Introduction: Osteosarcoma is the most common malignant bone tumor. It is currently treated with pre-and postoperative chemotherapy, associated with surgical resection of the tumor.
Area covered: ...Relapses occur in about one in three patients presenting with localized disease, and three in four of those with metastases at diagnosis. Relapsing disease carries a very poor prognosis, with 5-year survival rates ranging between 13% and 40%.
Expert opinion: Patients with unilateral lung involvement or solitary lung metastases and a recurrence-free interval (RFI) longer than 24 months have a better prognosis, and could be managed with surgical resection and close observation. Complete surgical resection of all sites of disease remains essential to survival: patients unable to achieve complete remission have a catastrophic overall survival rate. The role of second-line chemotherapy is not at all clear, and no controlled studies are available on this topic. It is worth considering for patients unable to achieve complete surgical remission, and those with multiple metastases and/or a RFI <24 months. Given their dismal prognosis, patients with multiple sites of disease not amenable to complete surgical resection should also be considered for innovative therapeutic approaches.
Secondary osteosarcoma: a challenge indeed Meazza, Cristina; Giovanna, Sironi; Nigro, Olga ...
International journal of clinical oncology,
2023/1, Letnik:
28, Številka:
1
Journal Article
Recenzirano
Background
The risk of survivors developing a secondary bone sarcoma after being treated for pediatric cancers is well established. The aim of this study was to examine the clinical characteristics ...and outcomes of patients with secondary osteosarcoma (SOS).
Methods
The study concerns survivors of childhood and adolescence primary neoplasms (PN) treated with chemotherapy, with or without radiotherapy and surgery, subsequently diagnosed with SOS.
Results
We identified 26 patients (13 females, 13 males) who developed SOS a median 7.3 years after being diagnosed with a PN (5/7 of these patients tested for Li–Fraumeni and found positive for the syndrome). The sample’s median age was 8.0 and 15.0 years when their PN and SOS were diagnosed, respectively. To treat their PN, 24 out of 26 patients had been given radiotherapy, and 19 had received chemotherapy including doxorubicin. A considerable number of SOS occurred at unfavorable sites (nine hip bone, six skull). All but one patient received chemotherapy with tailored schedules, omitting doxorubicin in 19 cases. Eighteen of the 26 patients underwent surgery. The 5- and 10-year overall survival and probabilities after the diagnosis of SOS (95% confidence interval) were 50% (32.7–76.5%) and 38.9% (22.4–67.4%); 5- and 10-year progression-free survival was 47% (29.9–73.7%) and 35.2% (19.3–64.4%), respectively.
Conclusions
The survival rates after SOS are lower than in patients with primary osteosarcoma, but not negligible. It is therefore mandatory to discuss the best choice of treatment for such patients at a referral center, in terms of their chances of cure and quality of life.
To improve the poor prognosis for children with metastatic osteosarcoma (OS), interleukin-2 (IL-2) was added to the standard treatment due to its capacity to activate lymphocytes and differentiate ...lymphocyte subsets into lymphokine-activated killer (LAK) cells that are capable of recognizing and killing various tumor cells. This study concerns a cohort of unselected patients aged < 18 years with metastatic OS, who were treated with IL-2, high-dose methotrexate, doxorubicin, cisplatin, ifosfamide, LAK reinfusion, and surgery, between 1995 and 2010. Thirty-five patients aged 4–17 years were involved. Thirty-two of the 35 patients underwent surgery on their primary tumor, and 25 had surgery on lung metastases too. Twenty-seven patients received IL-2 plus LAK reinfusion. The median follow-up was 130 months (77–228), and the 3-year event-free and overall survival rates were 34.3 and 45.0%, respectively. Eleven patients remained alive, all of whom achieved a complete surgical removal of the primary tumor and lung metastases (1 patient did not receive lung resections due to complete lung metastases remission). Patients who had a complete surgical remission of the primary and metastatic sites and who responded well to chemotherapy had a better event-free survival. These results confirm the importance of complete surgical remission and point to a noteworthy (though still be ameliorate) survival rate in our series of patients, underling a potential role for immunotherapy with IL-2 and LAK/NK cell activation.
