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Infantile fibrosarcoma of thigh--a case report.

Infantile fibrosarcoma is a rare soft tissue malignant tumor, when it occurs, it is usually seen in the first year of life. The clinical course of infantile fibrosarcoma is more favorable and metastasis is rare compared with that in adulthood. While adult fibrosarcoma are common in the thigh, infantile fibrosarcoma affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. In this case report, we present our experience of an infantile fibrosarcoma of thigh with good clinical course 36 months after tumor resection and the usefulness of detecting the ETV6-NTRK3 gene fusion in differential diagnosis.

DNA-Binding Proteins↗

Inhibitory effects of thymoquinone against 20-methylcholanthrene-induced fibrosarcoma tumorigenesis.

The potential antitumor effect of thymoquinone (TQ), the main constituent of the volatile oil of Nigella sativa seed, on fibrosarcoma induced by 20-methylcholanthrene (MC) in male Swiss albino mice was investigated in vivo and in vitro. Administration of TQ (0.01% in drinking water) I week before and after MC treatment significantly inhibited the tumor incidence and tumor burden by 43% and 34%, respectively, compared with the results in the group receiving MC alone. Moreover, TQ delayed the onset of MC-induced fibrosarcoma tumors that appeared at 12 weeks and produced less MC-induced mortality. Lipid peroxide accumulation, decreased glutathione (GSH) content, and decreased activities of glutathione S-transferase (GST) and quinone reductase (QR) were observed in the liver of MC-induced tumor-bearing mice. TQ alone showed a significant induction in the enzyme activities of hepatic GST and QR. Mice treated with TQ along with MC showed reduction in hepatic lipid peroxides and increased GSH content and increased enzyme activities of GST and QR as compared to results of the control group. The in vitro studies showed that TQ inhibited the survival of fibrosarcoma cells with IC50 of 15 microM. Conversely, TQ inhibited the incorporation of [3H] thymidine in fibrosarcoma cells with IC50 of microM. Our data indicate the potential of TQ as a powerful chemopreventive agent against MC-induced fibrosarcoma tumors. The possible modes of action of TQ may be through its antioxidant activity and interference with the DNA synthesis coupled with enhancement of detoxification processes.

Animals↗

Fibrosarcoma of the uterus: a case report.

BACKGROUND: This case report presents the management of a patient with fibrosarcoma of the uterus. Until recently, no cases of fibrosarcomas of the uterus have been reported in the literature. CASE REPORT: A 40-year-old woman presented with complaints of slight vaginal bleeding, watery, white discharge and abdominal pain of 3 weeks' duration. The patient underwent total abdominal hysterectomy. Intraoperative findings included an enlarged irregular-shaped uterus with lots of leiomyoma nodules. The largest leiomyoma nodule was about 8 cm in size and contained an area classified as a fibrosarcoma, presenting cellular immature-appearing fibroblasts growing in a fascicular arrangement. Because of the limited extension of the fibrosarcoma, no adjuvant therapy was performed. After surgical therapy the patient was followed without any evidence of disease for five years. CONCLUSION: Fibrosarcoma is a poorly-circumscribed infiltrative spindle soft tissue sarcoma, which is characterized by local growth and has a propensity for local recurrence. Metastases can occur, mostly to lung and bone. The treatment of choice is wide surgical excision usually followed by radiation therapy due to the high local recurrence rate.

Adult↗

Immunoprophylaxis of transplantable methylcholanthrene-induced murine fibrosarcomas by immunization with embryo cells expressing endogenous murine leukemia virus antigens.

