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  • 1
    Online Resource
    Online Resource
    Clinical characteristics of undifferentiated pleomorphic sarcoma of bone and the impact of adjuvant chemotherapy on the affected patients: a population-based cohort study
    Oxford University Press (OUP) ; 2022
    In:  Japanese Journal of Clinical Oncology Vol. 52, No. 6 ( 2022-05-31), p. 589-598
    Details
    In: Japanese Journal of Clinical Oncology, Oxford University Press (OUP), Vol. 52, No. 6 ( 2022-05-31), p. 589-598
    Abstract: Clinical characteristics of undifferentiated pleomorphic sarcoma of bone are not elucidated. Herein, we clarify its clinical features and analyze the efficacy of adjuvant chemotherapy in patients with undifferentiated pleomorphic sarcoma of bone. Methods Prognostic factors and estimated disease-specific survival in 247 patients with primary undifferentiated pleomorphic sarcoma of bone were identified from a registry in Japan. The effect of adjuvant chemotherapy was evaluated in localized resectable cases, and the characteristics of the two groups treated with or without chemotherapy were adjusted using propensity score matching. Results The 5-year disease-specific survival rates were 47.4% in the entire cohort and 56.4 and 16.9% in the M0 and M1 groups, respectively. Multivariate disease-specific survival analysis revealed that metastasis on initial presentation and age ≥ 65 years were poor prognostic factors. Overall, 132 localized and resectable primary lesions were extracted. Adjuvant chemotherapy administration was a favorable prognostic factor (hazard ratio: 0.43, P = 0.04), and it significantly prolonged disease-specific survival compared with no adjuvant chemotherapy (5-year disease-specific survival: 78.8% vs. 51.8%, P = 0.008). Adjuvant chemotherapy prolonged disease-specific survival in patients with tumor size & lt;8 cm (5-year disease-specific survival: 100% vs. 54.6%, P = 0.02); however, its efficacy decreased in those with tumor size ≥8 cm (5-year disease-specific survival: 68.7% vs. 42%, P = 0.09). After propensity score matching, adjuvant chemotherapy was significantly associated with good disease-specific survival (P = 0.02). Conclusions Metastasis in the initial presentation was the poorest prognostic factor. On evaluating localized and resectable cases only, adjuvant chemotherapy significantly improved disease-specific survival, although its effect decreased in cases with large tumors.
    Type of Medium: Online Resource
    ISSN: 1465-3621
    URL: Article
    DOI: 10.1093/jjco/hyac017
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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  • 2
    Online Resource
    Online Resource
    Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study
    Oxford University Press (OUP) ; 2023
    In:  Japanese Journal of Clinical Oncology Vol. 53, No. 2 ( 2023-01-28), p. 138-145
    Details
    In: Japanese Journal of Clinical Oncology, Oxford University Press (OUP), Vol. 53, No. 2 ( 2023-01-28), p. 138-145
    Abstract: Primary tumor resection is the mainstay of treatment for malignant peripheral nerve sheath tumors. However, the efficacy of perioperative chemotherapy and radiotherapy for malignant peripheral nerve sheath tumors has not been elucidated. Methods This retrospective analysis based on a Japanese registry included patients with localized malignant peripheral nerve sheath tumors arising at the extremities and trunk wall. Disease-specific overall survival and local recurrence-free survival were estimated using the Kaplan–Meier method. A Cox regression model was used to identify prognostic factors. Characteristics of groups with or without chemotherapy were adjusted using propensity score matching. Results In total, 291 patients were included. The 5-year disease-specific overall survival rate was 70.6%. Multivariate analysis of disease-specific overall survival revealed that deep-seated tumors were a poor prognostic factor, but perioperative chemotherapy was not associated with disease-specific overall survival (hazard ratio, 0.81; 95% confidence interval, 0.45–1.43, P = 0.46). Local recurrence was observed in 55 patients (19.0%), and surgical margins (R1 and R2) were significant risk factors. Overall, perioperative chemotherapy did not prolong disease-specific overall survival (5-year disease-specific overall survival: 74.1% vs. 69.3%, P = 0.75) and had limited efficacy in the group with tumor size ≥ 5 cm, although the difference was not statistically significant (5-year disease-specific overall survival: 77.2% vs. 68.6%, respectively, P = 0.13). After adjustment by propensity score matching, perioperative chemotherapy significantly prolonged disease-specific overall survival (5-year disease-specific overall survival: 74.9% vs. 57.1%, P = 0.03), but this effect was not observed in local recurrence-free survival. In all patients, perioperative radiotherapy did not correlate with local recurrence-free survival (hazard ratio, 1.43; 95% confidence interval 0.78–2.62, P = 0.25). Conclusions Perioperative chemotherapy had limited efficacy for disease-specific overall survival in patients with localized malignant peripheral nerve sheath tumors.
