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PubMed1981

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

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

Animals↗
PubMed1998

Principles of treatment for soft tissue sarcoma.

Soft tissue sarcomas (STS) are mesenchymal tumors arising from connective tissue elements and are grouped together based on a common biologic behavior. The most common histologic types include malignant peripheral nerve sheath tumors (schwannoma and neurofibrosarcoma) "hemangiopericytoma," fibrosarcoma, and malignant fibrous histiocytoma. These tumors are relatively slow growing yet locally invasive with a high rate of recurrence following conservative management. Appropriate preoperative planning and aggressive surgical resection often result in long-term remission or cure. Identification and evaluation of resection margins are paramount in appropriate case management. The addition of radiotherapy after surgical resection can aid in remission for incompletely resected masses. Systemic chemotherapy for STS should be considered for high-grade tumors with a moderate metastatic potential. Potential prognostic factors include grade, resection margins, size, location, histologic type, and previous treatment, with grade and margins being the most important. Tumor types classified as STS that differ slightly in their presentation or treatment, including synovial cell sarcoma, rhabdomyosarcoma, liposarcoma, and vaccine-associated STS in cats, are discussed. Soft tissue sarcomas can be a frustrating disease to treat, but adherence to solid surgical oncology principles can greatly increase the odds of good disease control.

Animals↗
PubMed1992

The value of immunohistochemistry in the diagnosis of soft tissue sarcomas.

Histological classification of soft tissue sarcomas can be made by light microscopy alone for those tumors with unique appearances, while a significant proportion of sarcomas require the implementation of electron microscopy or immunohistochemistry to obtain a final diagnostic classification. Immunostaining methods are particularly valuable in certain classes of sarcomas such as epithelioid sarcomas, malignant schwannomas, hemangiosarcomas, clear cell sarcomas, rhabdomyosarcomas, pleomorphic liposarcomas, leiomyosarcomas and synovial sarcomas through documentation of their cellular products. Immunohistochemistry is not a substitute for skilled interpretation of conventionally stained microscopic specimens; however, the availability of more specific determinants in the soft tissue neoplasms will expand the value of this approach in this area of diagnostic pathology.

Humans↗
PubMed2004

High-level expression of the coxsackievirus and adenovirus receptor messenger RNA in osteosarcoma, Ewing's sarcoma, and benign neurogenic tumors among musculoskeletal tumors.

PURPOSE: The sensitivity of human tumor tissues to infection with recombinant adenoviruses correlates with the expression of the coxsackievirus and adenovirus receptor (CAR). CAR has been shown to function as the primary receptor for adenoviruses and to play a critical role in adenovirus entry into host cells. It is important for clinical gene therapy to determine the expression level of CAR in tumor tissues. EXPERIMENTAL DESIGN: We analyzed the expression of CAR mRNA in 154 musculoskeletal tumor tissues from 154 patients and 10 normal mesenchymal tissues from 3 patients using reverse transcription-PCR and real-time quantitative PCR. An adenovirus infection assay was performed in two cell lines that were established from CAR-positive osteosarcoma tissue and CAR-negative malignant fibrous histiocytoma tissue. RESULTS: Ninety-nine of 154 tumors were detected as CAR positive by reverse transcription-PCR. We found that the expression levels of CAR mRNA varied markedly between different tumors as determined by real-time quantitative PCR. CAR mRNA was expressed at high levels in osteosarcoma, Ewing's sarcoma, neurofibroma, and schwannoma; at intermediate levels in exostosis, giant cell tumor, liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, and hemangioma; and at low levels in alveolar soft part sarcoma and desmoid. Whereas the osteosarcoma cell line that expressed a high level of CAR mRNA, like its parent tumor, had a high efficiency of adenovirus infection, the malignant fibrous histiocytoma cell line with almost undetectable expression of CAR mRNA, like its parent tumor, had a low efficiency of infection. CONCLUSIONS: Our data showed the great variations in CAR mRNA expression among human musculoskeletal tumors and mesenchymal tissues and implicated the potential usefulness of adenoviral vectors in gene therapy for osteosarcoma, Ewing's sarcoma, neurofibroma, and schwannoma. Efficient transduction with adenovirus for gene therapy could be realized in appropriate, sensitive tumor types.