We investigated the nature of a common tumor rejection antigen(s) in chemically induced murine fibrosarcomas. Two methylcholanthrene-induced fibrosarcomas, previously demonstrated to contain a common tumor rejection antigen(s), released infectious ecotropic murine leukemia virus and expressed the murine leukemia virus proteins, a glycoprotein with a molecular weight of 70,000 (gp70) and an envelope protein with a molecular weight of 15,000. To determine whether an antigen(s) specified by a murine leukemia virus might serve as a common tumor rejection antigen(s), primary cultures of syngeneic embryo cells or cultures of an allogeneic embryo cell line were infected with an endogenous ecotropic murine leukemia virus obtained from one of the cross-reacting fibrosarcomas; expression of infectious virus and/or viral proteins by infected and uninfected embryo cells was monitored and correlated with the results of transplantation protection tests. Uninfected allogeneic embryo cells (SC-1) did not release infectious virus or the viral protein gp70; mice immunized with SC-1 cells did not inhibit tumor growth. Uninfected syngeneic embryo cells did not release infectious virus but did release micrograms quantities of gp70 into supernatant fluids; mice immunized with uninfected syngeneic cells inhibited tumor growth in two of seven experiments. Virus-infected syngeneic and allogeneic embryo cells released both infectious ecotropic murine leukemia virus and gp70; mice immunized with virus-infected cells inhibited tumor growth in 11 of 11 experiments. Growth of the two cross-reacting fibrosarcomas was inhibited in mice immunized with virus-infected embryo cells. The results indicate that antigens coded for by endogenous murine leukemia virus may function as common tumor rejection antigen on chemically induced murine fibrosarcomas.

Animals↗

Experimental tumors with features of malignant fibrous histiocytomas. Light microscopic and electron microscopic investigations on tumors produced by cell transplantation of an established fibrosarcoma cell line.

An established fibrosarcoma cell line (RFS) derived from a cadmium induced fibrosarcoma of rat and RFS-cells in different subcultures produced tumors in nude mice and baby rats which showed characteristics of fibrosarcoma, malignant fibrous histiocytoma and completely undifferentiated sarcoma. After thorough examination of cells in culture and in experimental tumors by light microscopic and electron microscopic methods that conclusion has been drawn that malignant fibrous histiocytoma can develop from fibrosarcoma. By this the assumption that malignant fibrous histiocytomas derive from tissue histiocytes is questioned. After the discussion of the general unspecifity of the storiform growth pattern and the cytology of malignant fibrous histiocytomas the idea is presented that many defined malignant soft tissue tumors inclusively fibrosarcomas may pass a phase which should morphologically be diagnosed as malignant fibrous histiocytoma. Conceding the practical value of this diagnosis we emphasize that the concept of the entity "malignant fibrous histiocytoma" should be critically reevaluated because of the probable heterogeneity of this tumor group.

Animals↗

Epidemiologic evidence for a causal relation between vaccination and fibrosarcoma tumorigenesis in cats.

Within the past 2 years, a putative causal relationship has been reported between vaccination against rabies and the development of fibrosarcomas at injection sites in cats. A retrospective study was undertaken, involving 345 cats with fibrosarcomas diagnosed between January 1991 and May 1992, to assess the causal hypothesis. Cats with fibrosarcomas developing at body locations where vaccines are typically administered (n = 185) were compared with controls (n = 160) having fibrosarcomas at locations not typically used for vaccination. In cats receiving FeLV vaccination within 2 years of tumorigenesis, the time between vaccination and tumor development was significantly (P = 0.005) shorter for tumors developing at sites where vaccines are typically administered than for tumors at other sites. Univariate analysis, adjusted for age, revealed associations between FeLV vaccination (odds ratio [OR] = 2.82; 95% confidence interval [CI] = 1.54 to 5.15), rabies vaccination at the cervical/interscapular region (OR = 2.09; 95% CI = 1.01 to 4.31), and rabies vaccination at the femoral region (OR = 1.83; 95% CI = 0.65 to 5.10) with fibrosarcoma development at the vaccination site within 1 year of vaccination. Multivariate analysis, adjusted for age and other vaccines, also revealed increased risks after FeLV (OR = 5.49; 95% CI = 1.98 to 15.24) and rabies (OR = 1.99; 95% CI = 0.72 to 5.54) vaccination.(ABSTRACT TRUNCATED AT 250 WORDS)

Adjuvants, Immunologic↗

Multiple diffuse fibrosarcoma of bone.

Light and electron microscopic observations of a case of multiple diffuse fibrosarcoma of bone are presented. Two cases of diffuse fibrosarcoma of bone have been previously reported. Clinically the tumor presented as multiple osteolytic lesions involving the pelvis bone, vertebral bodies and skull with the radiologic appearance of a multiple plasma cell myeloma. Histologically the tumor was a spindle-cell fibrosarcoma. At the ultrastructural level the tumor cells represent fibroblasts separated by a scanty amount of collagen fibers. Electron microscopic findings substantiate the fibroblastic origin of multiple diffuse fibrosarcoma of bone.

Bone Neoplasms↗

Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. VI. Fibrous tissue tumors (fibroma, fibromatosis, fibrosarcoma).

