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PubMed1986

Soft tissue sarcomas of the distal extremities.

Multimodality therapy and limb salvage procedures constitute a significant advance in the treatment of soft tissue sarcoma of the extremity. The applicability of these procedures to soft tissue sarcoma of the distal extremities was evaluated in a retrospective study of 159 such cases treated during a 10-year period. Thirty-seven patients were treated by operation alone, 57 had operation and adjuvant chemotherapy, and 65 had multimodal therapy. The distal extremities are unusual sites for soft tissue sarcomas and accounted for only 20% of our patients with sarcoma. The majority of patients were younger than 50 years and the sex distribution was approximately equal. The major histologic types were synovial cell sarcoma (18.2%), fibrosarcoma (15%), liposarcoma (13.8%), and rhabdomyosarcoma (13.8%). The 5-year survival rate was 72% with multimodality therapy, 72% for operation and chemotherapy, and 51% for operation alone. The local recurrence rate was 13% with operation alone, 9% with operation and adjuvant chemotherapy, and 12% with multimodality therapy. Based on our review, wide local excision and adjuvant chemotherapy proved to be as effective as multimodal therapy.

Adult↗
PubMed1977

Growing human sarcomas in culture.

Efforts to establish human soft tissue and bone sarcomas in long-term cultures are described. Of 49 attempts 22 (45%) were successful, yielding cells that proliferated in vitro for 6 months or longer. Infrequent medium changes, maintenance of pH in physiologic range and avoidance of early trypsinization all appear to be important for survival of explanted cells and their initial growth under culture conditions. Cytologic criteria can be used to confirm that cells in established lines derive from malignant mesenchymal cells present in the tumors of origin.

Animals↗
PubMed1995

Recent developments in soft tissue tumors.

This article reviews the clinicopathological features of several recently described soft tissue tumours, namely ossifying fibromyxoid tumour, angiomyofibroblastoma, epithelioid angiosarcoma, retiform haemangioendothelioma, intra-abdominal desmoplastic small cell tumour, spindle cell liposarcoma and low grade fibromyxoid sarcoma. Conceptual changes are also discussed. These include the relationship between Ewing's sarcoma and peripheral primitive neuroectodermal tumour, the proposed use of the term atypical lipoma for a subset of well differentiated liposarcomas, and the occurrence at a wide variety of sites of inflammatory myofibroblastic lesions of uncertain biological potential. In addition, advances in the study of soft tissue lesions at the molecular and cytogenetic levels are outlined, with particular emphasis on the recent identification of tumour-specific karyotypic abnormalities in a wide variety of sarcomas.

Humans↗
PubMed1997

Interphase cytogenetic analysis of myxoid soft tissue tumors by fluorescence in situ hybridization and DNA flow cytometry using paraffin-embedded tissue.

BACKGROUND: Myxoid liposarcoma is one of the major myxoid malignancies of soft tissue and its histologic differentiation from other myxoid tumors is sometimes difficult. Recent cytogenetic analysis revealed that a characteristic reciprocal chromosomal translocation of t(12;16)(q13;p11) occurs in myxoid liposarcomas. In this study, retrospective cytogenetic analysis by means of fluorescence in situ hybridization (FISH) and DNA flow cytometry were performed on free nuclei isolated from paraffin embedded myxoid liposarcoma cells, and they were compared with myxoid malignant fibrous histiocytomas (MFHs), low grade fibromyxoid sarcomas, and intramuscular myxomas. METHODS: Nine myxoid liposarcomas, five myxoid MFHs, two low grade fibromyxoid sarcomas, and four intramuscular myxomas were examined. Chromosomal aberrations in chromosomes 12 and 16 were investigated by FISH, using both centromeric DNA probes and whole chromosome painting probes. Cellular DNA contents were determined by flow cytometry. RESULTS: All nine myxoid liposarcomas were shown to be diploid by DNA flow cytometry and exhibited the translocation t(12; 16), as shown by FISH. Four of the five myxoid MFHs exhibited aneuploidy, as shown by flow cytometry, and remarkable numeric aberrations of chromosomes 12 and 16. No abnormal DNA content or chromosomal aberrations were identified in the two low grade fibromyxoid sarcomas or the four intramuscular myxomas. CONCLUSIONS: FISH analysis performed on paraffin embedded materials can be used to differentiate myxoid liposarcomas from other myxoid soft tissue tumors.