Adenoviridae↗
PubMed1988

[Soft tissue sarcoma in the head and neck--analysis of 87 patients].

From 1964 to 1984, 87 patients with soft tissue sarcoma (excluding lymphreticulum system sarcoma) in the head and neck were treated in our hospital. All were proved by pathology. The incidence rate of fibrosarcoma was the highest (36.8%), hemangiosarcoma the lowest (5.8%). There was no liposarcoma or synovial sarcoma in this group. The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). Metastasis rate to the lymph nodes was 16-20% for rhabdomyosarcoma, hemangiosarcoma and malignant fibrohistiocyte tumor. No distant metastasis was found in dermatofibrosarcoma protuberans and malignant fibrohistiocyte tumor. Distant metastasis rate was 10-20% for the other types. Local recurrence rate was 9.1% for dermatofibrosarcoma protuberans, 88.9% for malignant fibrohistiocyte tumor, 30-66.7% for the other sarcomas. The 5- and 10-year survival rates were 20% and 0%, 37.5% and 0%, 91.6% and 91.6% for rhabdomyosarcoma, malignant fibrohistiocyte tumor and dermatofibrosarcoma protuberans. There were differences in clinical behavior for soft tissue sarcomas in the head, neck and in the trunk. Highly malignant sarcomas, such as liposarcoma, synovial sarcoma and rhabdomyosarcoma were rare in the head and neck but the low malignant sarcomas in the head and neck were 3-10 times as common as those in the trunk. Distant metastasis rate of the fibrosarcoma was 20% in head and neck and 6.7%in trunk, the 5- and 10-year survival rates were 36% and 77.9%, 25% and 73.6%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1998

[Cardiac tumors (II). Malignant primary tumors. Metastatic tumors. Carcinoid tumor].

Heart neoplasms are of increasing interest among clinicians and surgeons. A review of primary malignant cardiac tumors, secondary cardiac tumors and carcinoid heart tumors is presented, with special reference to their pathological and surgical aspects. Primary malignant heart tumors represent about 25% of all cardiac tumors, the great majority are sarcomas and the whole family of this group is described including angiosarcoma, rhabdomyosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, neurogenic sarcoma, synovial sarcoma and osteosarcoma; mesothelioma, lymphoma, malignant teratoma and thymoma are also included. Metastatic heart tumors are 20-40 times more common than primary malignancies, their behavior and more relevant aspects in diagnostic and surgical therapy are mentioned. Carcinoid heart tumors represent a distinctive entity and are discussed individually.

Carcinoid Tumor↗
PubMed2001

[Clinical patterns of soft-tissue sarcoma in children].

