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PubMed2002

Primary sarcomas of the brain and spinal cord: a study of 18 cases.

Primary sarcomas of the central nervous system are exceedingly rare. We reviewed the clinicopathologic features of 18 primary central nervous system sarcomas diagnosed from 1959 through 1999. Median age at diagnosis of the nine female and nine male patients was 28 years (range 3-63 years). Median tumor size was 4 cm (range 1.3-8 cm). Fifteen tumors arose in the cerebrum (83%), two in the cerebellum, and one in the spinal cord. Histopathologically, the most common tumor types included fibrosarcoma (six), malignant fibrous histiocytoma (five), and undifferentiated sarcoma (three). Immunohistochemical and ultrastructural studies supported the histologic diagnosis in 17 and six cases, respectively. All patients had subtotal to gross total tumor resection; 16 also received radiotherapy and/or chemotherapy. Twelve tumors (67%) were high-grade. Follow-up was obtained in all instances (median 2.3 years). Nine patients died of the disease, eight with high-grade tumors. Survival at 5 years for patients with high-grade tumors was 28% compared with 83% for those with low-grade neoplasms (p = 0.03). Primary central nervous system sarcomas most often affect young and middle-aged adults. Most involve the cerebrum and show fibrous, "fibrohistiocytic," or no specific differentiation. The prognosis for high-grade sarcomas seems better than that for glioblastoma multiforme.

Adolescent↗
PubMed1983

Post-irradiation sarcoma of the lower lip.

This report records the occurrence of an irradiation induced fibrosarcoma of the lower lip which appears to be the first case documented in this site. The problems associated with the histopathological diagnosis of such lesions are discussed and the value of electronmicroscopical examination of spindle cell sarcomas stressed.

Aged↗
PubMed2003

Localized extremity soft tissue sarcoma: improved knowledge with unchanged survival over time.

PURPOSE: The objective of this study was to define whether survival of patients with extremity soft tissue sarcoma (STS), stratified for known risk factors, has improved over the last 20 years. PATIENTS AND METHODS: From January 1982 to December 2001, 1,706 patients with primary and recurrent STS of the extremities were treated at our institution and were prospectively followed. From this cohort, we selected 1,261 patients who underwent complete macroscopic resection and had one of the following histopathologies: fibrosarcoma, liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, or synovial sarcoma. Median follow-up was 55 months. Patient, tumor, and treatment factors were analyzed as prognostic factors. RESULTS: The 5-year disease-specific actuarial survival was 79% (78% for patients treated from 1982 to 1986, 79% for patients treated from 1986 to 1991, 79% for patients treated from 1992 to 1996, and 85% for patients treated from 1997 to 2001; P = not significant). For high-risk patients (high-grade, > 10 cm, deep tumors; n = 247), 5-year disease-specific survival was 51% (50% for patients treated from 1982 to 1986, 45% for patients treated from 1986 to 1991, 52% for patients treated from 1992 to 1996, and 61% for patients treated from 1997 to 2001; P = not significant). Tumor depth, size, grade, microscopic margin status, patient age, presentation status (primary tumor versus local recurrence), location (proximal versus distal), and certain histopathologic subtypes were significant prognostic factors for disease-specific survival on multivariate analysis; however, time period of treatment was not. CONCLUSION: Prognosis of patients with extremity STS, stratified for known risk factors, has not improved over the last 20 years, indicating that current therapy has reached the limits of efficacy.

Adult↗
PubMed2004

[Inhibition effect of glycyrrhizin on transplanted mandibular gland fibrosarcoma of mice].

OBJECTIVE: To study glycyrrhizin's anticancer effect and its mechanism. METHODS: 3-methylcholanthrene were injected into mice's submandibular glands to induce tumor, then transplanted the tumor pieces (1 mm3) to mice. The transplanted tumors were measured, and flow cytometry analysis and cytomorphology observation were conducted. RESULTS: Glycyrrhizin (GL) inhibited the transplanted mandibular gland fibro-sarcoma of mice and the suitable GL dose for inhibiting fibrosarcoma of mice was 1.61 mg per 20 g weight. The GL dose below 3.22 mg per 20 g weight didn't produce remarkable toxicity and side effects. GL induced cytomorphological changes of tumor cells and enhanced immunosuppression of macrophage on fibrosarcoma. The result of flow cytometry showed that tumor cell counts of GL1 and GL2 groups increased remarkably in DNA synthetic prophase, and decreased in DNA synthetic phase. CONCLUSION: GL can inhibit transplanted mandibular gland fibro-sarcoma of mice. The anticancer mechanism of GL may be acting on related enzymes with phagocytosis. The result of flow cytometry showed that the shift of fibrosarcoma cells from G1 phase to S phase was blocked. This suggests that the anticancer action of GL is related to its inhibition of ribonucleotide reductase, a rate-limiting enzyme in DNA synthesis.

Animals↗
PubMed1984

Nucleotide sequence of v-fps in the PRCII strain of avian sarcoma virus.

PRCII is an avian retrovirus whose oncogene (v-fps) induces fibrosarcomas in birds. The viral gene v-fps arose by transduction of an undetermined portion of a cellular gene known as c-fps. PRCII is weakly oncogenic when compared with Fujinami sarcoma virus, another transforming virus containing v-fps. As a first step in the elucidation of the molecular basis for the decreased virulence of PRCII, we have determined the entire nucleotide sequence of v-fps in the PRCII genome. The v-fps domain in PRCII encodes a polypeptide with a molecular weight of ca. 60,500 fused to a portion of the polyprotein encoded by the viral structural gene gag. The hybrid gag-fps polyprotein of PRCII would have a molecular weight of ca. 98,100, in accord with results of previous studies of the protein encoded by the PRCII genome. The leftward junctions between fps and gag in Fujinami sarcoma virus and PRCII are located at the same position in fps, but at different positions in gag. A sequence of 1,020 nucleotides, bounded by direct repeats of 6 nucleotides, is present in v-fps of Fujinami sarcoma virus but absent from PRCII. Our data should permit further explorations of the relationship between structure and function in the transforming protein encoded by v-fps.

