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PubMed1981

Soft part sarcomas of the head and neck.

During the 28-yr-period from 1949-1977, 285 patients with sarcoma of the head and neck region were seen and treated at Memorial Cancer Center. Two hundred forty-two cases were determinate, and none was lost to follow-up study. The determinate and absolute 5-yr cure rate was 32%. More than 50% of the cases were seen in children (8 cases were congenital) and were almost universally anaplastic and prone to generalized metastasis with fatal outcome. Combined modalities of radical surgery, postoperative radiotherapy, and chemotherapy over a prolonged period have markedly altered the prognosis of these tumors. Soft part sarcomas are an unusual group of tumors with a common fibrosarcomatous background. The qualified pathologist can identify quite accurately the different histologic entities and their varying grade, a point of great significance as to clinical course and treatment.

Adolescent↗
PubMed1993

Liposarcoma.

Liposarcoma is second in frequency only to malignant fibrous histiocytoma among the soft-tissue sarcomas. It occurs almost exclusively in adults and is found most often in the thigh or retroperitoneum. It rarely arises from a lipoma and does not occur in the subcutaneous tissues. Liposarcomas are divided into histologic subtypes with different microscopic appearances and slightly different behavior characteristics. Surgical resection with a wide surgical margin is the treatment of choice. When amputation is required to obtain an adequate surgical margin, local irradiation can be used as an adjuvant and a limb-sparing operation can be done. Currently, no evidence exists that adjuvant chemotherapy is indicated for patients with liposarcoma, although numerous studies are being done to investigate its use. As is the case for all sarcomas, the lung is the most common site of metastasis; however, liposarcoma has an unusual propensity to metastasize to the retroperitoneum, mediastinum, and bone.

Adult↗
PubMed2000

[Liposarcoma in the area of the head-neck].

Liposarcoma is the second most common soft tissue sarcoma occurring in adults, though it rarely involves the head and neck region. Fewer than 100 such cases have been described in the literature. We present two additional cases and a review of the literature. Prognosis is mainly determined by histologic grade. A high incidence of local recurrence is typical of all subtypes. Complete surgical excision is the most effective treatment. In the head and neck region the extent of the excision is limited by the proximity of neurovascular structures. The benefits of adjuvant radiotherapy in head and neck liposarcoma remain uncertain. Studies on liposarcoma at other sites indicate that addition of radiotherapy to surgery may result in longer local recurrence-free survival, though without improvement of overall survival.

Adult↗
PubMedDate not supplied

Intrathoracic pleomorphic liposarcoma in a child: study of an unusual case.

Primary intrathoracic liposarcoma is an extremely rare neoplasm in the pediatric age group. We report an unusual case of a 3-year-old boy who presented with a large intrathoracic tumor arising from the right chest wall. Biopsy showed a pleomorphic sarcoma with histological, immunohistochemical, and ultrastructural features of pleomorphic liposarcoma. The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.

Child, Preschool↗
PubMed1999

H-ras and K-ras mutations in soft tissue sarcoma: comparative studies of sarcomas from Korean and American patients.

BACKGROUND: The authors' recent investigation of Korean patients with sarcoma has suggested that ras gene activation may play a role in oncogenesis. The authors attempted to extend the mutation analysis to sarcomas in American patients to determine whether there were racial or geographic factors relevant to the initiation or progression of sarcoma. METHODS: H-ras and K-ras genes were examined in sarcomas obtained from patients in the midwestern U. S. using the polymerase chain reaction technique and direct automated sequencing analysis. Tumors studied included 29 malignant fibrous histiocytomas, 7 liposarcomas, 5 rhabdomyosarcomas, and 9 leiomyosarcomas. RESULTS: Of the 50 sarcomas evaluated, only 1 (2%) definable mutation was found; a GGC to AGC transition at codon 12 of H-ras was found in a rhabdomyosarcoma. None of the patients had a K-ras mutation. The rates of incidence of ras point mutations in these samples were much lower (H-ras: 2%; 95% confidence interval [95% CI], 0-11.5% and K-ras: 0%) than described for both genes in Korean studies (H-ras: 16%; 95% CI, 5.2-26.8% and K-ras: 44%; 95% CI, 29.5-58.5%). CONCLUSIONS: Although the reason for this discrepancy is not clear, there were no major differences found in histology or clinical stages. Based on this study of 50 sarcoma samples from American patients and the authors' previous study of 45 Korean tumor samples, the authors conclude that differing genetic and/or environmental mechanisms can affect sarcoma development or progression. Mutation of the H-ras and K-ras genes appears to be uncommon in sarcomas occurring in American patients, suggesting that the activation by point mutations of the H-ras and K-ras genes does not play a significant role in the pathogenesis or progression of sarcoma in these patients.