INTRODUCTION AND OBJECTIVES: Soft tissue sarcomas are rare mesenchymal neoplasms that constitute less than 10% of all pediatric malignancies. Half of these are rhabdomyosarcomas, the remaining 50% have a varied and heterogenous histologic and clinical patterns (fibrosarcoma, synovial cell sarcoma, extraskeletal Ewing's sarcoma, angiosarcoma, liposarcoma, leiomyosarcoma, ...). The purpose of this work is to evaluate our clinical experience with soft tissue sarcomas in uncommon sites over the past 10 years in order to delimitate the prognostic factors in survival and modalities of treatment. MATERIAL AND METHODS: Between 1989 and 1998, 10 patients were diagnosed with soft tissue sarcomas in uncommon sites and treated by us over a total number of 139 pediatric neoplasms (7.19%). Data on these patients were obtained from careful review of medical records. Variables investigated included histologic findings, tumor size, age at presentation, primary site, clinical group, radiologic test performed, surgical treatment, radiotherapy and adjuvant chemotherapy, complications and survival rates. Charts were registered in database Access. Statistical analysis was performed by the SPSS 8.0 statistical program. RESULTS: The following histologic types of these 10 tumors were identified: 1 hemangiopericytoma in oral cavity, 2 extraosseous Ewing's sarcoma, 1 botryoid rhabdomyosarcoma of the bladder, 1 mediastinal fibrosarcoma, 1 retroperitoneal rhabdomyosarcoma, 1 paratesticular rhabdomyosarcoma, 1 cervical condrosarcoma, 1 alveolar rhabdomyosarcoma and 1 deltoid rhabdomyosarcoma. 50% were rhabdomyosarcomas and the remaining 50% have a variated sarcomatous histologic pattern with a difficult classification. The mean age at diagnosis was 7 years (4.6 years accounted for rhabdomyosarcoma alone). Surgery with complete excision were performed only in six cases. Adjuvant chemotherapy with IVA was followed by radiotherapy only in four patients. All the children classified in clinical groups II, III or IV needed 2nd. line regimens of chemotherapy. Three patients died in the follow-up instead of the multimodal treatment. Survival ranged from 6 months to 9 years (mean 4 years). CONCLUSIONS: The impact of surgical resectability on outcome in these tumors has been clearly demonstrated over other factors like histologic appearance. Distant metastasis are infrequent but local recurrence are a constant in all cases with incomplete surgical resection. Based on those facts we could advanced an adequate approach to this kind of neoplasms: 1) wide complete surgical resection is mandatory; 2) radiotherapy will only be necessary if margins of resection cannot control the local disease, and 3) chemotherapy have not clearly demonstrated his benefits as adjuvant therapy in clinical group I lesions but his employ is recommended in all cases because of the poor prognosis due to local recurrence.

Adolescent↗
PubMed1996

Urokinase-plasminogen-activator levels and prognosis in 69 soft-tissue sarcomas.

The local and systemic invasiveness of soft-tissue sarcomas may depend upon an interaction between the primary tumour and the extracellular matrix in which the proteolytic enzyme, urokinase plasminogen activator (uPA), may have an important role. We analyzed the expression of uPA in soft-tissue sarcoma using a luminescent immunoassay technique, and examined the relationships between different uPA levels and tumour characteristics and behaviour. We evaluated 69 adult patients with surgically treated soft-tissue sarcomas (MFH 43, leiomyosarcoma 8, liposarcoma 5, synovial sarcoma 4, others 9) of the extremities and trunk wall. Sixteen developed local recurrences, 26 developed metastases, and 5 had both. The median follow-up for survivors was 55 (30-80) months. The median uPA level was 1.4 (0.04-10.6) ng/mg protein. Increasing uPA levels correlated with increasing grade, malignant fibrous histiocytomas, leiomyosarcomas, DNA non-diploidy, tumour necrosis, local recurrence, and metastasis. Storiform-pleomorphic MFH had higher uPA levels than the myxoid variant. A cut-off value of 0.25 ng/mg protein was identified, above which local recurrence and metastasis occurred more frequently. High uPA levels appear to reflect the malignant phenotype in soft-tissue sarcoma, thus supporting the role of uPA as a prognostic indicator.

Adult↗
PubMed1987

[An ultrastructural analysis of malignant soft tissue tumors].

The differential diagnosis of malignant soft tissue tumors constitutes a major problem in surgical pathology. A distinction among sarcomas is sometimes difficult, but this is a situation in which histologic examination of representative tissue will give a diagnosis in the majority of cases. Electron microscopy sometimes contributes to the proper diagnosis of sarcomas. For electron microscopy, fresh samples from 110 cases of sarcoma filed at the Department of Pathology, Faculty of Medicine, Kyushu University, were examined. The distinguishing ultrastructural appearance of the following tumors are discussed: Spindle cell sarcomas (leiomyosarcoma 10 cases, malignant Schwannoma 5, fibrosarcoma 5, monophasic synovial sarcoma 7). Round cell sarcomas (rhabdomyosarcoma 10 cases, extraskeletal Ewing's sarcoma 1, malignant neuroepithelioma 1, malignant rhabdoid tumor of soft parts 3, monophasic synovial sarcoma 9). Pleomorphic sarcomas (malignant fibrous histiocytoma 24 cases, pleomorphic liposarcoma 1). Myxoid sarcomas (myxoid malignant fibrous histiocytoma 8 cases, myxoid liposarcoma 8, extraskeletal myxoid chondrosarcoma 4). Sarcomas with a specific pattern. Using electron microscopy, general analysis of cellular morphology and detection of specific structures are helpful for evaluating these sarcomas. Nevertheless, poorly differentiated sarcomas may still pose a diagnostic dilemma, since their specific features are poorly represented.