    Type of Medium: Online Resource
    ISSN: 1465-3621
    URL: Article
    DOI: 10.1093/jjco/hyac178
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
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  • 3
    Online Resource
    Online Resource
    Body composition as a predictor of toxicity after treatment with eribulin for advanced soft tissue sarcoma
    Springer Science and Business Media LLC ; 2019
    In:  International Journal of Clinical Oncology Vol. 24, No. 4 ( 2019-4), p. 437-444
    Details
    In: International Journal of Clinical Oncology, Springer Science and Business Media LLC, Vol. 24, No. 4 ( 2019-4), p. 437-444
    Type of Medium: Online Resource
    ISSN: 1341-9625 , 1437-7772
    URL: Article
    DOI: 10.1007/s10147-018-1370-8
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2019
    detail.hit.zdb_id: 1481773-1
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  • 4
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    BRAF mutations and concurrent alterations in patients with soft tissue sarcoma
    Wiley ; 2023
    In:  Genes, Chromosomes and Cancer Vol. 62, No. 11 ( 2023-11), p. 648-654
    Details
    In: Genes, Chromosomes and Cancer, Wiley, Vol. 62, No. 11 ( 2023-11), p. 648-654
    Abstract: BRAF alterations, including V600E and non‐V600E mutations and fusions, in soft tissue sarcoma (STS) have been identified in a limited case series. Here, we aimed to evaluate the frequency of BRAF mutations and concurrent alterations in STS to understand their therapeutic action. In this retrospective analysis, we included data from 1964 patients with advanced STS who underwent comprehensive genomic profiling tests at hospitals in Japan between June 2019 and March 2023. The prevalence of BRAF and recurrent concurrent gene alterations were also investigated. BRAF mutations were detected in 24 (1.2%) of 1964 STS patients, with a median age of 47 (range 1–69) years. BRAF V600E was detected in 11 (0.6%) of the 1964 patients with STS, BRAF non‐V600E mutations in 9 (4.6%), and BRAF fusions were detected in 4 (0.2%). BRAF V600E was identified in 4 (0.2%) cases of malignant peripheral nerve sheath tumors. The most common concurrent alteration was CDKN2A (11 cases, 45.8%), and the frequency was equivalent to that of the BRAF V600E (5/11 cases, 45.5%) and non‐V600E (5/9 cases, 55.6%) groups. Recurrent concurrent alterations, such as TERT promoter mutations (7 cases, 29.2%), were detected at the same frequency in the V600E and non‐V600E groups. In contrast, TP53 alterations (4/9 cases, 44.4%) and mitogen‐activated protein kinase (MAPK)‐activating genes, including NF1 , GNAQ , and GNA11 (3/9 cases, 33.3%), were identified as relatively higher in the non‐V600E group than in the V600E group (each 1/11 case, 9.1%). We identified BRAF alterations at a rate of 1.2% in all patients with advanced STS. Among them, BRAF V600E and BRAF fusions account for 45.8% and 16.7%, respectively. Collectively, our findings support the clinical characteristics and therapeutic strategies for patients with BRAF ‐altered advanced STS.