Adolescent↗
PubMed1980

Effect of desacetyl vinblastine amide (DVA) against human sarcomas heterotransplanted in nude mice.

The efficacy of desacetyl vinblastine amide (DVA) was tested against five human sarcomas heterotransplanted into nude mice. Marked antitumor effect was found against a lipoblastic liposarcoma, including complete regressions of tumor in some animals. A lesser, though statistically significant, antitumor effect was observed with a malignant schwannoma. No antitumor activity was seen against a leiomyosarcoma, epithelioid sarcoma, or Ewing's sarcoma. We conclude that DVA deserves study in the treatment of human patients with malignant sarcomas.

Animals↗
PubMed1976

[Therapeutic use of carminomycin in soft tissue sarcomas].

A total of 39 patients with sarcoma of the soft tissues were treated with carminomycin administered intravenously in doses of 10-15 mg twice a week, the total dose being 60-70 mg. The intervals between the courses were 1 month. The objective effect was observed in 17 out of 39 patients, i. e. 43.6%. The tumor decrease was registered in 6 out of 15 patients with leuomyosarcoma, in 3 out of 6 patients with synovial sarcoma, in 2 out of 4 patients with robdomyosarcoma, in 1 out of 4 patients with angiosarcoma, in 1 out of 3 patients with fibrosarcoma, in 1 patient with liposarcoma and in 3 out of 5 patients with stromal sarcoma of the uterus. A necessity for repeated treatment courses in spite of a positive effect or absence of the disease progress is underlined.

Aged↗
PubMedDate not supplied

[Myxoid liposarcoma of the retroperitoneum. Presentation of a clinical case].

Of all the soft tissue sarcomas, those localised in the retroperitoneum represent a particular challenge to the surgeon. Diagnosis is often late, when the tumour has already reached considerable proportions, creating particular problems for its removal. Liposarcomas are the most common histological form of retroperitoneal tumour. The authors report the case of a patient with retroperitoneal myxoid liposarcoma and use their analysis as the starting point for a review of the literature focused on the diagnosis and treatment of retroperitoneal tumours. They underline that modern imaging techniques (Eco, CT, NMR) are essential during the preoperative stage for a complete and detailed study of the neoplasm, as well as during follow-up. With regard to treatment, surgery is still the most effective approach in cases where this is feasible. The current trend towards aggressive surgery is backed by the improved results in terms of survival. An improved prognosis for patients with retroperitoneal tumours is linked to an early diagnosis and the development of more efficacious complementary therapies.

Humans↗
PubMed1986

[Treatment of soft tissue sarcomas of the leg].

An analysis of data of 548 patients with different diseases of soft tissues of lower extremities after using a complex of diagnostic measures (clinical, radionuclide data, thermography, puncture, biopsy) has shown that the number of erroneous conclusions may be minimized to 2%. Modern advances of plastic surgery can widen indications for preserving operations in malignant tumors of soft tissues of lower extremities without prejudice to the main principle of oncology concerning its fascial character. Combined treatment can also improve the results.

Female↗
PubMed1997

Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation.

