Search

We present the case of a 9-year-old girl who developed striking bone changes following two years of denosumab therapy for giant cell lesions of the jaw., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aim: To evaluate the mid-term clinical efficacy of microwave in situ inactivation combined with bone grafting or polymethyl methacrylate (PMMA) filling in the treatment of giant cell tumor of bone (GCTB)., Methods: This is a retrospective, descriptive, and analytical study. A total of 30 GCTB patients received microwave in situ inactivation from January 2012 to January 2020, whose clinical recurrence rate was evaluated at the last follow-up after microwave in situ inactivation surgery. The Musculoskeletal Tumor Society (MSTS) function score was used to evaluate the postoperative clinical panoramic results., Results: All patients were followed up for 21 to 110 months, with an average of 63.79 months. Distal femur (40%) and proximal tibia (28%) had a higher rate of GCTB incidence. Seventeen percent of tumor patients suffered from associated pathologic fracture. The rate of Campanacci classification stage III was 60%. The average MSTS score was evaluated as 27.53 points overall at the last follow-up. In terms of complications, three, two, two and one cases developed fat liquefaction, controllable tissue rejection reaction, incision infection and degenerative changes around lesion joint, respectively, without in situ recurrences and reoperation as well as distant lung metastasis., Conclusions: The method of microwave in situ inactivation combined with bone grafting or PMMA filling is prudently recommended as one of the options for the limb salvage treatment of giant cell tumor of long and periarticular bone., Level of Evidence: IV: case series., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Although total en bloc spondylectomy (TES) is strongly recommended for spinal giant cell tumor (GCT), it is extremely difficult to excise a L5 neoplasm intactly through the single-stage posterior approach. Given the risk of neurological and vascular injury, intralesional curettage (IC) is usually recommended for the treatment of L5 GCT. In this study, we presented our experience with the use of an improved TES to treat L5 GCT through the single-stage posterior approach., Methods: This study included 20 patients with L5 GCT who received surgical treatment in our department between September 2010 and April 2021. Of them, seven patients received improved TES without iliac osteotomy, and the other 13 patients received IC (n = 8), sagittal en bloc resection (n = 1), TES with iliac osteotomy (n = 3), and TES with radicotomy (n = 1) as control., Results: The mean operative time was 331.43 ± 92.95 min for improved TES group and 365.77 ± 85.17 min for the control group (p = 0.415), with the mean blood loss of 1142.86 ± 340.87 ml vs. 1969.23 ± 563.30 ml (p = 0.002). Postoperative treatment included bisphosphonates in nine patients and denosumab in 12 patients including one patient who changed from bisphosphonates to denosumab. Three patients who received IC experienced local recurrence, and no relapse was observed in improved TES group., Conclusion: Single-stage posterior TES for L5 GCT was previously considered impossible. In this study, we presented our experience with the use of an improved surgical technique for L5 TES through the single-stage posterior approach, which has proved to be superior to the conventional procedures in terms of blood loss control and complication and recurrence rates., Level of Evidence: IV., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The course of osteosarcoma patients primarily treated as such has been well described. Little, however, is known about patients who were primarily treated assuming a different tumor diagnosis., Methods: The database of the Cooperative Osteosarcoma Study Group COSS was searched (4.435 primary high-grade central osteosarcomas registered prior to 01/01/21). A different tumor entity had to have been assumed for at least one month after the initial diagnostic procedure before the correct diagnosis of osteosarcoma was finally made. Identified patients were analyzed for demographic, tumor-, and treatment-related factors as well as for survival outcomes., Results: 37 patients were identified. They were a median of 19.7 (2.7-60.4) years old at first presentation and were more likely to be females than males (23:14). Bone cysts (n = 8), giant cell tumor of bone (n = 6), and osteoblastoma (n = 6) were the most frequent of 29/37 (78%) benign, chondrosarcoma and its variants (n = 6) the most frequent of 8/37 (22%) malignant original diagnoses. Tumors affected the extremities in 23 (62%), the trunk in 11 (30%), and the craniofacial bones in 3 (8%). Only one patient received systemic treatment while assuming the different diagnosis (1/37, 3%). The median time until the correct diagnosis of osteosarcoma was made was 8 months (range: 1 month-14.1 years). At that time, 6/37 (16%) presented with metastatic disease. All patients went on to receive chemotherapy, 17/37 (46%) neo-adjuvantly. Histologic response was only evaluated in 13/17 (76%) patients and was good (< 10% viable tumor) in only 4/13 (31%) patients. In 31/37 (84%) patients, a surgically complete resection of all macroscopically identified tumor manifestations could be achieved. Five-year overall and event-free survival rates at 5 years were 50.2% (standard error: 8.6%) and 42.6% (8.5%), respectively., Conclusion: Osteosarcoma may initially be misdiagnosed and hence subjected to inappropriate treatment including misguided surgery. Once diagnosed correctly, some of the affected patients may still be cured if finally treated according to modern osteosarcoma standards., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To compare total en bloc spondylectomy with marginal margins against piecemeal spondylectomy with intralesional margins in the surgical treatment of Enneking stage III spinal giant cell tumor (GCT) in terms of local recurrence., Methods: A retrospective survival analysis of patients with Enneking stage III GCT who underwent TES with marginal margins or total piecemeal spondylectomy with intralesional margins was performed between January 2006 and April 2020. Local recurrence-free survival (LRFS) was the time between the date of surgery and recurrence. Factors with p-values < 0.05 in the univariate analysis were included in the multivariate analysis using proportional hazard analysis., Results: Sixty patients (25 men and 35 women) with a mean age of 35.6 (range 11-71) years were included. The mean follow-up duration was 93 (range 24-198) months. Two patients were lost to follow-up 6 and 14 years after the procedure. Over a 10-year period, the recurrence rate was 13.3%. The 2-, 5-, and 10-year LRFS rates were 95%, 88%, and 78%, respectively. Univariate analysis identified total piecemeal spondylectomy and no adjuvant radiotherapy as prognostic factors for LRFS. Multivariate Cox-regression models showed a significant association between local recurrence and total piecemeal spondylectomy and no adjuvant radiotherapy., Conclusion: TES with marginal margins is better than total piecemeal spondylectomy with intralesional margins owing to its lower postoperative recurrence rate. Adjuvant radiotherapy should be administered to reduce postoperative recurrence rates., (© 2022. The Author(s).)