Adult↗
PubMedDate not supplied

Multifactorial analysis of survival in primary extremity liposarcoma.

The treatment of extremity soft tissue sarcoma is now directed at limb preservation with the addition of various adjuvant therapies to improve treatment results. To achieve this goal, a knowledge of prognostic factors for extremity soft tissue sarcoma becomes increasingly critical. The object of this study was to analyze prognostic factors for survival in patients with extremity liposarcoma. Eighty-three patients with primary localized extremity liposarcoma, admitted from 1968 to 1978, were retrospectively reviewed. Surgical resection was the primary mode of treatment. Eleven prognostic factors were analyzed. Tumor factors included: histologic subtype, tumor grade, size, depth, invasion of vital structures, and site; operative factors included: type of operation, and surgical margins; and patient factors included: symptoms, age at diagnosis, and sex. Kaplan-Meier survival curves, and univariate and stratified log-rank tests of association were performed. Independent factors for predicting survival were identified using the Cox model stepwise regression technique. In univariate analysis of the entire group of patients, 5 factors were significant for tumor mortality: tumor grade (p = 0.00005), histologic subtype (p = 0.00025), tumor size greater than or equal to 5 cm (p = 0.005), type of surgery/margin (p = 0.0001), and invasion of vital structures (p = 0.008). When associations among all factors were analyzed, it was found that many factors were interdependent. The independent prognosticators were, therefore, determined according to the Cox model technique. For the multivariate analysis, well-differentiated and lipoblastic liposarcomas were excluded because of lack of survival variation within each group. The former group had no tumor-related deaths and the latter group showed 80% tumor mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance↗
PubMed2000

Molecular diagnosis and gene therapy in musculoskeletal tumors.

Significant progress has occurred in the molecular analyses of bone and soft-tissue tumors, and genetic studies have led to the development of important new diagnostic tools for the clinical management of patients with sarcomas. The detection of fusion genes induced by tumor-specific translocations, such as EWS-FLI1 in Ewing's sarcoma, SYT-SSX in synovial sarcoma, and CHOP-FUS in myxoid liposarcoma, is becoming significant for clinical diagnosis, because these sarcomas are often indistinguishable from other bone and soft-tissue tumors. Gene therapies with several gene transfer systems have been employed for some incurable cancers. It has been demonstrated that a Herpes simplex virus thymidine kinase (HSV-tk) gene can convert certain nucleoside analogs, such as ganciclovir, which disrupt DNA synthesis and are toxic to nucleosides. Human chondrosarcoma cells transduced with the HSV-tk gene were more sensitive to the cytotoxity of ganciclovir than non-transduced cells. Coculture of chondrosarcoma cells with and without the HSV-tk gene showed a bystander effect. The local injection of gene transduced cells into the chondrosarcoma implanted in nude mice markedly reduced tumor size after the administration of ganciclovir. These results suggested the possibility of gene therapy for chondrosarcoma.

Animals↗
PubMed2004

Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations.