We studied 155 cases of dedifferentiated liposarcoma to define its clinicopathologic features and behavior, in particular how the extent and grade of dedifferentiation affected outcome. Tumors occurred in late adult life (median, 61.5 years: range, 21-92 years), most commonly in the retroperitoneum (106 cases), extremities and trunk (32 cases), and scrotum/spermatic cord (13 cases). The majority of dedifferentiated liposarcomas presented as de novo lesions, whereas the remainder developed as a late complication of a preexisting well-differentiated liposarcoma after an average interval of 7.7 years. At the time of presentation, most of the dedifferentiated liposarcomas displayed extensive areas of high-grade dedifferentiation resembling malignant fibrous histiocytoma or high-grade fibrosarcoma, whereas a minority contained only areas of low-grade dedifferentiation resembling fibromatosis or well-differentiated fibrosarcoma. Divergent myosarcomatous or osteosarcomatous differentiation was observed focally in six cases. The behavior of dedifferentiated liposarcomas was that of a high-grade sarcoma with a local recurrence rate of 41%, a metastatic rate of 17%, and disease-related mortality of 28%. The most important prognostic factor was location in that retroperitoneal tumors had significantly worse survival than those in other sites. Tumors were divided into those having less than or those with more than 25% dedifferentiation, and dedifferentiated zones were classified into low grade or high grade. Neither low-grade dedifferentiation nor a low percentage of dedifferentiation was associated with an improved outcome for the tumors examined in this study; however, in no cases was the absolute size of the dedifferentiated focus <2 cm. Therefore, this study did not determine a minimum, or threshold, amount of dedifferentiation below which outcome was more favorable. The behavior of liposarcomas in which the dedifferentiated component was entirely low grade was more similar to that of traditional dedifferentiated liposarcoma than to that of well-dedifferentiated liposarcoma. Our study supports the expansion of the definition of dedifferentiated liposarcoma to include tumors with low-grade dedifferentiation and also suggests that low-grade dedifferentiation represents a precursor lesion of high-grade dedifferentiation.

Adult↗
PubMed1976

Transplantable sarcomas induced by 3-methylcholanthrene in inbred guinea pigs of JY-1 and Hartley/F strains.

Inbred strains of guinea pigs, JY-1 and Hartley/F, established in this Institute, were injected subcutaneously with 3-methylcholanthrene. During 1972 to 1974, 15 solid tumors (7 fibrosarcomas, 6 liposarcomas, and 2 atheroma-like tumors) have been induced in 12 animals out of 30 employed. Among the 15 tumors, 1 fibrosarcoma induced in JY-1 and 2 liposarcomas and 1 fibrosarcoma induced in Hartley/F were transplantable and established as the syngeneic lines named J4, H10, H12, and H9A, respectively. In addition, a transplant of J4 into the Hartley/F strain animal was established as an allogeneic subline and named JH4. Pathological and biological characteristics of these tumors are described and differences between these tumors and line 10 hepatoma, established by Rapp et al. in strain-2 guinea pig, are discussed.

Animals↗
PubMed1975

Distinctive banded marker chromosomes of human tumor cell lines.

Nine human tumor cell lines (five breast carcinomas and four sarcomas) have been studied and each revealed groups of distinctive banded marker chromosomes which can serve to identify them and aid in monitoring cell line specificity. This was possible neither by conventional karyology in terms of numbers and morphology of chromosomes nor by glucose-6-phosphate-dehydrogenase mobility which was type B for all cultures. The significance of the clonal nature of the cell lines is discussed.

Breast Neoplasms↗
PubMed1997

Chromosomal translocations in human soft tissue sarcomas by interphase fluorescence in situ hybridization.

In soft tissue sarcomas, clonal rearrangement of chromosomes has been shown by cytogenetic analysis to be unique and specific for tumor types. The development of fluorescence in situ hybridization (FISH) has allowed detection of chromosomal rearrangements in the interphase nuclei isolated from paraffin-embedded tissues. Three kinds of translocations in the interphase nuclei that were isolated from 47 cases of soft tissue sarcomas were examined by FISH with chromosome-specific DNA probes of centromeric and total probes. Of 47 soft tissue sarcomas 42 (89.4%) revealed tumor-specific translocations by retrospective cytogenetic analysis. Translocation t(X;18) was detected in 25/28 synovial sarcomas; translocation t(11;22) in 5/6 Ewing's sarcomas and primitive neuroectodermal tumors (PNET); and translocation t(12;16) was found in 12/13 liposarcomas, including 10 myxoid and two round cell types as clonal chromosomal aberrations specific for both subtypes. Based on the cytogenetic analysis, Ewing's sarcoma is related closely with PNET as shown by MIC2-protein reactivity. Other cytogenetic findings of translocation t(12;16) indicate that round cell liposarcomas share chromosomal changes with myxoid liposarcomas, and further suggest that both tumor subtypes of liposarcoma may possess common precursor cells. FISH is a useful aid in determining the tumor type of soft tissue sarcomas with regard to histogenetic origin.

Adolescent↗
PubMed1987

Malignant fibrous histiocytoma. Evidence of perivascular mesenchymal cell origin immunocytochemical studies with monoclonal anti-MFH antibodies.

