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PubMed2002

Aberrant immunohistochemical expression in nonrhabdomyosarcoma soft tissue sarcomas of infancy: retrospective review of clinical material.

Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma.

Humans↗
PubMed1978

Chemotherapy of sarcoma. A comparison of three regimens.

Three regimens have been used in the chemotherapeutic management of 75 adults with sarcoma. Methotrexate alone was used in 41 cases, a five-drug regimen was given in 12 cases, and adriamycin, T.I.C.-mustard, and methotrexate were given to 22 patients. Remissions, sometimes prolonged, were achieved with each regimen, and none was obviously better than the others. Because of the higher response-rate of primary or locally recurrent tumours it is suggested that sensitivity to drugs should be assessed earlier in the disease. It is proposed that a randomised study of methotrexate versus combination chemotherapy should be started in particular sarcoma subgroups, such as leiomyosarcoma and fibrosarcoma.

Adult↗
PubMed1986

Primary intraspinal fibrosarcoma.

Fibrosarcomas of dura mater are very rare tumors representing only 0.5% of all central nervous system sarcomas. A higher incidence of dural fibrosarcomas has been reported in patients with neurofibromatosis. We present a case of spinal dura mater fibrosarcoma in a patient without neurofibromatosis. The diagnostic and therapeutic strategy in the clinical management is discussed.

Aged↗
PubMed1992

A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group.

Nonrhabdomyosarcoma soft tissue sarcomas in infants and children are rare malignancies with most of the clinical data gained by retrospective analysis. In 1986, a prospective multicentered study was initiated by the Pediatric Oncology Group (POG) with a total of 75 cases now entered for epidemiologic examination. Median age of presentation was 12 years (range, newborn to 20 years). The male to female ratio was 2.3 to 1. The most common soft tissue tumor was synovial cell sarcoma (32/75, 42%), followed by fibrosarcoma (10/75, 13%), malignant fibrous histiocytoma (9/75, 12%), and malignant neurogenic tumors (8/75, 10%). Sixty-five percent of all tumors presented on the extremities (44% lower extremity, 21% upper extremity). Tumors of the trunk accounted for 28% (abdomen 15%, thorax 13%), whereas head and neck tumors were 7%. By TNMG classification, 16% presented as stage I, 21% as stage II, 33% as stage III, and 30% as stage IV. Age at presentation did not affect clinical site or stage. All upper extremity tumors presented with localized disease, whereas lower extremity tumors presented with regional nodal disease in 7% and metastatic disease (pulmonary) in 23% of the cases. Seventy-eight percent of the abdominal tumors were metastatic at diagnosis; the other 22% had extensive regional disease. In the thorax 78% had localized lesions with 22% having extensive regional disease. Ninety percent of synovial cell sarcomas were on the extremities: 84% localized disease and 12% metastatic spread at presentation. Sixty percent of the fibrosarcomas presented on the extremities with 80% having localized disease and 20% metastatic spread. Only 25% of the neurogenic tumors presented with localized disease, whereas 50% had metastases at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Rhabdomyosarcoma coexistent with ovarian mucinous cystadenocarcinoma: a case report.

This report describes a case of ovarian mucinous cystadenocarcinoma associated with rhabdomyosarcoma. Several cases of sarcoma-like mural nodules in ovarian mucinous tumor have been described previously, but only three well-documented cases of true sarcoma have been reported. The first case was a fibrosarcoma associated with a mucinous cystadenoma; the second was an undifferentiated sarcoma in a mucinous cystadenocarcinoma; and the third was a fibrosarcoma occurring in a mucinous cystadenocarcinoma. The occurrence of rhabdomyosarcoma in mucinous cystadenocarcinoma, as reported here, is to our knowledge the first case reported of this unusual combination of tumors.

Cystadenocarcinoma↗
PubMed2005

Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.

