The management of primary malignant bone tumors with metastatic disease at presentation remains a challenge. While surgical resection has been shown to improve overall survival among patients with non-metastatic malignant bone tumors, current evidence regarding the utility of surgery in improving overall survival in metastatic patients remains limited. The 2004–2016 National Cancer Database (NCDB) was queried using International Classification of Diseases 3rd Edition (ICD-O-3) topographical codes to identify patients with primary malignant bone tumors of the extremities (C40.0-C40.3, C40.8 and C40.9) and/or pelvis (C41.4). Patients with malignant bone tumors of the axial skeleton (head/skull, trunk and spinal column) were excluded, as these cases are not routinely encountered and/or managed by orthopaedic oncologists. Histological codes were used to categorize the tumors into the following groups - osteosarcomas, chondrosarcomas, and Ewing sarcomas. Patients who were classified as stage I, II or III, based on American Joint Commission of Cancer (AJCC) guidelines, were excluded. Only patients with metastatic disease at presentation were included in the final study sample. The study sample was divided into two distinct groups – those who underwent surgical resection of the primary tumors vs. those who did not receive any surgery of the primary tumor. Kaplan-Meier survival analysis was used to report unadjusted 5-year overall survival rates between patients who underwent surgical resection of the primary tumor, compared to those who did not. Multi-variate Cox regression analyses were used to assess whether undergoing surgical resection of the primary tumor was associated with improved overall survival, after controlling for differences in baseline demographics, tumor characteristics (grade, location, histological type and tumor size), and treatment patterns (underwent metastatectomy of distal and/or regional sites, positive vs. negative surgical margins, use of radiation therapy and/or chemotherapy). Additional sensitivity analyses, stratified by histologic type for osteosarcomas, chondrosarcomas and Ewing sarcomas, were used to assess prognostic factors for overall survival. A total of 2,288 primary malignant bone tumors (1,121 osteosarcomas, 345 chondrosarcomas, and 822 Ewing sarcomas) with metastatic disease at presentation were included – out of which 1,066 (46.0%) underwent a surgical resection of the primary site. Overall 5-year survival rates, on unadjusted Kaplan-Meier log-rank analysis, were significantly better for individuals who underwent surgical resection vs. those who did not receive any surgery (31.7% vs. 17.3%; p<0.001). After controlling for differences in baseline demographics, tumor characteristics and treatment patterns, undergoing surgical resection of primary site was associated with a reduced overall mortality (HR 0.42 [95% CI 0.36–0.49]; p<0.001). Undergoing metastectomy (HR 0.92 [95% CI 0.81–1.05]; p=0.235) was not associated with a significant improvement in overall survival. On stratified analysis, radiation therapy was associated with improved overall survival for Ewing Sarcoma (HR 0.71 [95% CI 0.57–0.88]; p=0.002), but not for osteosarcoma (HR 1.14 [95% CI 0.91–1.43]; p=0.643) or chondrosarcoma (HR 1.08 [95 % CI 0.78–1.50]; p=0.643). Chemotherapy was associated with improved overall survival for osteosarcoma (HR 0.50 [95% CI 0.39–0.64]; p<0.001) and chondrosarcoma (HR 0.62 [95% CI 0.45–0.85]; p=0.003), but not Ewing sarcoma (HR 0.79 [95% CI 0.46–1.35]; p=0.385). Surgical resection of the primary site significantly improves overall survival for primary malignant bone tumors with metastatic disease at presentation. Physicians should strongly consider surgical resection of the primary tumor, with adjunct systemic and/or radiation therapy (dependent on tumor histology), in patients presenting with metastatic disease at presentation

Introduction and Purposes. Custom made acetabular prosthesis are a valid option for the reconstruction after the resection of pelvic tumors. They should guarantee a stable and reliable reconstruction for the expected survival of the patient. Nevertheless in many cases periacetabular metastatic lesions have been compared to high grade (IIIA-B) Paprosky defects, but treated with low or intermediate longevity implants. Some complex post-traumatic scenarios or total hip arthroplasty (THA) multiple failures need a reconstruction according to oncologic criteria to fill in the huge defect and to obtain an acceptable function. The aim of the study is to compare 3D custom-made implants for tumors and for THA failures. Materials and Methods. Three custom-made implants after tumor resection (group A: 1 chondroblastic osteosarcoma, 1 bifasic synovialsarcoma, 1 high grade chondrosarcoma) were evaluated and compared to 3 acetabular complex reconstructions after non-oncologic bone defect (group B: 3 cases of aseptic loosening after at least 2 revisions). All the implants were case-based designed, 3D printed, and realized with porous or trabecular surfaces on a Titanium base prosthesis. Age range 16–70 ys in oncologic patients and 60–75 ys in non-oncologic patients. The bone defect to be reconstructed after tumor resection was classified according to Enneking zones (1 type 1-2-3 resection, 1 type 2 resection, 1 partial type 2 resection). Non-oncologic cases were comparable in term of remaining bone stock and classified according to Paprosky classification for acetabular defects as 1 type IIIA an 2 type IIIB. Complications, MSTS functional score, necessity of walking-aids were evaluated at minimum follow up of 1 year. Results. In both groups, good functional results were obtained (MSTS score 25/30 in both groups). No cases of aseptic loosening and no infection occured. After 3 months of partial or no weight-bearing on the operated limb, 3 patients were able to walk unaided and 3 walked with one cane or crutch. No limb length discrepancy (major than 2 cm) were observed. Limping was present in oncologic cases due to muscle resection. Overall better results in term of satisfaction and quality of life were obtained in younger (oncologic) patients. Conclusions. Complex THA revision cases can sometimes be considered for a reconstruction with oncologic criteria. Even if indications are limited an acceptable functional results can be obtained. In the past for these cases a Girldestone resection was the only option. A collaboration among orthopaedic oncologist and hip arthroplasty surgeons is advisable in major orthopaedic centers to improve the prosthetic design and the functional results

The orthopaedic surgeon is often consulted to manage pathologic fractures due to metastatic disease, even though he or she may not be an orthopaedic oncologist. A good understanding of the principles of management of metastatic disease is therefore important. The skeleton remains a common site for metastasis, and certain cancers have a predilection for bone, namely, tumors of the breast, prostate, lung, thyroid, and kidney. Myeloma and lymphoma also often involve bone. The proximal femur and pelvis are most commonly affected, so we will focus on those anatomic sites. The patient may present with pain and impending fracture, or with actual fracture. Careful preoperative medical optimization is recommended. If the lesion is solitary, or the primary is unknown, the diagnosis must be made by a full workup and biopsy before definitive treatment is planned. For patients with known metastasis (the most common situation), the options for treatment of pathologic lesions of the proximal femur generally center on internal fixation versus prosthetic replacement. Patients with breast or prostate metastasis can live for several years after pathologic fracture, so constructs must be relatively durable. If fixation is chosen, it must be stable enough to allow full weight bearing, since the overwhelming majority of pathologic fractures will never heal. In general, long constructs are chosen to protect the entire length of the bone. Nails should protect the femoral neck as well, so cephalomedullary devices are typically chosen. Megaprostheses can be useful in situations where bony destruction precludes stable internal fixation. Postoperative radiation is recommended after wound healing. Acetabular involvement typically requires reinforcement rings or cement augmentation with the Harrington technique. Careful multi-disciplinary medical management is recommended to minimise complications