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PubMed1995

Soft tissue sarcomas that mimic benign lesions.

Although it is well known that a variety of benign mesenchymal neoplasms and reactive proliferations may mimic sarcomas, the fact that some types of sarcoma commonly are misinterpreted as benign is less widely appreciated. This review considers five such lesions in soft tissue: low grade fibromyxoid sarcoma, low grade myxofibrosarcoma (myxoid "MFH"), well differentiated liposarcoma (atypical lipoma), epithelioid sarcoma, and so-called inflammatory fibrosarcoma. Principal reasons for their underrecognition are their generally bland cytology, often relative hypocellularity, and indolent clinical course. Common to several of these lesions, however, is a tendency for histological and hence biological progression in local recurrences, underlining the importance of accurate diagnosis and adequate treatment of the primary lesion. This review considers the principal clinicopathologic features, differential diagnosis and (where appropriate) nosologic status of this treacherous group of neoplasms.

Connective Tissue↗
PubMedDate not supplied

p53/MDM-2 immunohistochemical expression correlated with proliferative activity in different subtypes of human sarcomas: a ten-year follow-up study.

The aim of this study was the evaluation of p53/MDM-2 protein overexpression in different subtypes of human sarcomas, and their correlation with proliferative activity and patient outcome. We selected 40 cases of human sarcomas comprising 6 malignant fibrous histiocytomas (MFH), 1 fibrosarcoma, 1 dermatofibrosarcoma protuberans, 5 liposarcomas, 9 leiomyosarcomas, 1 rhabdomyosarcoma, 3 synovial sarcomas, 2 osteosarcomas, 1 chondrosarcoma, 4 Ewing's sarcomas, 2 Kaposi's sarcomas, 1 malignant haemangiopericytoma, 1 phylloides cystosarcoma, 1 neuroblastoma, 1 chordoma and 1 unclassified sarcoma. All the immunohistochemical markers, which had been used for the characterization of these sarcomas were re-examined. Additionally, the Streptavidin-Biotin peroxidase method was performed on paraffin sections using the monoclonal antibodies: anti-p53 antibody DO7, anti-MDM-2 antibody IF2 and anti-Ki-67 antibody MIB-1. According to our results, p53 protein nuclear expression was detected in 20% (8/40) of the tumours (1 fibrosarcoma, 2 liposarcomas, 1 leiomyosarcoma, 1 rhabdomyosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). MDM-2 nuclear staining was determined in 7.5% (3/40) of the cases (1 MFH and 2 liposarcomas). A high proliferative index was demonstrated in 27.5% (11/40) of the tumours (2 MFH, 4 leiomyosarcomas, 1 rhabdomyosarcoma, 1 osteosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). p53 overexpression was associated with high tumour grade (p < 0.05) and MIB-1 expression was correlated with reduced survival (p < 0.05), but p53 overexpression was not significantly associated with either MIB-1 score or with overall survival of the patients. In conclusion, from this limited and heterogeneous sample of cases, we suggest that the p53/MDM-2 pathway is involved in the tumourigenesis of several sarcoma subtypes, but it is unclear if the overexpression of these genes may become prognostic marker for patients affected with these highly aggressive tumours.

Adolescent↗
PubMed1976

Detection of private and common tumor-associated antigens in murine sarcomas induced by different chemical carcinogens.

Two fibrosarcomas of similar histological type, induced in C3Hf mice by either methylcholanthrene or 3,4-benz(a)pyrene, were shown to have individually unique tumor-rejection antigens in classical transplantation-type experiments. By contrast, sera of autochthonous mice, which resisted only transplants of the immunizing sarcoma, were found to contain complement-dependent cytotoxic antibodies, specific for both sarcomas, in vitro. The existence of individually unique as well as common tumor-associated antigens in chemically-induced murine sarcomas is suggested. The private "tumor transplantation-type" antigens elicited tumor rejection responses in vivo. The common tumor-associated antigens, although immunogenic in autochthonous hosts, inducing the production of tumor-specific antibodies, failed to induce transplantation cross-resistance in vivo. This study supports the contention that, in carcinogen-induced murine tumors, and perhaps in human neoplasms as well the evaluation of humoral (and cell-mediated) immune responses in vitro may not reflect tumor rejection-type immune responses in vivo.

Animals↗
PubMed2001

Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor.

Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of deep soft tissues, originally described in 1995 by Meis-Kindblom et al. In the current study, the authors identified 16 cases of SEF in the pathology files of their institutions and studied their pathologic features and disease course. The group consisted of six male and 10 female patients (age range, 14-55 years; mean age, 40 years), and the tumors were located in a limb or limb girdle (n = 7), base of the penis (n = 1), back or chest wall (n = 3), and head and neck (n = 5). Tumor size ranged from 3.7 to 22 cm (mean, 8.9 cm). Histologically, the SEFs were composed predominantly of small to moderate-size round to ovoid, relatively uniform cells, often with clear cytoplasm, embedded in a hyalinized fibrous stroma. The only consistent immunohistochemical finding was a strong, diffuse reactivity of tumor cells for vimentin. Ultrastructural analysis performed in eight cases confirmed their fibroblastic nature. Bone invasion and tumor necrosis, features not reported before, were found in six cases each. Treatment consisted of intralesional excision (n = 2), attempted wide local excision (n = 11), and amputation (n = 3), with either adjuvant radiation therapy (n = 9) or chemotherapy (n = 3). Follow-up of at least 1 year in 14 cases revealed persistent disease or local recurrence in seven patients (50%), and distant metastasis in 12 patients (86%). Eight patients (57%) died of disease 16 to 86 months after diagnosis. Five patients were alive with disease as of last follow-up. SEF shares some pathologic features with two other fibrosing fibrosarcomas, low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes, but in the authors' experience behaves clinically as a fully malignant sarcoma.

Adolescent↗
PubMed1989

Chest wall tumors in infancy and childhood.

The chest wall is an infrequent site of malignancy in infancy and childhood. Management of these tumors, however, is of particular concern because of their aggressive behavior and the functional impairment which may result from local treatment. From 1976 to 1987 we have treated seventeen infants and children with tumors of the chest wall. Askin's tumors and Ewing's sarcoma were considered as a single entity, malignant small round cell tumor (MSRCT), and account for the majority (11 of the 17 patients). Other tumors represented were infantile fibrosarcoma (1), undifferentiated spindle cell sarcomas (2), osteogenic sarcoma (1), large cell lymphoma (1), and synovial sarcoma (1). Nine of 17 patients have survived (median follow-up of survivors 5 years); six patients died of disease and two from complications of therapy. All four patients with MSRCT and metastasis at diagnosis died of disease despite chemotherapy and radiotherapy. Four of the seven patients with localized MSRCT, who received combined modality therapy including resection (two after initial chemotherapy), radiotherapy, and chemotherapy, were continuously disease-free 16 months to 10 years following diagnosis. One of the three patients who failed died of complications of surgery to her extensive primary. A second patient had a relapse of disease in a hilar node four years after finishing vincristine, actinomycin, and cytoxan (VAC) chemotherapy; she was retreated with Adriamycin (doxorubicin, Adria Laboratories, Columbus, Ohio), vincristine, and cyclophosphamide as well as radiotherapy to her hilum and remains in second remission 56 months following her recurrence. The third patient suffered a distant relapse in bone and, before succumbing to his MSRCT, developed acute monocytic leukemia and died during a remission induction attempt. Mixed results were obtained for the patients with other tumor types.

Adolescent↗
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↗
PubMed1982

Failure of short-term treatment with flurbiprofen to enhance the therapeutic effect of cyclophosphamide against rodent sarcomas and a leukaemia.

Animals bearing metastatic fibrosarcomas were treated with cyclophosphamide (CY) alone or in combination with flurbiprofen (FP), an inhibitor of prostaglandin synthesis. FP did not affect local growth of fibrosarcomas, and the incidence of distant metastases after resection of the "primary" implants was comparable in treated and control groups. Treatment with CY retarded growth of the fibrosarcomas and reduced the proportion of animals which succumbed to metastases, but this was not altered significantly by additional treatment with FP. FP did not affect the survival of rats bearing a lymphoid leukaemia. The lifespan of animals treated with CY was increased significantly, but the concomitant administration of FP did not enhance this effect.

Animals↗
PubMed1991

Chronic carcinogenic and toxic effects of a single subcutaneous dose of cadmium in the male Fischer rat.

