Customized accurate tumor resection and individualized reconstruction is a challenging in treatment of malignant bone tumor. Three-dimensional (3D)-printing technique is now widely used in the ...resection and following reconstruction of malignant bone tumor, which included but not limited to tumor model, osteotomy guide and customized implant.
We retrospectively reviewed 17 patients, who underwent limb salvage surgery by using 3D-printed guide at a single center between August 2014 and October 2019. The median duration of follow-up was 26.5 months. Osteosarcoma (41.2%) were the predominant diagnoses. The functional outcomes were assessed by Musculoskeletal Tumor Society (MSTS) functional score. We also analyzed survival status, intraoperative data (blood loss, operation time and resection length), reconstruction method, margin outcomes and complications.
We totally performed 93 guided osteotomies on affected bone and allograft bone in 17 patients. Reconstruction in 12 cases was performed with biological technique: allograft combined with autograft was used in 7 cases. 11 of 12 (91.7%) cases showed a good bone healing in both allograft and autograft. 1 of 12 (8.3%) cases had allograft necrosis. Additional intra-operative extracorporeal radiation was performed in 3 pelvic cases for reconstruction. 63 of 64 (98%) osteotomies achieved wide resection and negative margin. All the cases had successful limb salvage result without amputation. At the latest follow up, the mean MSTS Score was 24 (range: 13–30), 12 patients alive with no evidence of disease, 1 patient alive with disease, 5 patients had died of disease and 5 years overall survival is 73.3%. The most common complications are wound healing disorder in 4 cases (23.5%) and infection in 3 cases (17.6%).
The 3D-printed resection guide was easy to use and showed promise in the field of orthopedic oncology. It can not only used in primary malignant bone tumor personalized resection but also in shaping structural bone allograft in biological reconstruction, which can achieve a safety surgical margin and individualized resection at the same time.
•Analyze the treatment outcome by using 3D-printed guides in the resection and reconstruction of malignant bone tumors in our institution.•The use of personalized, 3D-printed guides show promising results in the field of orthopedic oncology. It can not only used in primary malignant bone tumor personalized resection but also in shaping structural bone allograft in biological reconstructions.•Reviewed the previous articles in treatment of bone tumors by 3D printed guide on pelvis and extremities.
The retrograde femoral approach is an established technique for implantation of nails for leg lengthening and correction and in cases of distal femoral fractures. The purpose of this study was to ...determine the 10-year outcome of this technique by analyzing the clinical long-term effects and radiological status of the knee after leg lengthening via a retrograde femoral approach.
This retrospective single-center study included 13 patients (median age at surgery 17 range 15-20 years) who underwent unilateral, retrograde, femoral lengthening with a motorized nail. Outcome measurements were graded variables of the SF-36, ISKD score, and Lysholm score. MRI of both knees was performed in all patients. MRI was evaluated for the presence of degenerative changes and compared with the healthy contralateral knee. Cartilage condition was graded according to the International Cartilage Repair Society (ICRS) scoring system.
All patients were pain-free and had a full range of motion 10 (range 10.0-12.2) years after surgery. All postoperative knees showed fibrosis of Hoffa's fat pad and moderate to severe cartilage defects (ICRS Grade 2-4) of the trochlear groove (nail entry site). 6 out of 13 operated knees exhibited retropatellar cartilage defects.
Our study showed that patients were pain-free, but cartilage defects at the entry point and arthrofibrosis at Hoffa's fat pad were observed without causing clinical impairment.
Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years ...that evaluate long-term outcomes for survivors.
Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years.
Mean age at diagnosis was 35.4 years (range 6–82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5–14.9 years) and metastases at a mean of 5.7 years (range 0.5–16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment.
In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.
Abstract Purpose Prognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ...ESOS prognosis. Patients and methods Members of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS. Results Data from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40–54%). Five-year OS for metastatic patients was 27% (95% CI 13–41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44–59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35–51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis. At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival. Conclusion Patient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin.
Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas ...is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.
Rearrangements of the transcription factors FOS and FOSB have recently been identified as the genetic driver event underlying osteoid osteoma and osteoblastoma. Nuclear overexpression of FOS and FOSB ...have since then emerged as a reliable surrogate marker despite limitations in specificity and sensitivity. Indeed, osteosarcoma can infrequently show nuclear FOS expression and a small fraction of osteoblastomas seem to arise independent of FOS/FOSB rearrangements. Acid decalcification and tissue preservation are additional factors that can negatively influence immunohistochemical testing and make diagnostic decision-making challenging in individual cases. Particularly aggressive appearing osteoblastomas, also referred to as epithelioid osteoblastomas, and osteoblastoma-like osteosarcoma can be difficult to distinguish, underlining the need for additional markers to support the diagnosis. Methylation and copy number profiling, a technique well established for the classification of brain tumors, might fill this gap. Here, we set out to comprehensively characterize a series of 77 osteoblastomas by immunohistochemistry, fluorescence in-situ hybridization as well as copy number and methylation profiling and compared our findings to histologic mimics. Our results show that osteoblastomas are uniformly characterized by flat copy number profiles that can add certainty in reaching the correct diagnosis. The methylation cluster formed by osteoblastomas, however, so far lacks specificity and can be misleading in individual cases.