The integrative evaluation of histology and corresponding imaging is essential for the classification of bone tumors. Until a few years ago, there were hardly any molecular markers that could be used for diagnostic purposes. However, exome- and genome-wide sequencing analyses have since uncovered a number of tumor-specific aberrations that can be very helpful in ambiguous cases. In addition to characteristic gene mutations (e.g. H3F3A and H3F3B in giant-cell tumors and chondroblastomas), the detection of fusion transcripts (e.g. structural rearrangements in the AP‑1 transcription factors FOS and FOSB in osteoid osteomas and osteoblastomas) plays an increasing role. The article gives an overview of the current state of knowledge of the most important alterations in bone tumors.

Background: The interest on the role of Denosumab in the treatment strategy of giant cell tumor of the spine is growing. En bloc resection is considered the Enneking appropriate treatment, but morbidity and functional loss are sometimes unacceptable. Denosumab could play a role as a stand-alone treatment, but also as preoperative treatment or as postoperative after intralesional surgery., Materials and Methods: A cohort of 10 out of 12 cases of spinal GCT consecutively treated with Denosumab are analyzed and discussed compared to the cases reported in the literature. A staging of the radiological effect of the treatment is proposed., Results: The stand-alone and postoperative treatments are still running (12 to 88 months). One therapy was stopped after 15 months, once a satisfactory local effect was achieved, but the treatment had to be restarted 2 months later due to the recurrence of the erosive images. The new treatment was successful. At 1-year follow-up after the gross total excision followed by postoperative Denosumab treatment, no evidence of local recurrence was found. The preoperative treatment duration ranged from 3 to 24 months. No local recurrence followed the en bloc resections., Conclusions: Denosumab alone is effective in relieving pain, increasing the ossification and sometimes reducing the tumor volume. It can be considered an excellent solution in spine GCTs whose surgical treatment cannot be Enneking appropriate or is associated with unacceptable morbidity or loss of functions. It is still impossible to state when to safely stop the treatment. Denosumab also plays a role as preoperative protocol. These slides can be retrieved under Electronic Supplementary Material.