| 1. |
- Boye, Kjetil, et al.
(författare)
-
High-Dose Chemotherapy with Stem Cell Rescue in the Primary Treatment of Metastatic and Pelvic Osteosarcoma: Final Results of the ISG/SSG II Study
- 2014
-
Ingår i: Pediatric Blood & Cancer. - : Wiley. - 1545-5017 .- 1545-5009. ; 61:5, s. 840-845
-
Tidskriftsartikel (refereegranskat)abstract
- BackgroundPatients with metastatic osteosarcoma at diagnosis or axial primary tumors have a poor prognosis. The aim of the study was to evaluate the feasibility and efficacy of intensified treatment with high-dose chemotherapy (HDCT) and stem cell rescue in this group. MethodsFrom May 1996 to August 2004, 71 patients were included in a Scandinavian-Italian single arm phase II study. Preoperative chemotherapy included methotrexate, doxorubicin, cisplatin and ifosfamide, and postoperative treatment consisted of two cycles of doxorubicin, one cycle of cyclophosphamide and etoposide and two courses of high-dose etoposide and carboplatin with stem cell rescue. ResultsTwenty-nine patients (43%) received two courses and 10 patients (15%) received one course of HDCT. HDCT was associated with significant toxicity, but no treatment-related deaths were recorded. Fourteen patients (20%) had disease progression before completion of the study protocol, and only 29/71 patients (41%) received the full planned treatment. Median event-free survival (EFS) was 18 months, and estimated 5-year EFS was 27%. Median overall survival (OS) was 34 months, and estimated 5-year OS was 31%. When patients who did not receive HDCT due to disease progression were excluded, there was no difference in EFS (P=0.72) or OS (P=0.49) between patients who did or did not receive HDCT. ConclusionsThe administration of high-dose chemotherapy with stem cell rescue was feasible, but associated with significant toxicity. Patient outcome seemed comparable to previous studies using conventional chemotherapy. We conclude that HDCT with carboplatin and etoposide should not be further explored as a treatment strategy in high-risk osteosarcoma. Pediatr Blood Cancer 2014;61:840-845. (c) 2013 Wiley Periodicals, Inc.
|
|
| 2. |
- Brandal, Petter, et al.
(författare)
-
Detection of a t(1;22)(q23;q 12) translocation leading to an EWSR1-PBX1 fusion gene in a myoepithelioma
- 2008
-
Ingår i: Genes, Chromosomes and Cancer. - : Wiley. - 1045-2257 .- 1098-2264. ; 47:7, s. 558-564
-
Tidskriftsartikel (refereegranskat)abstract
- Chromosome banding as well as molecular cytogenetic methods are of great help in the diagnosis of mesenchymal tumors. Myoepithelial neoplasms of soft tissue including myoepitheliomas, mixed tumors, and parachordomas are diagnoses that have been increasingly recognized the last few years. It is still debated which neoplasms should be included in these morphologically heterogeneous entities, and the boundaries between them are not clear-cut. The pathogenetic mechanisms behind myoepithelial tumors are unknown. Only five parachordomas and one mixed tumor have previously been karyotyped, and nothing is known about their molecular genetic characteristics. We present a mesenchymal tumor classified as a myoepithelioma that had a balanced translocation t(1;22)(q23;q12) as the sole karyotypic change. A novel EWSR1-PBX1 fusion gene consisting of exons 1-8 of the 5'-end of EWSR1 and exons 5-9 of the 3-end of PBX1 was shown to result from the translocation. Both genes are known to be targeted also by other neoplasia-specific translocations, PBX1 in acute lymphoblastic leukemia and EWSR1 in several solid tumors, most of which are malignant. Based on the structure of the novel fusion gene detected, its transforming mechanism is thought to be the same as for other fusion genes involving EWSR1 or PBX1.
|
|
| 3. |
- Hansen, Bjarne H, et al.
(författare)
-
The Scandinavian Sarcoma Group Skeletal Metastasis Registry Functional outcome and pain after surgery for bone metastases in the pelvis and extremities
- 2009
-
Ingår i: ACTA ORTHOPAEDICA. - : Medical Journals Sweden AB. - 1745-3674 .- 1745-3682. ; 80, s. 85-90
-
Tidskriftsartikel (refereegranskat)abstract
- Background Few authors have investigated function and pain after surgical treatment of patients with bone metastases. In 1999 the Scandinavian Sarcoma Group (SSG) initiated the Skeletal Metastasis Registry as a multi-centric, prospective study to provide a scientific basis for recommendations of treatment. Patients and methods We have analyzed function and pain in 530 patients (mean age 65 yr) operated on (599 operations) for non-spinal skeletal metastases at 9 SSG centres. 7% were operated for more than 1 metastasis. Carcinoma of the breast, prostate, kidney, and lung were the dominating sites for primary tumors. Results 25% of the patients died within 6 weeks after operation. 11% of the patients had complications. 6% had reoperation. In patients surviving more than 1 year the reoperation rate was 12%. 92% of the patients had no, light or moderate pain from metastasis at 6 weeks (first control) and 6 months follow-up. Patients using opioids were reduced from 40% preoperative to 30% at 6 months after surgery. In patients with metastases in pelvis or lower extremity 79% were walking with or without crutches, 6 weeks and 88%, 6 months after surgery. More patients with metastases; in proximal femur were mobile at 6 weeks and 6 months when treated with prosthetic replacement compared to internal fixation. Interpretation Palliative surgery for bone metastases improves function and reduce pain. Mobility is improved by surgery in patients with metastases in the pelvis or lower extremity. Prosthetic replacement seems to do better than internal fixation for metastases in the proximal femur. We need to analyze function and pain earlier than 6 weeks postoperative to investigate the benefit of surgery in patients with short time survival.
|
|