Introduction
The aim of this retrospective study was to investigate the clinicopathological characteristics of AYA sarcomas and their clinical outcomes at a high‐volume single center.
Methods
...Demographic, clinicopathological data on the diagnosis, treatment and follow‐up of all sarcoma patients aged 16–39 years (ys) observed at our Institute between January 2010 and December 2021 were retrospectively collected, including diagnostic (TTD) and treatment delay(TTT), clinical outcomes (OS and PFS), and late‐treatment effects.
Results
We identified 228 AYA patients, median age 30 years, 29% ≤ 25 years, 57% males, 88% soft tissue sarcomas (STS), and 12% bone sarcomas (BS). Among STSs, 13% were small round cell tumors (SRCT), 52% intermediate–high‐grade, 24% low‐grade STSs. Among BS, 32% were high‐grade. Median TTD and TTT were 120 (0–8255) and 7 days (0–83), respectively. Surgery was performed in 83%, radiotherapy in 29%, and systemic therapy in 27%. Median follow‐up was 72.9 months(1.6–145), 5‐year and 10‐year OS were 78.5% and 62%, respectively. Kaplan–Meyer analysis showed a significantly better 5‐year OS and PFS for patients with >92 days of TTD (OS 85.7% vs. 66.7%, p = 0.001, PFS 50.2% vs. 24.9%, p = 0.009). According to age (≤25 years vs. > 25 years), 5‐year OS was 69.8% versus 82.2%, respectively (p = 0.047).
Conclusion
Our analysis confirmed previous data on sarcoma AYA patients followed in a referral center. Unexpectedly, diagnostic delay was not associated with poor OS and PFS. Patients <25 years showed a poorer prognosis due to the higher incidence of SRCT.
Trabectedin is effective in leiomyosarcoma and liposarcoma, especially the myxoid variant, related to the presence of the FUS-CHOP transcript. We evaluated the efficacy of trabectedin in specific ...subgroups of patients with soft tissue sarcomas (STS).
Seventy-two patients with advanced anthracycline-pretreated STS, who received trabectedin at a dose of 1.5 mg/m(2) every 3 weeks by continuous 24-hour infusion, were retrospectively analyzed. Best response rate according to Response Evaluation Criteria In Solid Tumors (RECIST) criteria and severe adverse events (AEs) according to National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE v4.02) were evaluated. Secondary endpoints included progression-free survival and overall survival (OS).
Median age was 48 (range, 20-75) years, with a median Eastern Cooperative Oncology Group performance status of 0. The median number of previous chemotherapy regimens was 1 (range, 0-5). Median number of trabectedin cycles was 3 (range, 1-17). About 69/72 patients (95.8%) were evaluable for response: 9 patients (13%) achieved partial response and 26 (37.7%) stable disease. According to histotype, clinical benefit (partial response + stable disease) was reported in synovial sarcoma (n=5), retroperitoneal liposarcoma (n=10), myxoid liposarcoma (n=5), leiomyosarcoma (n=8), high-grade undifferentiated pleomorphic sarcoma (n=5), Ewing/peripheral primitive neuroectodermal tumor (n=1), and malignant peripheral nerve sheath tumor (n=1). Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%), neutropenia (n=7, 9.6%), liver toxicity (n=6, 8.3%), and fatigue (n=2, 2.8%). With a median follow-up time of 11 (range, 2-23) months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively.
Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and leiomyosarcoma and suggests promise in synovial sarcomas and high-grade undifferentiated pleomorphic sarcoma.
•Distal radius reconstruction with fibula flap after tumor resection allows to conserve wrist motion.•The fibulo-scapho-lunate fusion is a well described technique with good functional results.•The ...distal fixation of fibula on proximal carpal row give the sufficient stability for early rehabilitation.
Fibulo-scapho-lunate fusion is a technique that allows residual movement in the wrist in case of wide bone resection replacing the distal radius by a vascularised fibular transfer. Some authors have used this technique with favourable results but the distal synthesis seems to not be standardised at all, many different osteosynthesis methods have been proposed. This paper reports a complete review of the present day literature about this subject and, evaluating the different proposed osteosynthesis techniques referred in literature, suggests a standardization of the synthesis methods with dorsal plating. We report some technical considerations and results of three cases operated with a stable dorsal osteosynthesis (twice with a double plate and once with a long plate). We evaluate the time of healing and the clinical result.