Deep fibromatoses (desmoid tumors) are clonal myofibroblastic proliferations that are prone to aggressive local recurrences but that do not metastasize. They must be distinguished from a host of fibroblastic and myofibroblastic lesions as well as from smooth muscle neoplasms. Virtually all deep fibromatoses have somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations leading to intranuclear accumulation of beta-catenin. Since low-grade sarcomas in general lack beta-catenin and since reactive proliferations would not be expected to have it, we predicted that nuclear beta-catenin expression would be detected in deep fibromatoses but absent in other entities in the differential diagnosis. We evaluated the role of beta-catenin to help differentiate distinguish deep fibromatoses from congeners. Formalin-fixed, paraffin-embedded sections from 21 lesions from 20 patients with deep fibromatoses were stained with monoclonal beta-catenin antibody (Transduction Laboratories) and compared with low-grade fibromyxoid sarcoma (n=12), leiomyosarcoma (n=10), various other fibrosarcoma variants (n=13, including 3 myofibrosarcomas, 3 sclerosing epithelioid fibrosarcomas, 5 low-grade fibrosarcomas, 1 classic fibrosarcoma arising in dermatofibrosarcoma protuberans, 1 inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma), myofibroma/myofibromatosis (n=12), nodular fasciitis (n=11), and scars (n=9). Nuclear and cytoplasmic staining was assessed. All 21 examples of deep fibromatosis displayed nuclear beta-catenin (focal nuclear staining in one case to 90% staining). All other lesions tested (n=67) lacked nuclear labeling for beta-catenin, showing only cytoplasmic accumulation. beta-Catenin immunohistochemistry separates deep fibromatosis from entities in the differential diagnosis, a finding that can be exploited for diagnosis. Most fibromatoses have diffuse nuclear staining although occasional examples only focally label.

Adolescent↗
PubMed1998

Detection of SYT-SSX1/2 fusion transcripts by reverse transcriptase-polymerase chain reaction (RT-PCR) is a valuable diagnostic tool in synovial sarcoma.

Cytogenetically, most synovial sarcomas are characterised by a specific chromosomal translocation [(X;18) (p11.2;q11.2)], which results in the generation of fusion transcripts comprising SYT (18q11) and either SSX1 or SSX2 (Xp11) sequences. By using a sensitive reverse transcriptase-polymerase chain reaction (RT-PCR) protocol, specific SYT-SSX1/2 fusion transcripts were detected in 10 histopathologically confirmed synovial sarcomas. Control tumours with morphological spindle cell patterns mimicking monophasic synovial sarcoma tested negative (18/19) in the RT-PCR protocol, with the exception of one spindle cell sarcoma originally classified as a fibrosarcoma. Furthermore, the established RT-PCR protocol was used to evaluate the feasibility of SYT-SSX1/2 fusion transcript detection for minimal residual disease analysis. Analyses of surgical margins revealed a fusion transcript in two of four operations for synovial sarcoma analysed, one of which was diagnosed with tumour free margins by conventional histopathology. These data suggest that the RT-PCR amplification of SYT-SSX1/2 fusion transcripts is a valuable tool in the differentiation of synovial sarcomas, especially in cases of equivocal morphology. Additionally, the RT-PCR approach may be used for the detection of residual tumour cells in synovial sarcoma patients.

Adolescent↗
PubMed1986

Fibrohistiocytic tumors of soft tissues. An immunohistochemical study of 183 cases.

183 cases of soft tissue tumors were studied utilizing the immunoperoxidase technique to demonstrate alpha-1-antitrypsin, ferritin and lysozyme. The series comprises 50 malignant lesions, 34 intermediate malignancy lesions, 99 benign lesions of fibrohistiocytic origin, and 23 malignant tumors of non fibrohistiocytic origin. The actual results of the study are as follows: alpha-1-antitrypsin, ferritin and lysozyme were always absent in 10 fibrosarcomas, 2 liposarcomas, 2 Ewing sarcomas, 3 synovial sarcomas, 4 neurofibrosarcomas, and 2 rhabdomyosarcomas, but in 24 malignant fibrous histiocytomas, 34 cases of dermatofibrosarcoma protuberans and 102 benign fibrohistiocytic lesions, these activities were present in a percentage that ranged between 12% and 38% (average 25%). Differences in the frequency of positive reactions did not occur between benign and malignant fibrohistiocytic lesions. The immunohistological examinations carried out have, therefore, only a very limited value for the practical diagnostic evaluation, but, when positive, are important to clarify the histogenesis of the tumor.

Ferritins↗
PubMed2003

The role of electron microscopy in the diagnosis of pleomorphic sarcomas of soft tissue.