In a study of 256 nonepithelial neoplasms involving the nasal cavity, paranasal sinuses, and nasopharynx, 23 lesions were classified as fibrous tissue tumors, including four cases of "fibroma", six of fibromatosis, and thirteen of fibrosarcoma. The clinical findings associated with these lesions are described, their histologic features illustrated, results of therapy presented and clinicopathologic correlations made. The "fibromas" presented a small localized nodules. None recurred after local excision. Fibromatosis, a locally aggressive tumor, does not metastasize, but may cause considerable morbidity or even death due to local infiltration which may be difficult to control surgically. Fibrosarcoma may cause death either by local infiltration or by metastasis, but has a better prognosis than most other sarcomas of this region. We recommend that a large en block resection be performed initially for fibromatosis and fibrosarcoma growing in this area, after the diagnosis has been made by biopsy. In this series, including patients who had more than one operation, recurrent tumor was seen following 10 of 12 limited local excisions performed for fibromatosis and fibrosarcoma, but in only one of 13 patients after a large bloc resection. The problems involved in histologically differentiating fibrous tissue tumors from other lesions are discussed. A patient with the rare syndrome of multicentric fibromatosis with spontaneous regression of lesions is presented.

Adult↗

Soft tissue fibrosarcoma. A clinicopathologic study of 132 cases.

Over the last 20 to 30 years, a recognition and general acceptance of the entity of malignant fibrous histiocytoma and monophasic synovial sarcoma has occurred. The introduction of these diagnostic categories among the generic group of spindle cell sarcomas now requires a reevaluation of the more traditional and historic entity of fibrosarcoma and the clinical ramification of this diagnosis. A review of 132 cases of fibrosarcoma using current diagnostic criteria was undertaken to clarify the behavior of fibrosarcoma as diagnosed in current patients. Special attempts were made to correlate histologic grade and Enneking stage in fibrosarcomas in an effort to correlate their predictive value in metastatic rate and survival.

Actuarial Analysis↗

Recurrent allelic imbalance at the rat Pten locus in DMBA-induced fibrosarcomas.

The tumor-suppressor gene PTEN (phosphatase and tensin homolog) is frequently inactivated in different types of human tumors. Less is known about the involvement of the homologous gene Pten in animal model systems of cancer. By sequencing one of the introns of rat Pten, we found an informative intragenic PCR marker suitable for genetic studies. Through use of this marker, the position of Pten in the genetic linkage map was localized to the distal part of rat chromosome 1 (RNO1) by analysis of F2 progeny from an intercross between inbred strains BN and LE. Subsequently, 22 markers from this region (including the intragenic Pten marker) were used to study the occurrence of allelic imbalance in distal RNO1 in fibrosarcomas that had been induced by DMBA in F1(BNxLE) rats. The analysis revealed that allelic imbalance was common in the vicinity of Pten, and there was loss or reduction of one of the Pten alleles in more than 60% of the fibrosarcomas. DNA sequencing was preformed to investigate whether the Pten allele remaining in the tumors was inactivated by mutation. However, no mutations were detected in the genomic sequence of Pten exons 5 to 9 in any of the fibrosarcomas, and normal mRNA transcripts were expressed in all tumors. Thus, based on the targeted selection for loss of Pten observed in some of these tumors and the absence of inactivation of the remaining allele, we suggest that haploinsufficiency of Pten may be an important factor in rat DMBA-induced fibrosarcomas.

9,10-Dimethyl-1,2-benzanthracene↗

Tumor necrosis factor alpha induces early morphologic and metabolic alterations in Friend leukemia cell tumors and fibrosarcomas in mice.