Diagnosis, Differential↗
PubMed2001

[Resection of the sciatic nerve with preservation of leg function].

Four patients, three women aged 32, 65 and 77 years and one man aged 51 years, had a soft tissue tumour of the upper dorsal thigh or gluteal region. They were treated by resection of both the sciatic nerve and the tumour. This resulted in a good functional outcome with limited functional disturbances. In the case of the three women, metastases of their malignant schwannoma, sarcoma, or liposarcoma respectively were found after several months to years; 2 of them died from these. The man was treated on several occasions for local recurrences of the malignancy. Patients with soft tissue sarcomas involving the sciatic nerve are often treated by amputating the affected limb. Nerve resection is a good alternative for complete tumour resection whilst still preserving the limb.

Adult↗
PubMed1992

Prognostic significance of histologic parameters of soft tissue sarcomas.

BACKGROUND AND METHODS: A univariate and multivariate analysis for the correlation between histomorphologic factors and prognosis was made using data from 1116 patients with soft tissue sarcoma, including 1005 cases available with complete histologic and follow-up data. RESULTS: The overall 5-year survival rate was 43.6%. The univariate analysis using Kaplan-Meier survival curves showed that tumor differentiation, cellularity, nuclear atypia, cellular pleomorphism, mitotic activity, amount of fibrous stroma, extent of myxoid areas, extent of tumor necrosis, and histologic grading (determined by the estimated range of malignancy for each type of sarcoma using a reported guideline) were all significant prognostic factors in the overall soft tissue sarcoma group. By a multivariate analysis using a procedure based on the Weibull model to failure data, the histologic grading and extent of tumor necrosis were proved to be prognostically significant in the overall sarcoma group. However, after additional analysis on each histologic type of sarcoma, it was determined that both tumor necrosis and histologic grading were applicable in only two types: malignant fibrous histiocytoma and leiomyosarcoma. Liposarcoma, synovial sarcoma, and malignant schwannoma each also showed some determinable factor of prognostic significance. CONCLUSIONS: It was concluded that there seemed to be no single universal prognostic parameter that could be applied to each soft tissue sarcoma type. These results suggest that the predictive significance of the various histologic parameters should be based on each specific type of sarcoma.

Adolescent↗
PubMed1990

Expression of insulin-like growth factor 1 in sarcomas.

The expression of insulin-like growth factor-1 (IGF-1) was studied in normal tissues, in eight benign lesions and in 50 sarcomas. In palmar fibromatosis the spindle cells in cell-dense areas exhibited a strong immunoreactivity. IGF-1 was variably found in leiomyosarcomas (7/8), malignant schwannomas (7/9), synovial sarcomas (2/3), liposarcomas (3/6), fibrosarcomas (1/3), malignant fibrous histiocytomas (10/18) and in one angiosarcoma. Two rhabdomyosarcomas failed to express IGF-1 and only the spindle cell component of synovial sarcomas was positive. Immunoreactivity for IGF-1 in 10 malignant filrous histiocytomas (MFH) appeared to be related to co-expression of smooth muscle actin. These findings imply that MFHs can be subdivided into a group of tumours which are devoid of morphological and immunophenotypic evidence of differentiation and a group which manifest immunophenotypic differentiation.

Actins↗
PubMed1978

[Radiotherapy of the soft tissue sarcomas (author's transl)].

