Complete pathological response to neoadjuvant treatment is associated with better survival outcomes in patients with soft tissue sarcoma: Results of a retrospective multicenter study.

BACKGROUND: Locally advanced soft tissue sarcoma (STS) management may include neoadjuvant or adjuvant treatment by radiotherapy (RT), chemotherapy (CT) or chemoradiotherapy (CRT) followed by wide surgical excision. While pathological complete response (pCR) to preoperative treatment is prognostic for survival in osteosarcomas, its significance for STS is unclear. We aimed to evaluate the prognostic significance of pCR to pre-operative treatment on 3-year disease-free survival (3y-DFS) in STS patients. METHODS: This is an observational, retrospective, international, study of adult patients with primary non-metastatic STS of the extremities and trunk wall, any grade, diagnosed between 2008 and 2012, treated with at least neoadjuvant treatment and surgical resection and observed for a minimum of 3 years after diagnosis. The primary objective was to evaluate the effect of pCR. (≤5% viable tumor cells or ≥95% necrosis/fibrosis) on 3y-DFS. Effect on local recurrence-free survival (LRFS), distant recurrence-free survival (MFS) overall survival (OS) at 3 years was also analyzed. Statistical univariate analysis utilized chi-square independence test and odds ratio confidence interval (CI) estimate, multivariate analysis was performed using LASSO. RESULTS: A total of 330 patients (median age 56 years old, range:19-95) treated by preoperative RT (67%), CT (15%) or CRT (18%) followed by surgery were included. pCR was achieved in 74/330 (22%) of patients, of which 56/74 (76%) had received RT. 3-yr DFS was observed in 76% of patients with pCR vs 61% without pCR (p < 0.001). Multivariate analysis showed that pCR is statistically associated with better MFS (95% CI, 1.054-3.417; p = 0.033), LRFS (95% CI, 1.226-5.916; p = 0.014), DFS (95% CI, 1.165-4.040; p = 0.015) and OS at 3 years (95% CI, 1.072-5.210; p = 0.033). CONCLUSIONS: In a wide, heterogeneous STS population we showed that pCR to preoperative treatment is prognostic for survival.

Successful treatment of synchronous chemoresistant pulmonary metastasis from pleomorphic rhabdomyosarcoma with stereotaxic body radiation therapy: A case report and a review of the literature.

BACKGROUND: Rhabdomyosarcoma (RMS) is a highly malignant soft tissue sarcoma (STS), usually of adults, displaying skeletal muscle differentiation. STS principally metastasize to the lungs with more than 50% of metastatic patients presenting with isolated pulmonary metastasis. Paradoxically, the majority of drugs prescribed to treat RMS are associated with multidrug resistance. CASE REPORT: We report the case of a 53 year-old patient who developed three synchronous chemoresistant lung metastasis from pleomorphic RMS. Considering the poor prognosis, the patient's preference and the chemoresistance of her lung metastasis, we decided to perform two consecutive stereotactic body radiotherapy (SBRT) on two of these three lesions. DISCUSSION: Initially, the patient was referred to our institute with a painful mass in the anterior part of the left thigh increasing in volume for 3 months. Biopsy revealed a high-grade pleomorphic RMS. The cancer being staged IB, she had neoadjuvant radiotherapy. After complete surgical excision, pathology examination revealed a 6 cm Grade II pleomorphic RMS, with clear margins. Six months later, she developed three synchronous lung metastases. She got 4 courses of doxorubicin-ifosfamide which were poorly supported. After two courses, a heterogeneous (morphological and metabolic) response was observed, hence SBRT was delivered with a Biologically Equivalent Dose (α/ß10)> 100 Gray on the two more chemoresistant lesions. This SBRT was very well tolerated, no side effects were reported. The patient remained alive and achieved a complete response of these three metastases, which sustains after more than 3 years. CONCLUSION: Early recognition and proper management of these oligometastatic patients may lead to motivating results in a poor prognosis disease.

Clinical Outcome of Surgically Treated Leiomyosarcoma of the Extremities: A Retrospective Overview.

