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PubMed1976

Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases.

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.

Adolescent↗
PubMed2002

Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms.

Malignant peripheral nerve sheath tumors (MPNSTs) are diagnostically challenging neoplasms for which sensitive and specific immunohistochemical markers are lacking. Although limited to date, previous studies have suggested that NF1 (17q), NF2 (22q), p16 (9p), and EGFR (7p) alterations may be involved in MPNST tumorigenesis. To determine whether specific genetic changes differentiate between MPNST and morphologically similar neoplasms, we assessed these chromosomal regions in 22 MPNSTs (9 NF1-associated, 13 sporadic), 13 plexiform neurofibromas, 5 cellular schwannomas, 8 synovial sarcomas, 6 fibrosarcomas, and 13 hemangiopericytomas by 2-color FISH. NF1 deletions, often in the form of monosomy 17, were found in MPNSTs (76%). neurofibromas (31%), hemangiopericytomas (17%), and fibrosarcomas (17%), but not in synovial sarcomas or cellular schwannomas. NF1 losses were encountered more frequently in MPNSTs versus other sarcomas (p < 0.001), as were p16 homozygous deletions (45% vs 0%; p < 0.001), EGFR amplifications (26% vs 0%; p = 0.006), and polysomies for either chromosomes 7 (53% vs 12%; p = 0.003) or 22 (50% vs 4%; p < 0.001). Hemizygous or homozygous p16 deletions were detected in 75% of MPNSTs, but not in benign nerve sheath tumors (p < 0.001). Thus, FISH analysis identifies relatively specific genetic patterns that may be useful in selected cases, for which the differential diagnosis includes low- or high-grade MPNST.

Cytogenetic Analysis↗
PubMed1950

Fibrosarcoma.

Explore the source record for details and available documents.

Fibrosarcoma↗
PubMed1990

HLA-DR (Ia-like) reactivity in tumors of bone and soft tissue: an immunohistochemical comparison of monoclonal antibodies LN3 and LK8D3 in routinely processed specimens.

Recent studies of Class II histocompatibility antigen expression in bone and soft tissue sarcomas have suggested that malignant fibrous histiocytoma (MFH) may express HLA-DR, whereas histologically similar pleomorphic, epithelioid, and spindle cell malignant neoplasms generally do not. To test whether these observations are reproducible in the differential diagnosis of soft tissue sarcomas, anti-HLA-DR antibodies LK8D3 and LN3 were applied to formalin-fixed, paraffin-embedded sections of MFH, neurofibrosarcoma (NFS), leiomyosarcoma (LMS), synovial sarcoma (SS), fibrosarcoma (FS), angiosarcoma (AS), Kaposi's sarcoma (KS), chondrosarcoma (ChS), "dedifferentiated" chondrosarcoma (DChS), osteosarcoma (OS), epithelioid sarcoma (ES), and clear cell sarcoma (CCS; malignant melanoma of soft parts). The only consistent difference in Class II antigen expression was seen in the group of neoplasms composed of large polygonal cells. Among the latter lesions, four of six clear cell sarcomas were labeled by LK8D3 or LN3, but none of 12 epithelioid sarcomas were reactive. Otherwise, a diversity of tumors in other morphologic categories expressed Class II antigens, with no clear diagnostic patterns. These results may be of use in the diagnostic separation of large cell epithelioid tumors of soft tissue, but neither LN3 nor LK8D3 appears to be helpful in the identification of other sarcomas.

Antibodies, Monoclonal↗
PubMed1979

Ovarian low-grade stromal sarcoma with thecomatous features: a critical reappraisal of the so-called "malignant thecoma".