The carcinogenic effects of cadmium have not been thoroughly assessed in the commonly used Fischer (F344) rat. This study determined tumor incidence in various tissues of male F344/NCr rats after a single dose of cadmium. Cadmium (as CdCl2) was given sc in the dorsal thoracic midline at 30 mumole/kg to 70 8-week old male F344 rats while controls (n = 50) received saline. Rats were observed during the next 90 weeks. Early deaths (less than or equal to 32 weeks), due mostly to acute cadmium-induced hepatotoxicity, accounted for 37 of the cadmium-treated rats while no control rats died in the same period. A high incidence of injection site sarcomas (ISS) occurred in the cadmium-treated group (21 ISS/32 rats at risk; 66%) while only 1/50 occurred in controls (2%). In fact, ISS were the major cause of morbidity after 35 weeks in cadmium-treated rats. These tumors were mostly fibrosarcomas, although histiocytic and osteogenic sarcomas also occurred. Testicular interstitial cell tumors, which show a very high spontaneous incidence in this strain (41/49; 84%), were not markedly affected by cadmium (30/31; 97%). This is in sharp contrast to other strains, such as the Wistar, in which cadmium treatment is reported to cause as much as an eightfold increase in interstitial cell (Leydig cell) tumor incidence. The incidence of large granular lymphocyte (LGL) leukemia, which also occurred frequently in control F344 rats (12/47; 26%) was markedly decreased (2/31; 7%) by cadmium. Our recent studies indicate acute lymphonecrotic effects occur with cadmium in lymphoid tissues of rats, and this may be related to the suppression of the leukemia. These results indicate that cadmium is very effective in inducing ISS in F344 rats, as is the case with other strains thus far tested, and also markedly reduces spontaneous leukemia incidence.

Animals↗
PubMed1988

Sarcoma metastatic to the brain.

Five patients with nonlymphomatous sarcoma metastatic to the brain are presented and the literature reviewed. In 50 patients, brain metastasis was verified pathologically and some case data were available, including the five reported here. In five patients, there was pathologic verification but no case data. Thirty-nine other patients were found without such verification. Reportedly, most types of sarcomas are able to metastasize to the brain, and are represented in these 94 patients. Fibrosarcoma and alveolar soft-part sarcoma tumors metastasize to the brain without regard to treatment technique. There may be a group of tumors, including malignant fibrous histiocytoma, rhabdomyosarcoma, and perhaps leiomyosarcoma and osteosarcoma, in which the incidence of brain metastases has increased with improved sarcoma chemotherapy (CT). In this group particularly, but also in alveolar soft-part sarcoma and others, the presence of lung metastases may increase the probability of brain metastasis occurring subsequently.

Adolescent↗
PubMed1992

Primary sarcomas of the heart.

Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.

Adolescent↗
PubMed1992

Karyotype instability and altered differentiation of rat sarcoma cells after retroviral infection.

The karyotypic and phenotypic stability of cultured rat fibrosarcoma cells was challenged by infection with Moloney murine sarcoma virus (MoMuSV). After transformation, the spindle-like morphology of the parental HH-16 cl.2/1 cells had altered to a rounded phenotype, which was maintained in tumors produced by inoculating transformed cells into congenic animals. In contrast to the parental cells, transformed cells lacked cables of cytokeratins 14-16 and 19 and showed reduction of the mesenchymal marker protein vimentin. Additionally, the morphologically altered cell clones tf-1 to tf-3 had lost growth arrest in the presence of dexamethasone. The DNA of the transformed cells contained between four and six randomly integrated proviral copies. Karyotypic alterations were manifested by reduction of morphologically intact chromosomes in the MoMuSV-transformed cells together with increase of structural aberrations. Three additional markers were identified in the virus-transformed cell clones. Karyotypic instability induced by MoMuSV infection appeared closely related to reduction of the cellular differentiation status, although only cells of clone tf-1 had increased metastatic potential.

Age Factors↗
PubMed1969

Inhibition of tumor growth by polyinosinic-polycytidylic acid.

The synthetic double-stranded RNA, polyinosinic-cytidylic acid, inhibits the growth of some tumors in mice. Two days after implantation of a reticulum cell sarcoma, a lymphatic lymphoma, a fibrosarcoma, two leukemias, and a human adenovirus 12-induced tumor, treatment of groups of mice resulted in decreased growth rates of the tumors and increased survival times of the animals. In the two tumors tested (the reticulum cell sarcoma and the adenovirus 12-induced tumor) initiation of treatment after the tumor was grown to moderate size caused a regression of the tumor. In the case of the reticulum cell sarcoma, the tumor had not reappeared in some of the animals two months after cessation of treatment.