Using whole cell antigens prepared from the established lines of human malignant fibrous histiocytoma (MFH), the authors have generated two different monoclonal antibodies (FU3 and FU4) by a mouse hybridoma technique. By indirect immunoperoxidase in frozen tissue sections, FU3 and FU4 revealed strong staining of perivascular mesenchymal cells and fibroblasts. In the spleen FU3 stained perivascular cells of the ellipsoids and the marginal zone of the lymph follicles. Macrophages in granulation tissues as well as monocytes and other blood cells in normal peripheral blood were uniformly negative for the antigen detected by FU3. Among various soft-tissue tumors, MFH and liposarcoma reacted strongly with FU3 and FU4, but synovial sarcoma revealed no reaction with either of the antibodies. Immunoelectron-microscopic studies demonstrated positive reactions with FU3 and FU4 on the surface of MFH cell membrane, which suggests that these antibodies recognized cell surface antigens. In conclusion, MFH shares antigenicity with perivascular mesenchymal cells and fibroblasts as well as liposarcoma. MFH and liposarcoma may have a common origin from the perivascular mesenchymal cells that are supposed to have a potential for multidirectional differentiation.

Animals↗
PubMed2004

Liposarcoma circumscriptum (lipoma-like) of the tongue: report of a case.

Liposarcoma, first described by Virchow in 1857, is the second most frequent sarcoma of soft tissues, although it is rare both in the head and neck and the oral cavity. Intra-orally, liposarcoma has been reported in the jawbones (particularly the maxilla) and the soft tissues -- mainly the cheek and floor of mouth, but it is rare in the tongue. A case of well-differentiated, superficial liposarcoma circumscriptum of the tongue is reported. The authors underline the difficulties in the clinical and histopathological diagnosis, as this tumour may be confused with lipoma. In view of the indolent behaviour of this tumour type, local recurrence or metastasis rarely occur; consequently, conservative surgical therapy is advised without adjuvant chemo-radio therapy.

Diagnosis, Differential↗
PubMed2000

An analysis of 78 breast sarcoma patients without distant metastases at presentation.

PURPOSE: A retrospective review of a single cancer center experience was undertaken to identify clinical or treatment prognostic factors for these unusual tumors, to allow for a recommendation regarding management. METHODS AND MATERIALS: The charts of 76 women and 2 men with breast sarcoma and without distant metastases at presentation registered from 1958 to 1990 were reviewed. Pathology was centrally reviewed in 54 cases. Histology, tumor size, grade, nodal status, age, menopausal status, history of benign breast disease, extent of surgery, resection margins, and radiation dose were each examined as potential prognostic factors by univariate analysis. To allow an analysis of radiation dose, total dose was normalized to a daily fraction size of 2 Gy. RESULTS: The median age at diagnosis was 50.5 years (13-82 years). The pathologic diagnosis was found to be malignant cystosarcoma phyllodes in 32 patients, with the remainder being stromal sarcoma (14), angiosarcoma (8), fibrosarcoma (7), carcinosarcoma (5), liposarcoma (4), other (8). Eighteen patients had grade I or II tumors, 43 had grade III or IV, and 18 were not evaluable. The 5- and 10-year actuarial rates for all 78 patients were 57% and 48% for cause-specific survival (CSS), and 47% and 42% for the relapse-free rates (RFR), respectively. The local relapse-free rate (LRFR) was 75% at both 5 and 10 years. The 5-year CSS for grade I or II tumors was 84% versus 55% for grade III or IV tumors (p = 0.01). Conservative surgery versus mastectomy did not lead to statistically significant different outcomes for CSS, RFR, or LRFR. The comparison of positive versus negative margins showed a 5-year LRFR of 33% versus 80% (p = 0.009). Pairwise comparisons of the 5-year CSS of 91% for > 48 Gy versus either 50% for < or = 48 Gy or 50% for no radiation showed p-values of 0.03 and 0.06, respectively. CONCLUSION: The authors propose that if negative surgical margins can be achieved, breast sarcoma should be managed by conservative surgery with postoperative irradiation to a microscopic tumoricidal dose (50 Gy) to the whole beast, and at least 60 Gy to the tumor bed. The decision to treat should be preceded by a preoperative multidisciplinary assessment. It is also recommended that an axillary lymph node dissection is not indicated, with the possible exception of patients with carcinosarcoma.

Adolescent↗