The review included 163 cases of paraneoplastic pemphigus (PNP) reported between 1990 and 2003, including a new unique case of PNP associated with occult breast cancer and an ovarian cyst of borderline malignancy. Hematologic-related neoplasms or disorders were associated with 84% of the cases, with non-Hodgkin lymphoma (38.6%) as the most frequent, followed by chronic lymphocytic leukemia (18.4%) and Castleman's disease (18.4%). The non-hematologic neoplasms comprised 16% of all cases: epithelial origin-carcinoma (8.6%), mesenchymal origin-sarcoma (6.2%), and malignant melanoma (0.6%). Carcinoma cases comprised 58% of the non-hematologic neoplasms. Carcinoma cases (n = 14) consisted of adenocarcinoma (n = 7), squamous cell carcinoma (n = 2), multiple skin tumors probably basal cell carcinoma (n = 1), and bronchogenic carcinoma (n = 1). Of the 10 (6.2%) sarcoma cases, there was one case each of leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, poorly differentiated sarcoma, reticulum cell sarcoma, dendritic cell sarcoma and inflammatory myofibroblastic tumor. The oral mucosa was involved in all of cases. Isolated oral ulcerations were the first sign in 45% of the cases. Diffuse and persistent oral ulcerations with a progressive course could be a sign of malignancy, either recognized or occult. In the absence of a clear diagnosis, malignancy should be suspected and extensive work-up performed. The full spectrum of signs of PNP may not be present initially. Repeated biopsies, direct and indirect immunofluorescence as well as screening indirect immunofluorescence on murine bladder are required for diagnosis. Clinicians should be highly suspicious when signs and symptoms suggestive of PNP are present in cancer patients, of hematologic and non-hematologic origin.

Adenocarcinoma↗
PubMedDate not supplied

Liposarcoma of the breast: a case report and review of the literature.

Sarcoma of the breast constitutes <1% of all malignant breast tumors, and liposarcoma of the breast represents 3-24% of the primary breast sarcomas. We report a case of a 74-year-old woman presenting with liposarcoma of the left breast. The patient presented with a fibrous and hard mass measuring approximately 10 x 5 cm in diameter in the upper part of the left breast. Aspiration breast cytology showed inflammatory changes and had no malignant element. Differential diagnosis with sonography, computed tomography, and magnetic resonance imaging was not successful. A wide excision was performed with a 2-cm margin of healthy tissue. The tumor was diagnosed histologically as a well-differentiated liposarcoma (sclerosing type). The patient was discharged and her recovery was uneventful. We report a case of liposarcoma of the breast and discuss this rare malignant tumor and other types of sarcoma of the breast in light of the previously published literature.

Aged↗
PubMed1990

Soft tissue sarcoma with additional anaplastic components. A clinicopathologic and immunohistochemical study of 27 cases.

This clinicopathologic study concerns 27 cases of "dedifferentiated" soft tissue sarcoma (DSTS), including 14 liposarcomas, six leiomyosarcomas, five chondrosarcomas, and two rhabdomyosarcomas. In addition, the authors conducted an immunohistochemical survey of 23 cases and an electron microscopic examination of three. The findings were compared with observations of 32 cases of de novo malignant fibrous histiocytoma (MFH). All tumors contained additional distinct anaplastic portions indistinguishable from MFH under conventional light microscopy, ultrastructurally, and in cases of immunoreactivity for alpha-1-antichymotrypsin and alpha-1-antitrypsin and on lectin histochemical findings for ricinus communis agglutinin and concanavalin agglutinin. The desmin reactivity present in anaplastic portions of 14 DSTS and in eight de novo MFH is taken to mean that myofibroblasts are present in these tumors. The anaplastic components of DSTS are presumed to represent the proliferation of another clone of undifferentiated mesenchymal cells that fail to differentiate along any specific lineage other than fibroblast-like cells, histiocyte-like cells, and myofibroblasts. Nineteen patients died of tumor and four are alive and well 1.6, 1.7, 2.1, and 5.2 years after the initial treatment, respectively.

Adolescent↗
PubMed2002

Karnofsky performance score as a predictor of survival in soft tissue sarcoma.