Amino Acid Sequence↗
PubMed1992

Soft tissue sarcomas of the extremities and trunk in the adult. Report of 124 cases.

Between 1970 and 1988 surgery was performed on 124 patients with soft tissue sarcomas of the extremities and trunk in the University Clinics of Hamburg. Liposarcoma, malignant fibrous histiocytoma, fibrosarcoma and malignant schwannoma were the most common histological types. High-grade sarcomas (G3) predominated, with 41%, while 26% were graded G2 and 33% G1. Resection with wide margins all round was achieved in only 54% of the patients. The quality of the operation proved to be only therapy-related prognostic factor. In addition, tumour grade, size, regional lymph node and distant metastasis and histological type proved to be relevant to the prognosis. With multivariate analysis, distant metastasis, grade, resectability and histological type retained prognostic significance. The efficacy of adjuvant chemo- and radiotherapy was related to the quality of the preceding tumour resection. In case of gross tumour the rate of either partial or complete response was 28% for chemotherapy and 22% for radiotherapy. The mean survival time was 102 months; the 5- and 10-year survival rates were 48% and 37%, respectively.

Adolescent↗
PubMed1992

[Digital image processing of radiography of 32 cases with mandible sarcomas].

The radiographys of 32 cases with mandible sarcoma were analysed with digital image processing. The processed image of fibrosarcoma showed many scattered fine grains after the X-ray film were edge detected. In the same processing, the image of chondrosarcoma showed many little rings or arc structures. For osteosarcoma, the processed image showed very irregular veins. These digital processing pictures can help us to differentiate mandible sarcomas.

Chondrosarcoma↗
PubMed1998

[Late results of radiotherapy of soft tissue sarcoma].

PURPOSE: To evaluate the effect of radiation therapy in the treatment of soft-tissue sarcomas. MATERIALS AND METHODS: Between March 1970 and January 1990, 58 patients with soft-tissue sarcoma were referred for radiation therapy. The most frequent histologic diagnoses included fibrosarcoma (n = 15), neurofibrosarcoma (n = 5) and rhabdomyosarcoma (n = 5). Central tumor sites in the trunk (n = 31) were much more frequent than in the head and neck region (n = 14) or the extremities (n = 13). Thirty-nine of 58 primary tumors were bigger than 5 centimeters. Forty-five patients were irradiated after surgery, 5 patients prior to surgery; in 8 cases only radiation therapy was used. Radiation therapy was performed with Co-60 photons and an average total dose of 58 Gy, fractionated in single doses of 2 Gy. The treatment results were obtained by actual follow-up examinations. RESULTS: Twenty-three of 58 patients survived at least 5 years (39.9%). Of 15 patients with R0 resection 11 were alive after 5 years (73.3%). Local tumor control was achieved in 34 of 58 patients (58.6%). Low 5-year-survival rates were associated with dedifferentiation of the primary tumor (three survivors in 10 patients with G3 tumor), tumor diameters over 5 cm (13 survivors of 39), R2 resection (3 survivors of 16) and tumor sites in the body trunk (11 survivors of 31). CONCLUSIONS: (1) The best results of radiation therapy were achieved after R0 resection of the primary tumor. (2) Tumors in the trunk are prognostically worse because of bigger tumor diameters due to later diagnosis.

Female↗
PubMed1996

Head and neck sarcomas: a review.

Sarcomas of the head and neck are rare. They present a challenge to the head and neck oncologist that is frequently quite different from that found in epithelial, salivary, and endocrine malignancies of the head and neck. The focus of this article is on diagnostic criteria, prognostic indicators, and therapy, and due to the large number of lesions, an in-depth review of the commonest: osteosarcoma (OS), chondrosarcoma (CS), rhabdomyosarcoma (RMS), fibrosarcoma (FS), malignant fibrous histiocytoma (MFH), and synovial sarcoma (SS). Less common variants will also be mentioned. Twelve cases are presented, consisting of OS(2), RMS(1), CS(3), MFH(2), SS(2), FS(1), and miscellaneous(1). Conclusions consist of an enunciation of the general principles of management of sarcoma of the head and neck.

Adult↗
PubMed1984

Sarcoma of bone and soft tissue following treatment of Hodgkin's disease.

Intensive chemotherapy and radiotherapy of Hodgkin's Disease has resulted in improved survival rates. With long-term follow-up, however, a risk of secondary malignancy in these individuals is now appreciated. The authors of this report have encountered five patients who developed bone or soft tissue sarcomata more than 5 years after treatment of Hodgkin's Disease. The four males and one female ranged in age from 14 to 74 years at the time of diagnosis of Hodgkin's disease. Two had received radiotherapy alone for treatment of Hodgkin's disease, two were treated with radiation and chemotherapy, and one received only chemotherapy. The latent period prior to diagnosis of sarcoma ranged from 6 to 11 years. There was one case each of neurofibrosarcoma, fibrosarcoma, osteosarcoma, liposarcoma and leiomyosarcoma. Four patients died within 1 year of the diagnosis of sarcoma. One is alive with no evidence of disease 2 years following diagnosis and surgical excision of the sarcoma. On the basis of the Massachusetts General Hospital experience in the treatment of Hodgkin's Disease, the authors calculate a risk of 0.9% of sarcoma occurring in five year survivors of Hodgkin's disease. Previously reported cases of sarcoma following treatment of Hodgkin's disease are summarized. The pertinent literature is reviewed.

Adult↗