A case of low-grade ovarian stromal sarcoma in a postmenopausal woman is described. Although pelvic recurrences of the tumor followed 5 and 7 years after the original surgery, the patient has remained well and without evidence of tumor 3 years since the last operation. Histopathologic, electronmicroscopic, and hormonal studies are described. There was evidence of estrogenic stimulation by the theca elements of the tumor in this patient. Cases previously reported in the world literature as malignant thecoma were analyzed, and most of them were considered inadequately documented; indeed most of them were probably either sarcomatoid granulosa cell tumors, stromal sarcomas, or fibrosarcomas. If a thecoma ever becomes malignant, the tumor cells dedifferentiate so that they cannot be recognized any longer as theca cells; instead, they proliferate as a stromal sarcoma or fibrosarcoma. It is proposed, therefore, that the term "malignant thecoma" not be used. On the other hand, very rare malignant ovarian stromal tumors do exist, consisting of undifferentiated stromal cells, fibroblasts, and theca cells, which can show evidence of hormonal activity.

Diagnosis, Differential↗
PubMed2000

Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience.

Eighty-six children to 18 years of age were treated for nonrhabdomyosarcoma soft tissue sarcomas of the trunk and extremities. Synovial sarcoma (31), fibrosarcoma (13), malignant fibrous histiocytoma (11), epithelioid sarcoma (10), and clear cell sarcoma (7) were the most common diagnoses. Four patients presented with metastatic disease. A high percentage of patients presented after biopsy by the referring physician, although this could not be shown to affect outcome. Patients were treated with wide removal of the tumor when possible, with judicious use of adjuvant radiation, or with chemotherapy in selected cases. Mean followup was 11 years. Five- and 10-year survival was 92% and 84%, respectively. Tumors larger than 5 cm were associated with a worse prognosis. When compared with published data in adults, the prognosis of primary, localized nonrhabdomyosarcoma soft tissue sarcomas in children appears to be more favorable.

Adolescent↗
PubMed1997

Evidence that porcine pancreatic phospholipase A2 via its high affinity receptor stimulates extracellular matrix invasion by normal and cancer cells.

Cellular migration and extracellular matrix (ECM) invasion are some of the critical steps in embryonic implantation, inflammation, wound healing, and cancer metastasis. Extracellular phospholipases A2 (PLA2s), belonging to either group I (PLA2-I) or group II (PLA2-II), play an essential role in the generation of proinflammatory lipid mediators (e.g. prostaglandins, leukotrienes, etc.). Recent reports indicate that PLA2-I, in addition to its digestive function, has receptor-mediated effects. For instance, PLA2-I, via its receptor, can induce cell proliferation and airway as well as vascular smooth muscle contraction. Here, we report that both porcine pancreatic and Naja naja PLA2s, in a dose-dependent manner, stimulate dramatic invasion of an artificial ECM (MatrigelTM) by NIH 3T3, mouse fibrosarcoma, and sarcoma cells in vitro, but it has no such effect on lymphoma and mastocytoma cells. That this is a receptor-mediated process is strongly suggested by the following findings. (a) While NIH 3T3, mouse fibrosarcoma, and sarcoma cells, which respond to PLA2-I stimulation, express high affinity PLA2-I receptor mRNA and protein, this receptor expression is not detectable in nonresponder lymphoma and mastocytoma cells. (b) There is a close correlation between the Kd values for 125I-PLA2-I binding to its receptor and the ED50 values for PLA2-I-induced ECM-invasion. (c) Catalytically inactivated PLA2-I is as effective as the active enzyme in stimulating invasion. (d) Suppression of PLA2-I receptor expression in responder cells causes inhibition of ECM invasion. (e) Treatment of the same cells with PLA2-I receptor-antibody also produces virtually identical effects. Taken together, our results identify a novel receptor-mediated function of PLA2-I and raises the possibility that extracellular PLA2s play important physiological as well as pathological roles via this receptor. To our knowledge, this is the first report of this novel receptor-mediated effect (i.e. ECM invasion) of PLA2-I on normal as well as malignant cancer cells.

3T3 Cells↗
PubMed2006

Carcinogenicity and chronic toxicity of para-chloronitrobenzene in rats and mice by two-year feeding.