Forty-six consecutive patients with nonmetastatic, large, high-grade soft tissue sarcomas were treated with surgical resection and radiation therapy but no adjuvant chemotherapy. Clinical and radiographic followup ranged from 36 to 90 months (mean, 50 months). One patient died of unrelated causes 10 months after the completion of radiation and surgery, leaving the oncologic outcome of 45 patients to be evaluated. Twenty-seven of 45 patients (60%) survived with an average followup of 50 months. The limb salvage rate was 91% and local control was obtained in 43 of 45 patients (96%). During the time of the study, 73% of patients could have been offered chemotherapy. However, only 53% of patients who died of their disease would have been candidates for chemotherapy trials because of medical comorbidities. More importantly, patients with a Karnofsky performance score of 80 or more had a significantly better survival rate than patients with a Karnofsky performance score less than 80 without the use of chemotherapy. Current chemotherapy trials that exclude patients because of medical comorbidities are introducing bias toward improved survival that may not be attributable to the chemotherapy. If a real survival benefit of adjuvant chemotherapy exists, it would be most evident in the largest (> 15 cm) high-grade tumors. Aggressive metastasectomy seems to be most beneficial for patients with isolated nodules.

Comorbidity↗
PubMed1994

Cytogenetics and experimental models of sarcomas.

Many sarcomas contain characteristic chromosome translocations that result in the fusion of genes from two different chromosomes. This review focuses on the translocated genes that have been identified recently in Ewing's sarcoma, peripheral primitive neuroectodermal tumor, clear cell sarcoma, alveolar rhabdomyosarcoma, and myxoid liposarcoma. Each of these fusion genes encodes a DNA transcription factor that presumably regulates the function of other genes. Thus, a common mechanism of oncogenesis has been defined in histologically disparate varieties of sarcoma.

Adult↗
PubMed1987

Renal sarcoma and sarcomatoid renal cell carcinoma: CT and angiographic features.

Computed tomographic (CT) and angiographic findings in 14 patients (eight men, six women, aged 30-72 years) with sarcomatoid renal cell carcinoma (SRCC) and various types of renal sarcoma are described. There were four patients with SRCC; three, leiomyosarcoma; two, liposarcoma; two, fibrosarcoma; and one each of unclassified renal sarcoma, clear cell sarcoma, and malignant fibrous histiocytoma. The most frequent presenting symptom was an abdominal mass or pain. All 14 patients underwent CT, and 11 underwent selective renal angiography. The diagnosis of renal sarcoma should be suspected when CT findings suggest that the tumor arises from the renal capsule or renal sinus and when the tumor is hypovascular or avascular on angiograms. The characteristic negative attenuation values for liposarcomas permit a specific diagnosis. Sarcomas that originate in the renal parenchyma and SRCC cannot be easily differentiated from renal cell carcinoma; however, renal sarcomas do not appear to have a propensity for extension into the renal vein or the inferior vena cava.

Adult↗
PubMed1989

Sonography of unusual extratesticular lesions.

Sonograms of seven patients with extratesticular lesions are presented because of their relatively rare occurrence or unusual sonographic pattern. The sonographic appearance of a malignant extratesticular liposarcoma is described. Infected sebaceous glands and slow-growing malignant tumors of the spermatic cord are demonstrated as possible problems in differential diagnosis. Extratesticular septated cysts or fluid collections surrounding the spermatic cord are documented as complications in hemophilia and acute pancreatitis. These entities are presented because of their differential diagnostic challenge compared with more common disorders such as infected hydroceles and hernias.

Adult↗
PubMed1995

Identification of two distinct chromosome 12-derived amplification units in neuroblastoma cell line NGP.

The neuroblastoma cell line NGP contains two homogeneously staining regions (hsr). One of these hsrs contains MYCN sequences. Reverse painting experiments demonstrated that the second HSR consisted of two chromosome 12-derived amplification units, located at 12q14-15 and 12q24. Southern blot and fluorescence in situ hybridization (FISH) analysis showed amplification of genes located at 12q14-15: SAS, MDM2, and CDK4, GLI, CHOP, CDK2, and A2MR were found not to be amplified. FISH further demonstrated amplification of RSN, a gene located at 12q24. The finding of two distinct chromosome 12 amplification units in a neuroblastoma cell line NGP is reminiscent of recent findings in well-differentiated liposarcoma (WDLPS) and other sarcomas. The second amplification unit on chromosome 12 in NGP is located more distal (12q24) than the one observed in WDLPS (12q21). The mechanism and biologic significance of this amplification process in neuroblastoma and WDLPS remain to be elucidated.