AIM: This study was interested in extremity leiomyosarcoma with focus on clinical outcome after surgery with or without adjuvant therapy. PATIENTS AND METHODS: A retrospective case series of all patients with leiomyosarcoma, surgically treated between 2000 and 2015 and a minimum follow-up of 2 years, was drawn from institutional databases in Belgium and the Netherlands. Postoperative complications were reported with the Radiation Therapy Oncology Group (RTOG) and the Henderson classification. RESULTS: Seventy-five patients were operated on, of whom 47 underwent (neo)adjuvant therapy. Infection was observed in 11 patients, seven associated with (neo)adjuvant radiotherapy. Dermatological complaints were observed in 26 patients, 10 associated with (neo)adjuvant radiotherapy. Overall survival was 60%. Local recurrence occurred in 11 (15%) patients. CONCLUSION: This study describes favourable clinical outcome following (neo)adjuvant radiotherapy. In the future, larger databases on leiomyosarcoma should enhance the power of these findings and define the benefits of adjuvant therapy in leiomyosarcoma.

Accuracy assessment of surgical planning and three-dimensional-printed patient-specific guides for orthopaedic osteotomies.

This study analyses the accuracy of three-dimensional pre-operative planning and patient-specific guides for orthopaedic osteotomies. To this end, patient-specific guides were compared to the classical freehand method in an experimental setup with saw bones in two phases. In the first phase, the effect of guide design and oscillating versus reciprocating saws was analysed. The difference between target and performed cuts was quantified by the average distance deviation and average angular deviations in the sagittal and coronal planes for the different osteotomies. The results indicated that for one model osteotomy, the use of guides resulted in a more accurate cut when compared to the freehand technique. Reciprocating saws and slot guides improved accuracy in all planes, while oscillating saws and open guides lead to larger deviations from the planned cut. In the second phase, the accuracy of transfer of the planning to the surgical field with slot guides and a reciprocating saw was assessed and compared to the classical planning and freehand cutting method. The pre-operative plan was transferred with high accuracy. Three-dimensional-printed patient-specific guides improve the accuracy of osteotomies and bony resections in an experimental setup compared to conventional freehand methods. The improved accuracy is related to (1) a detailed and qualitative pre-operative plan and (2) an accurate transfer of the planning to the operation room with patient-specific guides by an accurate guidance of the surgical tools to perform the desired cuts.

Management of periprosthetic fractures in patients treated with a megaprosthesis for malignant bone tumours around the knee.

Early and late infection as well as loosening and mechanical failure are among the postoperative complications most often reported following reconstruction with a tumour prosthesis in patients with a sarcoma around the knee. Scarce instances of periprosthetic fractures have been reported. The literature includes the description of two patients with such a complication, however no treatment recommendation was detailed in those articles. We recorded in our experience two patients with a traumatic fracture following a massive prosthetic bone and joint replacement after major resection for a bone sarcoma. The surgical treatment was open reduction and internal fixation using a cable and plate system, allowing preservation of the prosthesis and providing stable fracture fixation and excellent fracture healing.

Extra-skeletal Ewing's sarcoma in adults: presentation of two cases.

Extraosseous Ewing's sarcoma represents about 5% of the Ewing family of tumours. Two cases in adult patients are presented, emphasizing the complexity of a multi-modality treatment approach of this tumour. Clinical presentation, chemotherapeutical, surgical and radiotherapeutical approaches are discussed. A thorough literature search was done to correlate our therapeutic attitude with current knowledge of this very rare disease.

Case reports: subtrochanteric femoral stress fractures after prolonged alendronate therapy.

BACKGROUND: Alendronate is known for its ability to reduce bone loss in osteoporotic and osseous metastatic conditions. Its long-term effects remain unclear although several reports describe cases of proximal femur stress fractures associated with long-term alendronate use. CASE DESCRIPTION: We report the cases of four women who sustained low-energy subtrochanteric or femoral shaft stress fractures while being on alendronate therapy for more than 5 years. All radiographs showed typical patterns consisting of a transverse fracture line with external cortical bone reaction and medial cortical spike. Alendronate discontinuation along with nonoperative treatment was sufficient for one patient, whereas surgical stabilization was required in three patients. LITERATURE REVIEW: The side effects of alendronate therapy include osteonecrosis of the jaw, esophageal irritation, and musculoskeletal pain. Several cases of insufficiency femoral fractures associated with prolonged alendronate use have been reported. Their radiographic pattern and clinical presentation are consistent with our observations. Although various hypotheses have been suggested, the physiopathogenesis of these stress fractures is not completely understood. PURPOSES AND CLINICAL RELEVANCE: Although bisphosphonates play an important role in preventing pathologic fractures in patients with cancer, these subtrochanteric stress fractures associated with prolonged use of alendronate should not be ignored.