Carcinogenicity and chronic toxicity of para-chloronitrobenzene (p-CNB) were examined by feeding diets containing p-CNB to rats and mice of both sexes for two years. The dietary concentration of p-CNB was 0 (control), 40, 200, or 1000 ppm (w/w) for rats and 0, 125, 500, or 2000 ppm for mice. Survival rates of the high-dosed male rats and male mice were significantly decreased compared with those of the respective controls, and this was attributed to the increased number of cancer deaths. Therefore, the high-dose levels were considered not to exceed the maximum tolerated dose. Significant decreases in red blood cell counts and hematocrit value and an increase in mean corpuscular volume were noted in the p-CNB-fed rats and mice. Nonneoplastic splenic lesions were characterized by capsule hyperplasia, fibrosis, fatty metamorphosis, and increased extramedullary hematopoiesis in rats, and congestion, increased extramedullary hematopoiesis, hemosiderin deposition, and ossification in mice. Incidences of fibromas, fibrosarcomas, osteosarcomas, sarcomas (NOS), and hemangiosarcomas in males and fibrosarcomas in females were significantly increased in the spleen of high-dosed rats. The most frequently observed splenic tumor was fibrosarcomas, followed by fibromas. The tumor incidences were increased in a dose-related manner and were more prevalent in males than in females. The malignant tumors metastasized mainly to the liver, peritoneum, and pancreas. Adrena/medullary hyperplasia and pheochromocytomas were significantly increased in the p-CNB-fed females. No tumor was induced in any of the p-CNB-fed mice of either sex except hepatic hemangiosarcomas in the 2000 ppm-fed females. Causative factors of p-CNB-induced carcinogenicity and chronic toxicity are discussed in light of the subchronic and chronic hematotoxicity reported in our present and previous studies and in the literature.

Administration, Oral↗
PubMedDate not supplied

Spectrum of low-grade fibrosarcomas: a comparative ultrastructural analysis of low-grade myxofibrosarcoma and fibromyxoid sarcoma.

Low-grade fibrosarcomas have recently gained increasing attention in the literature, especially with the fall in popularity polls of the ubiquitous malignant fibrous histiocytoma (MFH). Firstly, most tumors previously known as myxoid MFH are labeled presently as myxofibrosarcomas. Secondly, the recognition and better understanding of a family of fibrosing-type fibrosarcoma, encompassing 3 members: fibromyxoid sarcoma (FMS), hyalinizing spindle cell tumor with giant rosettes (HSTGR), and sclerosing epithelioid fibrosarcoma (SEF). To expand further their understanding of the overlapping and distinct features of members included in the spectrum of low-grade fibrosarcoma, the authors carried out a comparative ultrastructural study among 15 low-grade myxofibrosarcomas (MFS) and 12 fibromyxoid sarcomas (FMS), after review of pathology and confirmation of diagnosis. The ultrastructural findings of the LG MFS identified spindle to plump cells, with abundant cytoplasm, rich in well-developed RER cisternae, often distended and sometimes cystically dilated, containing an electronlucent granular material. These results were in keeping with a well-differentiated fibroblastic-type cell phenotype. In addition, a less prominent cellular component included cells with RER, well-developed Golgi apparatus, lysosomes, and filopodia. These latter features define a fibroblastic variant with histiocytic-like properties, also known as histiofibroblasts. Myofibroblastic differentiation was quite limited and mostly absent in most of the cases. In summary, these findings recapitulate a similar spectrum with the cell constituents of so-called MFH. In contrast, the fine microscopic findings of the 12 FMS cases showed an inactive or more primitive form of fibroblastic type cells. The RER cisternae were generally underdeveloped, as expected for a generic fibroblastic-type proliferation. The cytoplasm was scant and showed a paucity of organelles, with the exception of abundant arrays of vimentin-type intermediate filaments. The very long, thin cell processes, sometimes associated with pinocytotic vesicles, were reminiscent of perineurioma ultrastructure.

Adult↗