Although 82,5% of the total body weight consist of mesenchymal tissues, only 1%--3% of all malignant tumors are soft tissue sarcomas. The necessary irradiation dose can only be determined after an exact histopathological diagnosis; therefore this diagnosis has to be made prior to the radiotherapy. Except the fibro- and liposarcomas, all sarcoma form very early lymphogenic metastases. All sarcomas show a remarquable tendency to form recurrences. Surgery is the primary therapy. After that, an irradiation of the tumor region and the regional lymph nodes should be effected under all circumstances. We have treated and checked up 749 patients. After this therapeutic method, the five-year survival rate in the stages T1 and T2 is 60% for the myxosarcoma, 67% for the angiosarcoma, 41% for the fasciculated sarcoma, 40% for the globocellular sarcoma, 30% for the polymorphous sarcoma, 69% for the fibrosarcoma, and 61% for the leiomyosarcoma.

Fibrosarcoma↗
PubMed1999

Occupational risk factors for sarcoma subtypes.

Herbicides, chlorophenols, and other occupational exposures are suspected risk factors for soft-tissue sarcoma, but the epidemiologic evidence is inconsistent. Given that soft-tissue sarcomas represent a heterogeneous mix of cancer subtypes and that these subtypes have different disease patterns by race, sex, and age at diagnosis, studying all soft-tissue sarcomas combined may mask subtype-specific associations. Using the Selected Cancers Study, a large population-based case-control study of sarcoma conducted among U.S. men aged 30 to 60 in 1984 to 1988, we explored the occupational risk factors for soft-tissue sarcoma subtypes and skeletal sarcoma. The analysis included 251 living sarcoma cases (48 dermatofibrosarcoma protuberans, 32 malignant fibrohistiocytic sarcoma, 67 leiomyosarcoma, 53 liposarcoma, and 51 skeletal sarcoma) and 1908 living controls. Exact conditional logistic regression models suggested patterns of subtype specificity for occupational exposures. Self-reported herbicide use was associated with malignant fibrohistiocytic sarcoma (OR = 2.9, 95% CI = 1.1-7.3). We found elevated risks for chlorophenol exposure and cutting oil exposure and malignant fibrohistiocytic sarcoma and leiomyosarcoma. We found no occupational risk factor for liposarcoma. Polytomous regression models identified different odds ratios across subtypes for plywood exposure and exposure to wood and saw dust. Although exploratory, this analysis suggests that occupational risk factors for sarcoma are not uniform across subtypes.

Adult↗
PubMed1990

[Clinico-pathological study of primary malignant chest wall tumors].

Clinicopathological studies of primary malignant chest wall tumor on 16 cases operated between 1962 and 1988 were made. Of 9 osteogenic sarcomas, 8 cases were chondrosarcoma and 1 case was Ewing's sarcoma. Of 7 soft part sarcomas, 2 cases were fibrosarcoma, liposarcoma and neurogenic sarcoma respectively, and one case was hemangiosarcoma. Most of the cases had symptoms, such as chest mass and/or chest pain comprehend symptoms for more than one year. The intrathoracic growth of tumor is common, especially in osteogenic sarcoma. The maximum size of tumor was 8.2 cm in a mean diameter. Preoperative histological diagnosis is difficult to make even though various radiologic diagnosis or pathological technique as biopsy or cytology were assessed. And true rate of preoperative diagnosis is limited only 43.8%. Wide resection combined with the tissue distant more than 3 cm length from tumor is recommended and 6 cases underwent combined resection of diaphragm, pericardium or lung. 3 cases underwent chest wall reconstruction using the Marlex mesh and 10 cases were able to direct closure, in 13 cases with ribs resection. The 5 year survival rate of endurable cases was 62.2%, and that of soft tissue sarcoma (68.6%) is better than that of osteogenic sarcoma (41.7%). The recurrent or metastatic rate in high, 7 cases (43.8%), but reoperation was added for 5 cases of local recurrence or for a case of lung metastasis. 5 year survival rate of cases with recurrence or metastases is relatively good, 46.8%, especially excellent in 4 cases with recurred lesions after more than 2 years of tumor free interval.

Adult↗