Classification of pleomorphic malignancies is frequently problematic and important with regard to treatment. Their histologic differential diagnosis is extremely wide, including sarcomatoid carcinoma, melanoma, anaplastic lymphoma, and a large number of sarcomas with overlapping light microscopic appearances. Not infrequently, immunohistochemical investigations of such tumors yield conflicting or confusing results. In such cases, electron microscopy remains an invaluable investigative and diagnostic adjunct, revealing certain subcellular features that indicate a specific line of differentiation. Combining ultrastructural and immunohistochemical studies is particularly useful in these tumors. This article focuses on the ultrastructural aspects of certain sarcomas that are predominantly pleomorphic, including high-grade fibrosarcoma, myxofibrosarcoma-malignant fibrous histiocytoma, acral myxoinflammatory fibroblastic sarcoma, pleomorphic liposarcoma, pleomorphic leiomyosarcoma, and pleomorphic rhabdomyosarcoma, as well as certain sarcomas that are occasionally quite pleomorphic, including angiosarcoma, malignant granular cell tumor, alveolar soft part sarcoma, and extraskeletal osteosarcoma. We also briefly comment on the common simulators of pleomorphic sarcomas, including melanoma, carcinoma, and lymphoma.

Biomarkers, Tumor↗
PubMed1978

[Fibrosarcoma of the right ventricle of the heart--a contribution to the pathology of the primary sarcoma of heart and pulmonary arteries (author's transl)].

The clinical signs as well as the pathological-anatomical and histological peculiarities of a case of fibrosarcoma of the right ventricle of the heart in a fifty-years-old women are described. Examining of all cases described in literature it becomes evident that when ascertaining the starting points, there is a fluent transition from the sarcoma of the endocardium to the sarcoma of the pulmonary arteries. Four types become evident. The morphological pecularities of the appearance of Anitschkow-cells are described. Origin and importance of these cells are not known yet in all their details. The Survey over the single observations speaks in favour of their affiliation to the cellular defence system of the heart.

Female↗
PubMedDate not supplied

Fibrosarcoma: observations on the ultrastructure.

Ultrastructural observations on 60 fibrosarcomas are presented. Myofibroblastic differentiation was found in 33 tumors but usually in scattered cells. Diffuse aggregates of nonspecific intermediate filaments were present in a number of the tumors. Electron microscopy is useful to distinguish fibrosarcoma from other soft tissue sarcomas that it can resemble by routine light microscopy, and its value is enhanced when selected immunocytochemical procedures are also performed.

Adolescent↗
PubMed2002

Expression profiling of synovial sarcoma by cDNA microarrays: association of ERBB2, IGFBP2, and ELF3 with epithelial differentiation.

Synovial sarcoma is an aggressive spindle cell sarcoma with two major histological subtypes, biphasic and monophasic, defined respectively by the presence or absence of areas of glandular epithelial differentiation. It is characterized by a specific chromosomal translocation, t(X;18)(p11.2;q11.2), which juxtaposes the SYT gene on chromosome 18 to either the SSX1 or the SSX2 gene on chromosome X. The chimeric SYT-SSX products are thought to function as transcriptional proteins that deregulate gene expression, thereby providing a putative oncogenic stimulus. We investigated the pattern of gene expression in synovial sarcoma using cDNA microarrays containing 6548 sequence-verified human cDNAs. A tissue microarray containing 37 synovial sarcoma samples verified to bear the SYT-SSX fusion was constructed for complementary analyses. Gene expression analyses were performed on individual tumor samples; 14 synovial sarcomas, 4 malignant fibrous histiocytomas, and 1 fibrosarcoma. Statistical analysis showed a distinct expression profile for the group of synovial sarcomas as compared to the other soft tissue sarcomas, which included variably high expression of ERBB2, IGFBP2, and IGF2 in the synovial sarcomas. Immunohistochemical analysis of protein expression in tissue microarrays of 37 synovial sarcomas demonstrated strong expression of ERBB2 and IGFBP2 in the glandular epithelial component of biphasic tumors and in solid epithelioid areas of some monophasic tumors. Fluorescence in situ hybridization analysis indicated that the ERBB2 overexpression was not because of gene amplification. Differentially expressed genes were also found in a comparison of the expression profiles of the biphasic and monophasic histological subgroups of synovial sarcoma, notably several keratin genes, and ELF3, an epithelial-specific transcription factor gene. Finally, we also noted differential overexpression of several neural- or neuroectodermal-associated genes in synovial sarcomas relative to the comparison sarcoma group, including OLFM1, TLE2, CNTNAP1, and DRPLA. Our high-throughput studies of gene expression patterns, complemented by tissue microarray studies, confirm the distinctive expression profile of synovial sarcoma, provide leads for the study of glandular morphogenesis in this tumor, and identify a new potential therapeutic target, ERBB2, in a subset of cases.

Cell Differentiation↗