    Type of Medium: Online Resource
    ISSN: 1045-2257 , 1098-2264
    URL: Issue
    URL: Article
    DOI: 10.1002/gcc.v62.11
    DOI: 10.1002/gcc.23182
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 1018988-9
    detail.hit.zdb_id: 1492641-6
    SSG: 12
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  • 5
    Online Resource
    Online Resource
    Role of adjuvant chemotherapy in patients with localized, undifferentiated pleomorphic sarcoma of soft tissue: a population-based cohort study
    Springer Science and Business Media LLC ; 2022
    In:  International Journal of Clinical Oncology Vol. 27, No. 4 ( 2022-04), p. 802-810
    Details
    In: International Journal of Clinical Oncology, Springer Science and Business Media LLC, Vol. 27, No. 4 ( 2022-04), p. 802-810
    Type of Medium: Online Resource
    ISSN: 1341-9625 , 1437-7772
    URL: Article
    DOI: 10.1007/s10147-021-02102-8
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
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  • 6
    Online Resource
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    Prognostic factors and treatment outcomes in patients with pleomorphic rhabdomyosarcoma: a population-based cohort study
    Oxford University Press (OUP) ; 2024
    In:  Japanese Journal of Clinical Oncology ( 2024-01-05)
    Details
    In: Japanese Journal of Clinical Oncology, Oxford University Press (OUP), ( 2024-01-05)
    Abstract: Pleomorphic rhabdomyosarcoma is a rare sarcoma in adults. The clinical characteristics, outcomes and prognostic factors associated with pleomorphic rhabdomyosarcoma remain unclear. Methods We retrospectively analyzed data from the Bone and Soft Tissue Tumor Registry of Japan, and enrolled patients with pleomorphic rhabdomyosarcoma. Disease-specific overall survival, local recurrence-free survival and distant metastasis-free survival were estimated using the Kaplan–Meier method; Cox regression model was used to identify prognostic factors. Results In total, 182 patients with pleomorphic rhabdomyosarcoma were included. Median age was 63 (range 20–95) years. The lower extremity (48%) was the most frequent tumor origin site, while head and neck were rare (4%). A total of 43 patients (24%) had distant or regional nodal metastases at first presentation. In all cases, the 2-year and 5-year survival rates were 66.3% and 54.1%, respectively. Distant metastasis was a significant poor prognostic factor (Hazard ratio 6.65; 95% confidence intervals, 3.00–14.75, P  & lt; 0.0001), with median survival of such patients being 9.4 (95% confidence intervals: 5.3–12.2) months. In 134 localized cases, the 2-year and 5-year survival rates were 91.5% and 68.3%, respectively. Large tumor size and older age were associated with poorer prognosis. Through data from localized and locally curative cases extracted and adjusted by propensity score matching, we found that perioperative chemotherapy did not improve disease-specific overall survival, distant metastasis-free survival or local recurrence-free survival. Conclusions Clinical characteristics and outcomes of pleomorphic rhabdomyosarcoma are similar to those of other high-grade soft tissue sarcomas. Pleomorphic rhabdomyosarcoma may be less chemosensitive, and a strategy other than the standard cytotoxic chemotherapy is required to improve its prognosis.
    Type of Medium: Online Resource
    ISSN: 1465-3621
    URL: Article
    DOI: 10.1093/jjco/hyad188
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2024
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  • 7
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    Comparison of clinical features and outcomes of patients with leiomyosarcoma of bone and soft tissue: a population-based cohort study
    Oxford University Press (OUP) ; 2022
    In:  Japanese Journal of Clinical Oncology Vol. 52, No. 2 ( 2022-02-05), p. 143-150
    Details
    In: Japanese Journal of Clinical Oncology, Oxford University Press (OUP), Vol. 52, No. 2 ( 2022-02-05), p. 143-150
    Abstract: Leiomyosarcoma commonly occurs in soft tissue but rarely in the bone. Whether leiomyosarcoma of bone and soft tissue have similar clinical characteristics and outcomes remains unknown. Methods This retrospective analysis was based on data from the Bone and Soft Tissue Tumor Registry in Japan. Patients with leiomyosarcoma of bone and soft tissue were enrolled. Overall survival and distant metastasis-free survival were estimated using the Kaplan–Meier method, and the Cox regression model was used to identify the prognostic factors. Results A total of 888 patients (60 leiomyosarcoma of bone and 828 leiomyosarcoma of soft tissue) were included in the study. Clinical characteristics were similar between the two groups, except for younger age in leiomyosarcoma of bone than in leiomyosarcoma of soft tissue (median 56 years vs. 66 years, P  & lt; 0.0001). To evaluate the prognostic factors and efficacy of adjuvant chemotherapy, data of localized and locally curative cases were extracted (total 572: 33 leiomyosarcoma of bone and 539 leiomyosarcoma of soft tissue). The 5-year overall survival rates of leiomyosarcoma of bone and soft tissue patients were similar (63.8% vs. 75.2%, P = 0.43); the 5-year distant metastasis-free survival tended to be worse in leiomyosarcoma of bone than in leiomyosarcoma of soft tissue (37.4% vs. 57.9%, P = 0.28). Larger tumor size (≥5 cm) and older age (≥65 years) correlated with poor overall survival in leiomyosarcoma of soft tissue patients. Adjuvant chemotherapy tended to prolong the overall survival of both leiomyosarcoma of bone (P = 0.11) and leiomyosarcoma of soft tissue patients with tumor size & gt;10 cm (P = 0.06). Conclusions The clinical characteristics and outcomes of leiomyosarcoma of bone and soft tissue patients were similar. In localized cases, adjuvant chemotherapy may improve the survival of leiomyosarcoma of bone and soft tissue patients with large-size tumor.
    Type of Medium: Online Resource
    ISSN: 1465-3621
    URL: Article
    DOI: 10.1093/jjco/hyab176
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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