aggressive surgical therapy. Computed tomographic scans of the chest were useful in the diagnosis and accurate delineation of the extent of tumor.
Aged↗
Ultrastructural studies in the preoperative cytologic diagnosis of soft tissue tumors.
A consecutive series of 100 patients operated on for lesions that were assumed to be soft tissue tumors, all of whom had been the subject of fine-needle aspiration in the preoperative investigation, is described. A correlative study of smears and the light- and electron-microscopic findings of embedded fine-needle aspirates and the histopathology of the surgical specimens was performed. Eighty of the lesions were found to be genuine soft tissue tumors, of which 51 were sarcomas. The other 20 cases were either metastatic carcinoma, malignant melanoma, or malignant lymphoma. The embedding technique produced additional light-microscopic information about tissue structure and growth pattern, and electron-microscopic information about tissue and cell differentiation of importance to the diagnosis. In the case of certain types of soft tissue tumor, such as lipoma, neurilemmoma, liposarcoma, and malignant fibrous histiocytoma, and for well-differentiated metastatic carcinoma and pigmented malignant melanoma, the diagnosis may be strongly suggested by the appearance of the smears; the embedding technique serves to further support the diagnosis. In the case of small round-cell malignancies, the ultrastructural examination proved to be of special value, ie, in the distinction of rhabdomyosarcoma, poorly differentiated metastatic small cell carcinoma and malignant melanoma, and occasional cases of malignant lymphoma. Spindle cell sarcomas, such as leiomyosarcoma when well differentiated, biphasic synovial sarcoma when it includes glandular structures, and malignant hemangiopericytoma, could be recognized ultrastructurally, although electron-microscopy generally failed to reach a definite diagnosis as to the subtype in most cases of poorly differentiated spindle-cell sarcoma.
Adult↗