Morphologic and metabolic studies have been carried out on Friend leukemia cell (FLC) tumors (grown in DBA/2 mice) or fibrosarcomas (grown in C3H/HeN or C3H/HeJ mice) shortly after peritumoral injection of recombinant mouse tumor necrosis factor (TNF) alpha. Marked vascular congestion and focal extravasation of erythrocytes were observed as soon as 1 hr after injection of either FLC tumors or fibrosarcomas with TNF. Focal areas of disaggregation of tumor cells were observed 1 hr after injection of TNF. Intraluminal thrombi (composed of degranulated platelets and fibrin) were detected 3 and 6 hr after TNF treatment, and were associated with areas of depletion of endothelial cell cytoplasm. To correlate these morphologic changes in the tumor with alterations in tumor metabolism, NMR spectroscopy and biochemical studies were undertaken on freshly dissected FLC tumors and fibrosarcomas shortly after injection of TNF. The earliest metabolic alterations observed after 1 hr in TNF-treated FLC tumors of fibrosarcomas were: (i) increase in the average intratumoral pH; (ii) decrease in the levels of ATP. These phenomena were not associated with a reduced glycolytic capacity of TNF-treated tumors as, at these early times after injection, the levels of lactic acid were virtually the same for TNF-treated or control treated tumors. Alterations in the levels of some products of phospholipid degradation (GroPCho, GroPEtn, GroP and Cho) also occurred in these tumors as early as 3 hr after TNF treatment. These metabolic changes were not observed in ascitic FLC tumors after TNF treatment. We suggest that TNF induces alterations in tumor blood vessels which subsequently lead to changes in tumor metabolism and tumor degeneration.

Adenosine Triphosphate↗

Fibrosarcoma of nose and paranasal sinuses.

Involvement of nose and maxilla by fibrosarcoma is extremely rare. Only a few cases of fibrosarcoma arising in this region are reported in the world literature. Two cases of fibrosarcoma are described, and the literature relating to this disease entity is briefly reviewed. Salient features helping in the diagnosis and management of fibrosarcoma also are highlighted.

Adult↗

Genetics of susceptibility in the platyfish/swordtail tumor system to develop fibrosarcoma and rhabdomyosarcoma following treatment with N-methyl-N-nitrosourea (MNU).

About 7000 animals of 65 different genotypes of the xiphophorine fish were treated with the direct acting chemical carcinogen N-methyl-N-nitrosourea (MNU; 10(-3)M; four times for 1 hour in two week intervals), in order to find out whether the susceptibility for development of fibrosarcomas and rhabdomyosarcomas is directly related to the genotype. A genotype specific susceptibility was found, ranging from zero to about nine percent. The highest susceptibles were found in certain backcross hybrids involving P.variatus/X.helleri-hybrids and X.helleri, as the recurrent parent. These genotypes were further analysed. Both P.variatus and X.helleri, as werr as their F1 proved to be insusceptible; while from the three backcrosses, which were tested, namely the BC1, BC4 and BC15, both the BC1, and the BC4, were susceptible, but the BC15 was insusceptible. The results are interpreted on the basis of the assumption that the differential susceptibility is a function of the type of control of a tumor gene (Tu-Fi-Rh) endogenous to P.variatus and involved in development of fibrosarcomas and rhabdomyosarcomas. Accordingly, in P.variatus and in the F1 the Tu-Fi-Rh is controlled by repressing genes (R-genes) linked as well as non-linked to Tu-Fi-Rh; because simultaneous mutation of both R-genes following treatment with MNU is an extremely unlikely event, these genotypes have an extremely low susceptibility. By contrast, in the BC1 and the BC4 the non-linked R-genes become eliminated and only the linked R-gene remains for repression of Tu-Fi-Rh; this condition confers a high degree of susceptibility, because one single mutation may lead to impairment of the R-gene and to Tu-Fi-Rh-mediated formulation of fibrosarcomas and rhabdomysarcomas. In the BC15, furthermore, also the Tu-Fi-Rh has become eliminated, resulting in a loss of the susceptibility. The results suggest that in the xiphophophorine fish the susceptibility for responding to MNU-treatment with the development of fibrosarcomas and rhabdomysarcomas is related directly to the genotype.

Age Factors↗

Effect of alpha-tocopherol on lipid peroxidation and antioxidant system in fibrosarcoma bearing rats.

The anticarcinogenic activity of alpha-tocopherol (Vitamin E) was tried in fibrosarcoma induced rats through its antioxidative potential. The rate of formation of malondialdehyde (MDA), the end product of lipid peroxidation was analysed in alpha-tocopherol (400 mg/kg body weight) treated and untreated fibrosarcoma bearing rats with respective controls. The levels of non-enzymic antioxidants like, glutathione and vitamin E, and enzymic antioxidants viz., catalase (CAT), superoxide dismutase (SOD), glutathione peroxidase (GSH-PX), and glutathione-S-transferase (GST) were assayed as well. Significantly increased (p < 0.001) level of lipid peroxide was observed with concomitant decreases in the level of enzymic and non-enzymic antioxidants in fibrosarcoma bearing rats when compared with control animals. In alpha-tocopherol supplemented animals, the corrected level of these parameters were observed likely to near normal values. Thus, alpha-tocopherol can be accepted to pose first line of defense mechanism against excessively formed reactive species due to impaired antioxidant systems in fibrosarcoma conditions, that cause membrane damage leading to deleterious effects.