Introduction
Pelvic ring reconstruction after internal hemipelvectomy is an extremely challenging surgical procedure, and mandatory reconstruction is to provide a durable and pain‐free functional ...outcome, especially for young, active patients. One of the most widely employed techniques is reconstruction with a double‐barreled fibular free flap (DBF). The aim of our work was an in‐depth analysis of the outcome of pelvic ring reconstruction performed using the above‐mentioned method, in particular looking for a correlation between the fixation technique and either ambulation status or complications.
Materials and methods
A systematic review was performed in November 2020 using PubMed and MedLine Ovid databases according to the PRISMA guidelines and the results were statistically analyzed.
Results
Studies were published between 1994 and 2015. A DBF was used in each case, with a total of 30 patients. Reconstruction was performed with screws in 13 cases, Cotrel‐Dubousset rod fixation in 6, screws and plate in 5, screws with external fixation in 4, and ISOLA in 2. Follow‐up time ranged from 3 to 131 months. Functional outcome was excellent in 4 patients and good in 26 patients. Statistical analysis showed no statistical evidence of existing correlation between fixation technique and complications (p = .873), while statistical correlation between age and fixation technique was found (p < .001).
Conclusion
Reconstruction of pelvic ring with DBF provides an overall good functional outcome. Our data indicate that there is no statistical evidence of existing correlation between the fixation technique and either complications or ambulation status.
Introduction
Calcaneal tumors being a rare occurrence, surgical options and outcomes are not well‐known. Extensive defects following wide resection, especially in weight‐bearing areas, still remain a ...challenge and different reconstructive techniques have been proposed. The aim of this report was to analyze the clinical and functional long‐term outcomes of heel reconstruction using an iliac crest free flap.
Patients and methods
Four patients who underwent calcaneal reconstruction between 1999 and 2012 were included. Two were females and mean age was 27 years, ranging 18–42 years. Each patient underwent total calcanectomy, for two osteoblastomas, one osteosarcoma, and one Ewing's sarcoma. An iliac crest flap was harvested and shaped to fit the residual space. After the articular cartilage at recipient site was debrided, the flap was fixed to the talus and the cuboid.
Results
The average size of the flaps was 2 x 7 x 5 cm. Postoperatively wound dehiscence, screw breaking, and graft fracture healed conservatively. All the arthrodesis healed successfully and no donor site complication occurred. At an average follow‐up of 13 years (range 6–19 years) any patient claimed pain, evident limp or limitation of daily activities. Computerized pedobarographic examination and gait analysis revealed a satisfactory result and an acceptable weight‐bearing area in the reconstructed limb in each patient.
Conclusions
Calcaneal reconstruction with iliac crest free flap is likely to provide good chances of a long‐lasting result, especially in young patients. Particularly, it provides the possibility to adequately shape the graft to fit the bone loss while using the crest as the weight‐bearing surface.
Reconstruction of foot structures is challenging because of the need to restore both anatomy and function. Extensor hallucis longus (EHL) excision without reconstruction could result in a flexion ...deformity of the toe at the interphalangeal joint. In this report, we present a case of the use of a palmaris longus tendon included in a fasciocutaneous radial forearm free flap to reconstruct EHL tendon and soft tissues of the distal foot dorsum. A 41‐year‐old woman presented with a recurrence of myxoinflammatory fibroblastic sarcoma on the medial part of the left foot dorsum. The EHL tendon was sacrificed, leaving a soft‐tissue defect of 14 × 5 cm and 14‐cm tendon gap. A 14 × 5 cm radial forearm flap with a 16‐cm section of palmaris longus tendon was harvested from the left forearm. Radial artery and its comitant vein were anastomosed with dorsalis pedis artery and vein. EHL tendon repair was performed from the composite mass to the proximal and distal stumps. No complication in the postoperative period occurred. At latest follow‐up, 12 months postoperatively, the patient showed a normal joint function and was satisfied with the treatment. Palmaris longus tendon included in a radial forearm free flap could be recommended for combined soft tissue and tendon defects, particularly on the foot dorsum.
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