Chromosomes, Human, Pair 12↗
PubMed2002

Nup88 (karyoporin) in human malignant neoplasms and dysplasias: correlations of immunostaining of tissue sections, cytologic smears, and immunoblot analysis.

Nuclear pore complexes (NPCs) are elaborate macromolecular structures that regulate the bidirectional nucleocytoplasmic traffic system. In vertebrate cells, NPCs include a family of 50 to 100 proteins termed nucleoporins (Nups). The 88-kD Nup has been found to be linked in a dynamic subcomplex with the oncogenic CAN/Nup214. Applying a polyclonal antiserum to Nup88 on paraffin sections, we found that it immunoreacts with numerous malignant neoplasms. All carcinomas reacted irrespective of site, type, or degree of differentiation; often, high-grade carcinomas stained more strongly and extensively. Some sarcomas (e.g., fibrosarcomas, leiomyosarcomas, liposarcomas, and rhabdomyosarcomas) reacted intensely; melanomas, gliomas, mesotheliomas, and malignant lymphomas also stained. In situ carcinomas of the colon, stomach, breast, and prostate stained convincingly, as did in situ melanomas; some samples of fetal tissues also reacted. Cytologic smears of some of the aforementioned tumors also stained. In selected samples, enhanced immunostaining of tissue sections and cytologic smears correlated strongly and consistently with immunoblot data. Immunoblots of the same tumors with antibodies to 2 other Nups (Nup214 and Nup153) showed no comparable enhancement. Therefore, it seems that in some malignant tumors, Nup88 overexpression is not parallelled by an overexpression of other Nups. Benign tumors, hyperplasias, and normal tissues showed weak and sporadic staining or absence of staining; immunoblots of the same samples yielded weak signals. Occasional highly proliferative hyperplastic-reactive processes showed focal staining. Thus, our correlative histologic, cytologic, and molecular data indicate that Nup88 may be viewed as a potentially useful, broadly based histodiagnostic and molecular marker of many malignancies and premalignant dysplasias, and further suggest that in some malignant tumors, Nup88 may be selectively overexpressed as compared with other Nups. Thus, we propose that Nup88 be designated as karyoporin.

Biomarkers, Tumor↗
PubMed2000

Scintigraphic imaging of sarcomatous tumors with [(111)In-DTPA-phe-1]-octreotide.

The objective of the present study was to investigate the efficacy of (111)In-DTPA-octreotide (OC) for in vivo scintigraphic imaging of these relatively uncommon tumors. Thirteen patients (9 males, 4 females, mean age 59 years) with known sarcomatous lesions were studied. All patients had known lesions as demonstrated by previous investigation with other modalities, e.g. CAT, MRI. Following intravenous injection of 10 microg of OC labeled with 2.8-4.2 mCi (111)In, planar imaging was done at 6 +/- 1 and 22 +/- 2 h, respectively. Histologic verification was obtained in all cases, either from fine needle aspiration or from surgically removed tissue. Positive imaging was observed in 12/13 cases (92.3%). One scan was false-negative (7.7%). Occult lesions were demonstrated in two patients. The histologic typing and the scintigraphy results were: fibrosarcoma (1+/1), embryonic rhabdomyosarcoma (1+/1), leiomyosarcomas (3+/3), liposarcomas (2+/2), uterine sarcomas (2+/2), HIV (-) Kaposi sarcoma (1+/1), osteosarcoma (1+/1), chondrosarcoma (1-/1) and neurogenous sarcoma (1+/1). OC appears to have properties that lead to a new indication for its use. Other possible applications relate to the therapeutic use of octreotide either unlabeled or labeled with a beta-emitting radionuclide, as well as its use in radioimmunoguided surgery. Regarding the latter, our preliminary results are encouraging.

Female↗