Animals↗

Neoplastic association of enhanced type V collagen production in rat fibrosarcoma.

Collagens present in the connective tissues of the extracellular matrix of fibrosarcoma were isolated and characterized. The fibrosarcoma was induced in rats by the administration of 3-methylcholanthrene. The results obtained were compared with normal muscle. An excess amount of type V collagen was found to be produced by the fibrosarcoma tissue compared to the normal muscle. Type V collagen from fibrosarcoma was characterized on the basis of solubility behavior in sodium chloride solutions, electrophoretic mobility on SDS-polyacrylamide gels, elution pattern of phosphocellulose chromatography and amino acid composition.

Amino Acids↗

An effective chemotherapy regimen for liver metastasis from retroperitoneal fibrosarcoma: report of a case.

Retroperitoneal fibrosarcoma is a rare disease that has proven difficult to treat due to its high incidence of postoperative local recurrence. We recently experienced a patient in whom retroperitoneal fibrosarcoma was followed by liver metastasis without local recurrence. A 34-year-old woman who initially presented with right upper quadrant pain was found to have a retroperitoneal tumor by diagnostic imaging techniques. Extirpation of the tumor was performed and the histopathological diagnosis was fibrosarcoma. A solitary metastasis was detected in the lateral segment 1 year after this operation and a lateral segmentectomy was carried out; however, a short time later, multiple liver metastases were found. Initially, ethanol injections were given with little effect, following which CYVADIC chemotherapy, consisting of cyclophosphamide, vincristine, farmorubicin, and dacarbazine was administered. An excellent responsiveness without severe toxicity was achieved after five cycles, with a significant reduction in tumor size, being estimated as a complete response. Thus, we consider that this chemotherapy regimen could be a promising mode of treatment for liver metastasis from retroperitoneal fibrosarcoma without local recurrence.

Adult↗

Anal fibrosarcoma: report of a case and review of literature.

A unique case of anal fibrosarcoma is reported. Review of the literature in the past half century revealed only 13 cases of rectal fibrosarcoma, and no case of anal fibrosarcoma. Abdominoperineal resection is the usual treatment if the disease is confined to the rectum or anus. An extended resection may be indicated in those patients who have more extensive disease. Long-term follow-up information was not available in the literature. The prognosis is presumed to be the same as if not worse than for fibrosarcomas in other parts of the body. Reporting of this rare tumor when found in unusual locations is encouraged.

Aged↗

[Malignant fibrous histiocytoma: pleomorphic sarcoma NOS or pleomorphic fibrosarcoma].

The entity and nosology of pleomorphic malignant fibrous histiocytoma (MFH) is still ambiguous. The actual WHO-Classification uses pleomorphic malignant fibrous histiocytoma (MFH) and pleomorphic sarcoma NOS (not otherwise specified) synonymously. On the other hand text and illustrations convey the impression, that these tumors also could be pleomorphic lipo-, leio- or rhabdomyosarcomas etc. It would have been more informative to emphasize, that with the above mentioned specific sarcoma types MFH-like appearance may occur. Furthermore it would have been more up to date to consider pleomorphic sarcomas NOS as pleomorphic fibrosarcomas and include them in the chapter of fibroblastic and myofibroblastic tumors. This concept already has been carried out for the former myxoid variant of MFH, nowadays preferentially called myxofibrosarcoma. There is controversial discussion about the clinical significance of exact typing of pleomorphic sarcomas. Problems may also occur due to the lack of standards, which degree of desmin expression signifies leiomyosarcoma or just indicates myofibroblasts in MFH. The requirement of exclusion of other tumor-types before diagnosing pleomorphic fibrosarcoma still remains obligatory. After verification of the diagnosis pleomorphic sarcoma NOS or pleomorphic fibrosarcoma, grading e.g. according to criteria of the FFCCS can be carried out. Most cases of pleomorphic fibrosarcoma will qualify as high grade malignant.

Diagnosis, Differential↗