Adenoviridae↗
PubMed1991

Primary intracranial sarcomas: histopathological features of 19 cases.

Nineteen primary intracranial sarcomas out of a total of about 25,000 brain tumour biopsies are reported. Subtypes included malignant fibrous histiocytoma (6 cases), leiomyosarcoma (3), rhabdomyosarcoma (2), angiosarcoma (2), and one case each of fibrosarcoma, low-grade fibromyxoid sarcoma, malignant ectomesenchymoma, mesenchymal chondrosarcoma, differentiated chondrosarcoma and Ewing's sarcoma. Histological and immunohistochemical features corresponded to those of extracranial sarcomas. Nests of pleomorphic astrocytes mimicking glioma were detected in the five storiform-pleomorphic malignant fibrous histiocytomas. Our results indicate that intracranial sarcomas can be classified like their extracranial counterparts. The low incidence compared with earlier series is related to changes in classification and progress in histogenetic clarification.

Adult↗
PubMed1976

Effect of Clostridium toxoids, especially of Clostridium perfringens toxoid, on mouse transplanted tumors.

The antitumor activities of toxoids of Clostridium perfringens, C. nouyi, C. septicum, and C. tetani were tested against sarcoma-180 and Ehrlich carcinoma. Among them, C. perfringens toxoid showed a high antitumor activity against the growth of the implanted sarcoma-180 ascites form. The results of the inhibiting effect of C. perfringens toxoid on Nakahara-Tokuzen-Fukuoka (NTF) reticulum cell sarcoma and methylcholanthrene-induced fibrosarcoma were also described.

Animals↗
PubMed1980

Inhibition of benzo(a)pyrene carcinogenesis in rats with vitamin C.

The s.c. infection of 10 mg benzo(a)pyrene dissolved in 1 ml tricaprylin induced in Wistar rats local malignant tumors, such as fibrosarcoma, rhabdomyosarcoma, and polymorph cell sarcoma. The growth of the tumors was relatively rapid, reaching weights of 140-155 g before rats died 142-168 days after the administration of the carcinogen. On the contrary, under the same experimental conditions, high doses of Vitamin C about 525 mg/day/rat administered orally in drinking water (total amount of Vitamin C 55 g/rat corresponding to 40% of their body weight ) inhibited to a great extent the benzo(a)pyrene carcinogenesis. Only one slowly growing rhabdomyosarcoma (13 g of weight) was developed showing characteristic damage of malignant cells and partial replacement of the neoplastic area with granuloma tissue. The significance ov Vitamin C for cancer prevention and treatment is discussed.

Animals↗
PubMed1997

Cytogenetics as an adjunct in establishing a definitive diagnosis of synovial sarcoma by fine-needle aspiration.

BACKGROUND: Synovial sarcomas account for up to 10% of all soft tissue sarcomas and are characterized by a specific chromosomal abnormality, t(X; 18)(p11.2; q11.2), that is observed in both monophasic and biphasic variants. METHODS: A 9-cm suprascapular mass in a 43-year-old man and a 10-cm leg mass in a 45-year-old man were aspirated. Rapid evaluation of air-dried Diff-Quick-stained smears in both cases showed a malignant spindle cell lesion. Cytogenetic material was collected in RPMI 1640 tissue culture medium. Additional alcohol-fixed slides and cell blocks were prepared on both cases. Immunohistochemical studies were performed on paraffin embedded cell block sections in both cases whereas electron microscopic examination was performed in one case. G-banded chromosome preparations from tumor cells were made after short term culture using standard cytogenetic techniques. RESULTS: Evaluation of the smears showed densely cellular and tightly cohesive malignant spindle cells without discernible epithelial differentiation. A differential diagnosis of synovial sarcoma, leiomyosarcoma, malignant schwannoma, and fibrosarcoma was considered. Immunohistochemical stains and electron microscopy were noncharacteristic and noncontributory in establishing a diagnosis. However, cytogenetic results revealed a t(X; 18)(p11.2; q11.2) translocation, thus establishing the diagnosis of synovial sarcoma. Both patients were offered definitive therapy based on fine-needle aspiration (FNA) diagnosis only. CONCLUSIONS: FNA can be used effectively to obtain karyotypes of sarcomas. The specific cytogenetic abnormality associated with synovial sarcoma provides an opportunity to make definitive diagnoses using a combination of FNA and cytogenetics, obviating open surgical biopsy preceding therapy.

Adult↗